2010 ASN Abstracts

1.  Vertebral Artery Pseudoaneurysm Presenting as Swishing Noise Jayashree Sundararajan, Manoj Mittal, Saud Khan Kansas University Medical Center, Kansas City, Kansas, United States A 48‐year‐old male with history of gun shot wound to the neck 20 years ago presented with chronic daily headaches, left face numbness, left ear “swishing “ noise and intermittent dizziess. Examination revealed tongue deviation to the left and decreased sensation on the left facial on light touch and pinprick. Computer tomographic angiography showed left vertebral artery pseudoaneurysm (measuring 18 mm × 13 mm × 10 mm) which was located between the C1 vertebral body and skull base. Four vessel ateriogram confirms the same findings. MRI brain was not performed due to the presence of the bullet. It is hypothesized that this large pseudoaneurysm caused thromboembolic phenomena. Extracranial vertebral artery injury and pseudoaneurysm formation is most often due to trauma often penetrating in nature (stabbing, gunshot wound, sharpnel) (1). Symptoms secondary to vertebral artery pseudoaneurysm are variable and result from mass effect or arterial insufficiency. Lower cranial nerve dysfunction can be observed if the pseudoaneurysm is large with intraspinal or intracranial extension or secondary to infarction or ischemia from microembolization or thrombosis of vertebral artery or PICA (1). Sometimes the speudoaneurysms can be safely observed; however spontaneous thrombosis or rupture may occur (2). References: 1. Detwiler K, Godersky JC, Gentry L. Pseudoaneurysm of the extracranial vertebral artery. Journal of Neurosurgery 1987;67(6):935–9394. 2. Schittek A. Pseudoaneurysm of the vertebral artery. Tex Heart Inst J. 1999;26:90–95. 2.  Giant Dolichoectatic Vertebrobasilar System. Clinical Presentation and Endovascular Approach: A Case Report Esteban Cheng‐Ching, Lama Chahine, Muhammad S Hussain, Thinesh Sivapatham, Mark Bain, Shaye Moskowitz, Rishi Gupta The Cleveland Clinic Foundation, Cleveland, OH, United States Introduction: Vertebrobasilar dolichoectasia (VBD) is associated with increased morbidity and mortality. Disease progression has been linked to increased rates of stroke and death. Unfortunately the treatment options are limited, carrying a significant high risk. We report a case of a patient with a gigantic VBD presenting with clinical worsening, who underwent stent‐assisted coil embolization (SACE). Patients (or Materials) and Methods: Patient medical records and imaging studies were reviewed. Results: 54 year old man with history of hypertension, aortic dissection, and known stable VBD, was lost to follow up and presented 2 years later with headaches, progressive diplopia, facial palsy, dysarthria, dysphagia, and ataxia. A brain CT showed a marked interval enlargement of the VBD producing mass effect on the brainstem. Anterior circulation aneurysmal dilatation was also seen. An angiogram showed a diffusely enlarged vertebrobasilar system with partially thrombosed aneurysm. Endovascular reconstruction was performed. Angiographic catheters were advanced to the distal basilar artery, and positioned assisted by an inflated balloon. A stent was deployed across the aneurysmal dilatation in order to recreate a lumen, and coil embolization was performed with near complete occlusion of the aneurysm. After the procedure, the patient remained in the Neurointensive care unit, and he was discharged few days later. Conclusion: VBD can enlarge to significant dimensions, producing compressive symptoms in the brainstem, as well as thrombosis and ischemic strokes. SACE of the dolichoectasia can be effective for the vessel reconstruction, to prevent progression of the dilatation, and other potential clinical events. 3.  Three Case Reports of Multiple Carotid and/or Vertebral Artery Dissections in Female Mei Lu, Heather Gornik, Esteban Cheng Ching, Vikram Kashyap, Rishi Gupta Cleveland Clinic Foundation, Cleveland, OH 44195, United States Introduction: Multiple carotid and/or vertebral artery dissections are rare. However, patients with multiple dissections can suffer from severe stroke, leading to significant mobility and mortality. The etiology underlying the bilateral carotid dissection is not clear. The effective treatment approaches are not known. Patients (or Materials) and Methods: Case review of 3 young females with multiple carotid and/or vertebral artery dissections. Results: Three cases of young females presented with spectrum of clinical symptoms ranging from ear pain, neck pain, headaches, ptosis to frank stroke symptoms. Each of them has different possible etiology, from post‐partum, possible inflammation with elevated ESR, and genetic disorder Loeys‐Dietz syndrome with TGFBR2 mutation. All three patients underwent medical treatment including anti‐platelet and/or anticoagulant. In addition, They all had endovascular interventions, including stenting, and/or coiling. Patient's clinical symptoms and signs had been improved post intervention during follow‐up visits. Conclusion: Bilateral carotid dissection should be considered even in patient with mild symptoms, such as ear, head and neck pain in young female. Early detection is essential for better outcome. Post‐partum, inflammation, and genetic disorders are possible etiology. Extensive genetic workup for connective tissue disorders, such as Loeys‐Dietz syndrome is important. Even though different treatment regimen and modality may be needed depending on different etiology. Intravascular interventions may be critical in certain situations despite of different underlying causes. 4.  Choreoathetosis and Personality Change as a Manifestation of Fahr's Syndrome due to Hypocalcemia and of the Pituitary Tumor Adel Olshansky, Nerses Sanossian University of Southern California, Los Angeles, CA, United States Introduction: Hypocalcemia can present with neurological symptoms such as choreoathetosis and personality change and can result from hypoparathyroidism, hypermagnesemia, vitamin D deficiency, renal failure, and hyperphosphatemia. Neuroimaging can often reveal extensive cerebral calcification and help to establish this diagnosis and its etiology. We present a case in which neuroimaging helped establish the diagnosis of Fahr's syndrome and pituitary adenoma. Patients (or Materials) and Methods: Case Report Results: A 55 year old Hispanic male presented with 4 months of progressive involuntary movements of the left arm and leg and with 3 weeks of blurry vision. His wife noted worsening irritability and confusion. Neurological examination revealed limited orientation, difficulty with attention, overshooting on saccadic pursuit, bilateral choreoathetoid movements, mildly increased leg tone, and inability to perform coordination tasks on left due to involuntary movements. A non‐contrast head CT revealed diffuse symmetrical calcification sparing the cortex and involving the subcortical and cerebellar white matter. Calcium and PTH levels were low at 5.7 and 3.0. Patient improved with intravenous calcium gluconate, and was placed on calcium and vitamin D supplementation. Brain MRI showed suprasellar mass consistent with macroadenoma and prolactin was elevated at 335.9. Bromocriptine therapy was initiated. Conclusion: Neuroimaging is the key to the evaluation of hypocalcemia for diagnosis of Fahr's syndrome. Prompt identification and correction of hypocalcimia led to clinical improvement. 5.  Does a 320 Slice CT Scanner Change our Management in Acute Ischemic Stroke? The Buffalo Experience Bijal Mehta, Ziad Darkhabani, Maxim Mokin, Tareq Kass‐Hout, Vladan Radovic SUNY Buffalo, Buffalo, NY, United States Background: The first 320 slice CT scanner was introduced in late 2007. Since then more centers have installed this updated scanner to replace widely used 64 slice scanner. Millard Fillmore Gates Hospital in Buffalo is a JHACO accredited primary stroke center, with 24 hour stroke, neurosurgical, and endovascular care available. Our Toshiba Aquillon 320 slice CT scanner was installed in May 2009. Since that time, almost every acute stroke is scanned using this CT scanner. However, the utility as to whether this scanner has changed our management of acute stroke and specifically which treatment modality used. Acute stroke patients, after initial history, physical exam, and NIHSS calculation, are determined to be eligible to receive IV tPA, IA tPA or interventional procedure candidate, or have an intracerebral hemorrhage and are seen by the neurosurgical team. A CT of the head and CT angiogram of the head and neck vessels are performed on all acute strokes in our facility. A treatment and diagnostic plan is usually decided based on the history, physical exam, the time of onset of the stroke, and findings on the patient's CT scan. Objective: To determine if the numbers of IV tPA cases, IA tPA‐interventional procedures, or neurosurgical cases changed in frequency from 2008 to 2009 when only the 64 slice scanner was used versus after installation of the 320 slice CT scanner. Results: A retrospective chart review will be performed on all acute stroke patients presenting to MFG in the 12 months prior and following installation of the 320 slice CT scanner. Information regarding demographic data, stroke severity (NIHSS), which CT scanner used, and CT modalities will be obtained. Patients will be initially separated into two groups: those who had the 64 slice CT scan in the 12 months prior to installation of the 320 scanner, and those who had a 320 slice scan in the 12 months since installation. The numbers of IV tPA, IA tPA‐interventional procedures, and neurosurgical hemorrhage cases will be obtained in these two groups. Student t‐test and ANOVA will be used to determine if the monthly average number of each case type (i.e. “no intervention, IV‐tPA, IA‐tPA or procedural, or hemorrhage” group) varies from those who had the 64 slice CT scans versus the 320 slice CT scans. Conclusion: Does the installation of 320 slice CT scanner increase the numbers of strokes diagnoses and alter the management of such cases? Our conclusions will be presented in the final presentation at the meeting as this is a work in progress and based on the ongoing collection of data as described above. 6.  Vertebral Artery Origin Disease: A Neglected Cause of Posterior Circulation Stroke and TIA Nishant Kumar, Paisith Piriyawat, Salvador Cruz‐Flores, Nirav Vora, Aninda Acharya, Eliahu Feen, Jacob Kitchner, Sushant Kale, Randall Edgell Saint Louis University, Saint Louis, MO, United States Background and Purpose: Vertebral Artery Origin Disease (VAOD) is present in 20% of patients with posterior circulation ischemia; in 9% of these patients no other possible etiology will be identified. Given the high incidence of VAOD ands its treatable nature, we performed this investigation into the efficacy of screen for VAOD at Saint Louis University. Methods: All patients admitted to the vascular neurology service at Saint Louis University Hospital between November 2005 and April 2007 were identified using an institutional database. Those patients who had posterior circulation ischemic strokes were included. Patients diagnosed with transient ischemic attacks (TIAs) were included if there were no symptoms or signs of anterior circulation involvement. Patients were said to have been screened for VAOD if they underwent a computed tomographic angiogram (CTA), magnetic resonance angiogram (MRA), or catheter angiogram in which the vertebral artery origins were visualized. VAOD was defined as an occluded or >50% stenosed vertebral artery origin. The screening studies were then reviewed, blinded to the original interpretation. Results: There were 257 patients admitted for ischemic stroke or TIA in the specified period. Of these, 44 (17%) were found to have a posterior circulation stroke or TIA (22 stroke; 22 TIA). The average age of these patients was 63 years and 22 were male. Thirteen (29%; 1 TIA and 12 stroke) patients underwent screening for VAOD. Seven (16%) of these screening studies were interpreted as demonstrating VAOD. When the images of those patients who underwent a screening test were retrospectively reviewed, 3 (7%) additional cases were identified. No patients underwent a revascularization procedure. Conclusions: Despite the high incidence of VAOD in the setting of posterior circulation ischemia in this study (23%), screening was only performed in a minority of patients (13 of 44). When screening was performed, not all cases were identified. These findings highlight the need for more consistent inclusion of vertebral artery origin imaging when searching for the cause of posterior circulation ischemia. This is particularly important with the increasing availability of low risk interventional treatments. 7.  Reversible Cerebral Vasoconstriction Syndrome: Case Report and Review of the Literature Michael Lee, Harpreet Johl, Nerses Sanossian University of Southern California, CA, United States Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by a sudden, severe headache at onset, vascular narrowing involving the circle of Willis and its immediate branches, and evidence of vasoconstriction with reversibility within days to weeks of onset. The diversity of classification, pathology, and subspecialties that may encounter RCVS has hampered diagnosis of this recently characterized entity. Patients (or Materials) and Methods: A 37 year old Caucasian female with a past medical history of migraine and mixed connective tissue disorder presented with a history of maximal onset headache after sexual intercourse. She had been using pseudoephedrine for upper respiratory symptoms at the time. The next day a lumbar puncture showed protein 46 glucose 60, 2 wbc, tube 1 360 RBC tube 4 323 RBC without xanthochromia. Extensive serologies including anticentromere, anti‐RNP, ANA, SSA, lupus anticoagulant, complement, DS‐DNA, anti‐Smith, anti‐histone, hepatitis panel, cryoglobulin, HIV and urine toxicology were unremarkable. Results: MRI brain showed evidence of several punctuate non‐specific T2/FLAIR hyperintensities in the periventricular white matter. CTA was significant for long segments of narrowing in the anterior and posterior circulations involving bilateral A1 and left A2 segments, bilateral M2 segments, superior cerebellar arteries as well as the right Vertebral artery. No evidence of SAH or an unruptured aneurysm was found. Conclusion: RCVS is an under‐recognized cause of headache. It is typified by a young woman with acute, severe headache, a history of migraine, cold medication usage, dynamic improvement of angiographic abnormalities, and can be treated with steroids and calcium channel blockers. 8.  Fatal ICH and SDH Following Intravenous Cerebral Thrombolysis Mohammed Zaman 1 , Robert Malka 1 , Karen Wilson 2 , Reza Behrouz 1 , Michael Sloan 1 
 1 University of South Florida, Tampa, FL, United States, 2 Tampa General Hospital, Tampa, FL, United States Introduction: Intracranial hemorrhage (ICH) follows intravenous tissue plasminogen activator (tPA) therapy for acute ischemic stroke (AIS) in 1–16% of cases. Combined ICH and subdural hematoma (SDH) has rarely been reported following myocardial infarction but not AIS. Patients (or Materials) and Methods:Case ReportResults: We report a 76 year old woman with prior R frontotemporal stroke who presented with right hemisphere AIS, NIHSS 23, and received tPA at 2:10 hrs after symptom onset. At 1 hr, she had improved to NIHSS 11, and was admitted to the NeuroICU for care. At 9.5 hrs after treatment, she became drowsy and was intubated; CT showed a right temporal ICH and SDH. She deteriorated to NIHSS 20; follow‐up CT showed enlargement of the ICH (with blood‐ fluid level) and SDH, with 5 mm right to left shift. The patient received 6 units of cryoprecipitate and 6 units of platelets. She underwent emergent right hemicraniectomy with good operative result. Despite vigorous neurocritical care management, she expired the next day. Conclusion: Combined ICH and SDH may rarely complicate tPA therapy for AIS. Outcome may be poor, similar to ICH and SDH following tPA for acute myocardial infarction. 9.  Delayed Cerebral Edema Following Parenchymal Intracerebral Hemorrhage Robert Malka 1 , Yamile Vidal 1 , Karen Wilson 2 , Reza Behrouz 1 , Michael Sloan 1 
 1 University of South Florida, Tampa, FL, United States, 2 Tampa General Hospital, Tampa, FL, United States Introduction: Parenchymal intracerebral hemorrhage (PIH) is typically accompanied by cerebral edema. Little information exists regarding the occurrence of cerebral edema more than 10 days after PIH onset. Patients (or Materials) and Methods:Case ReportsResults: We report two cases of late cerebral edema following PIH. Case 1: A 59 year old woman with prior strokes, antiphospholipid antibody syndrome, two prosthetic heart valves on Coumadin therapy developed a 13 cc L frontoparietal PIH with INR 4.5. After reversal of Coumadin, she improved. Between 11–14 days after PIH onset, she worsened; CT showed extensive edema, diffuse L hemisphere sulcal effacement and 5 mm right to left shift. With medical management and treatment for polymicrobial bacteremia, she markedly improved on day 26; CT showed marked reduction in edema. She was transferred to rehab four days later. Case 2: A 48 year old woman with hypertension, diabetes and end stage renal disease on hemodialysis presented with severe aphasia, right hemiparesis and required intubation; GCS = 9T and NIHSS = 20. CT showed a 50 cc L basal ganglia PIH with edema and 5 mm left to right shift. In the NeuroICU, she received Endotool, 3% saline. By day 8, she was extubated, had improved and was transferred to the floor. By day 18, she became drowsy and hypotensive and was transferred to the NeuroICU. CT showed diffuse cerebral edema and 12 mm left to right shift. Despite intubation and 3% saline, she herniated and received left hemicraniectomy. Postoperatively, she developed refractory intracranial hypertension and expired three days later. Conclusion: Late cerebral edema following PIH is uncommon and outcomes may vary. 10.  Hashimoto Encephalopathy with Neuroimaging Findings Mimicking Multiple Sclerosis Lucas Harmon Bradley, Aarti Sarwal, Upinder Dhand University of Missouri, Columbia, MO, United States Introduction: Hashimoto encephalopathy(HE) has varying clinical presentation with confusion, stroke‐like episodes, coma, seizures etc. Diffuse periventricular white matter changes with increased T2 and FLAIR signal on brain MRI are reported in 50% patients. Elevated CSF anti‐thyroid peroxidase(TPO) antibody is considered diagnostic, albeit not causally related. We report unique presentation of HE with dementia, relapsing neurological deficits, and MRI findings mimicking multiple sclerosis. Patients (or Materials) and Methods: Case ReportResults: 62‐year‐old male presented with progressive cognitive decline over four years, one episode of left side weakness, fluctuating paresthesias in extremities, urinary incontinence and fatigue. His forgetfulness and difficulty in complex tasks caused loss of job as satellite engineer. He was referred for evaluation of multiple sclerosis. Examination showed no motor, sensory or cranial nerve deficits. Neuropsychological assessment revealed academic ability at 6th grade level with impaired cognitive and executive function. Routine chemistry, B12, folate, TSH, paraneoplastic panel and electroencephalograph were normal. MRI brain showed multiple, non‐enhancing, hyperintense, bifrontal subcortical and posterior periventricular areas on T2 and FLAIR images. Serum antinuclear antibody(1:1240) and anti‐TPO antibody 153.7(0–3.9 IU/ml) were elevated. CSF examination showed normal chemistry, cytology, myelin basic protein, IgG synthesis rate, no oligoclonal bands. Elevated CSF anti‐TPO antibody at 977.6(0–60 IU/ml) confirmed diagnosis of HE. Conclusion: Our patient with chronic dementia, transient neurological deficits and multifocal white matter lesions on brain MRI associated with anti‐TPO antibody adds to the expanding clinical and radiological spectrum of demyelinating disease. HE should be considered in differential diagnosis of acute encephalopathy as well as chronic dementia of unobvious cause. 11.  An Unusual Presentation of Cerebral Venous Sinus Thrombosis Adrienne Keener, May Kim University of Southern California/Keck School of Medicine, Los Angeles, CA, United States Introduction: Thromboembolic disease is a well‐established complication of ulcerative colitis (UC), but cerebral venous sinus thrombosis is an extremely rare occurrence. Methods: A case report of a young female admitted to Los Angeles County Hospital in late June of 2009 with review of the medical literature. Results: A 39 year old right handed Hispanic female with no known past medical history was seen in the emergency department (ED) following new‐onset seizures, fever, and stiff neck. Upon interview, she was confused and disoriented with a non‐focal neurologic exam. A lumbar puncture was performed which was normal and a non‐contrast head computed tomography (HCT) scan showed a right posterior parietal hypodensity with no surrounding edema. She was empirically treated with acyclovir in the ED and admitted to the neurology service. Subsequently, a recent history of rectal bleeding was elicited from the patient one week prior to seizure onset. A colonoscopy with biopsy demonstrating ulcerative colitis was discovered during her admission. Magnetic resonance imaging/magnetic resonance venogram (MRI/MRV) revealed an impressive thrombosis of the right transverse and sigmoid sinuses extending down into the internal jugular vein with a venous infarct of the right posterior temporal lobe. A hypercoagulable work‐up was unremarkable. Conclusion: The underlying etiology of venous thromboembolic disease in the setting of ulcerative colitis is poorly understood. The unique presentation of this case emphasizes the need to maintain high clinical suspicion of cerebral venous thrombosis in patients with UC who present with acute neurologic symptoms and signs. 12.  Age and Insulin Resistance are Associated with Periventricular White Matter Hyperintensities in older HIV Seropositive Individuals Aaron McMurtray Pacific Health Research Institute, Honolulu, HI, United States Introduction: Human immunodeficiency virus infection is associated with the development of insulin resistance. This study was conducted to determine the relationship between presence of white matter hyperintensities (WMHs) and insulin resistance in older HIV‐seropositive individuals. Patients and Methods: Twenty‐six HIV seropositive individuals with age greater than 50 years were prospectively enrolled in the study. Participants underwent general physical and neurological examinations, oral glucose tolerance testing, laboratory blood analysis, and either or both of 3‐Tesla Magnetic Resonance Imaging (MRI) and/or Positron Emission Tomography (PET) imaging of the brain. Presence of WMHs was determined by visual analysis of T2 and FLAIR weighted images. Voxel‐based‐morphometry (VBM) was used to compare cortical lobar volumes between groups and statistical parametric mapping (SPM) was used to compare cortical metabolism between groups. Results: Presence of WMHs was significantly associated with greater age (p = 0.043), and insulin resistance (p = 0.05). A trend towards greater history of pack‐years smoking (p = 0.086) was noted as well. Other demographic and HIV related factors did not significantly differ between the groups. Cortical lobar volumes did not significantly differ between the groups and statistical parametric mapping did not detect any significant differences in cortical metabolism between groups as well. Conclusion: Age and insulin resistance are associated with presence of periventricular WMHs in older individuals living with HIV infection. These findings may be of particular interest to clinicians caring for older HIV seropositive individuals. 13.  Dissociable Left‐Right Mammillothalamic Functional Connectivity for Verbal and Nonverbal Memory in Wernicke Encephalopathy Young‐Chul Jung Yonsei University, College of Medicine, Seoul, Korea, Republic of Introduction: Wernicke's encephalopathy is an acute, neuropsychiatric disorder caused by vitamin B1 deficiency. We aimed to validate the clinical validity of resting‐state functional connectivity analysis in a patient who suffered from two independent episodes of Wernicke's encephalopathy during a 20 month follow‐up. Patients and Methods: We repeatedly measured the mamillothalamic functional connectivity and memory function three times in a 68 year‐old male patient: First, when the patient completely recovered from the first episode of Wernicke's encephalopathy (month 0); Second, when the patient readmitted due to relapse of Wernicke's encephalopathy (month 17); Third, when the patient partially recovered from the second episode of Wernicke's encephalopathy after three months of hospitalization (month 20). Functional images were acquired on 1.5 T GE scanner and were collected by using a gradient echo EPI sequence, while the subject underwent a 5 minute passive‐viewing block scan, instructed to fixate on a white crosshair in the center of a black background screen The memory function was evaluated by the Auditory Verbal Learning Test and the Rey Complex Figure Test. Results: The left side mammillothalamic functional connectivity strength paralleled with the verbal memory function. Both demonstrated significant impairment at month 17 and significant restoration at month 20. In contrast, the right side mammillothalamic functional connectivity strength and the nonverbal memory function did not show any accordance during the clinical course. Conclusion: The current findings support the feasibility of mammillothalamic functional connectivity analysis in clinical settings to evaluate treatment response in patients recovering from Wernicke's encephalopathy. 14.  Central Pontine and Extra‐Pontine Myelinolysis in a Patient with Prolonged Hyponatermia Due to Oxcarbazepine Treatment Ziad Darkhabani, Bijal Mehta, Tareq Kass‐Hout, Maxim Mokin, Cornelia Mihai SUNY Buffalo, Buffalo, NY, United States Background: Central pontine myelinolysis (CPM), also known as osmotic demyelination syndrome, was first described in 1959. The most common etiology involves osmotic changes within the pons and extra‐pontine areas while rapidly treating hyponatermia. However, prolonged hyponatremia and fluctuating osmotic forces is also a risk for demyelination. The causes of hyponatermia can vary as well. Case: We report a novel case of a patient with multiple sclerosis who presented with mild bulbar symptoms. She was also found to have chronic hyponatremia related to Oxcarbazepine treatment, which she was taking for pain management. Magnetic resonance imaging (MRI) findings were consistent with central pontine myelinolysis (CPM) and extra‐pontine myelinolysis (EPM). Subsequent MRI findings showed significant resolution of the CPM/EPM changes on a 3 month follow‐up MRI after discontinuing the Oxcarbamazepine and treating the hyponatremia. Discussion: CPM/EPM is a serious side effect secondary to fluctuations in serum sodium levels and chronic hyponatremia. The classical presentation of CPM is “ Locked‐in Syndrome,” however severity of symptoms may vary and can be asymptomatic. MRI findings can also vary from minimal T2/FLAIR changes within the pons to larger areas encompassing the entire pons and surrounding extra‐pontine structures. Many antiepileptic medications, including Oxcarbazepine, are well known to cause hyponatremia. This case report showed that the Oxcarbazepine‐induced hyponatremia may lead to CPM and EPM, as noted on MRI, if sodium levels are not properly monitored and corrected. Conclusion: Oxcarbazepine is an effective antiepileptic and mood stabilizing medication, but proper attention and monitoring is required in order to prevent fluctuations in serum sodium levels. Appropriate caution should be used when treating patients with Oxcarbazepine, especially for off label indications. 15.  Susac's Syndrome: Evolution of MRI Findings Following Treatment with Immune‐Modulating Therapy Lama Chahine, Esteban Cheng, Ferdinand Hui, Jinny Tavee Cleveland Clinic, Cleveland, OH, United States Introduction: Susac's Syndrome (SS) is an autoimmune endotheliopathy characterized by the triad of encephalopathy, retinal arteriopathy, and hearing loss, though all three features may not be present at disease onset. We report a case of SS and describe the clinical and MRI changes seen over time with immune‐modulating therapy. Patients (or Materials) Methods: The patient's medical record, laboratory tests, and imaging studies were reviewed. Results: A 24‐year‐old man presented with vertigo, vision loss, dysarthria, and mental status changes. MRI of the brain showed diffuse dural enhancement with multiple T2 and FLAIR hyperintensities in the central corpus callosum (CC), right cerebellar hemisphere, and periventricular white matter perpendicular to the CC. Ophthalmologic evaluation showed multiple retinal arteriolar occlusions. Cerebrospinal fluid analysis showed evidence of active inflammation with negative cultures and cytology. Although audiologic evaluation was normal, the diagnosis of SS was made based on the characteristic appearance of the corpus callosal lesion, retinal lesions, and clinical findings. After treatment with 2 gm/kg of intravenous (IV) immunoglobulins and IV methylprednisolone, there was marked radiographic and clinical improvement, with the patient returning to near baseline status. Conclusion: Susac's syndrome (SS) typically involves the cerebral, retinal, and inner ear microvasculature. Central CC involvement with sparing of the peripheral and inferior CC are classic features on MRI. Combined with auditory and visual findings, these MRI findings help distinguish SS from multiple sclerosis. The majority of patients have a monophasic course and respond well to immunotherapy, although relapses may occur. 16.  Global vs. Regional Spinal Cord Atrophy in Multiple Sclerosis Ashish Arora, Mohit Neema, Shahamat Tauhid, Daniel Goldberg‐Zimring, Brian Healy, James Stankiewicz, Christian Chavarro‐Nieto, Namitha Thomas, Antonia Ceccarelli, Elisa Dell’Oglio, Charles Guttmann, Rohit Bakshi 1 Brigham and Women's Hospital, Boston, United States, 2 Harvard Medical School, Boston, United States Introduction: Spinal cord damage is common in MS and provides a unique and clinically relevant means to monitor disease progression and therapeutic effects. While regional spinal cord atrophy has been studied extensively in MS, whole spinal cord atrophy has not been previously assessed. Patients (or Materials) and Methods: Whole spinal cord 3 mm thick T2‐weighted axial fast spin‐echo MRI images were obtained at 3 T on 34 patients with MS [EDSS 1.95 ±1.72, range 0–6.5] and 15 normal controls. The global and regional (cervical, thoracic) cord volumes and cross‐sectional volume at C2–3 were determined using a semi‐automated tool and were normalized by the segment length and intracranial volume. Results: Whole cord, C2–3, cervical and thoracic volumes were lower in progressive vs. relapsing patients or normal controls (all p < 0.05). Spinal cord volumes were not related to spinal cord lesion load but were related to EDSS scores (r −0.35 to −0.46; p < 0.05). Only the C2–3 volume correlated with timed 25‐foot walk (r ‐0.40; p < 0.05). The regional and whole cord volumes were all highly inter‐correlated (r = 0.83 to 0.98; p < 0.05). Conclusion: The established method of estimating spinal cord atrophy, C2–3 cross‐sectional volume, provides a useful surrogate of overall global or regional spinal cord atrophy. Spinal cord atrophy is most common in progressive forms of MS and is related to neurologic disability. 17.  FLAIR Vascular Hyperintensities on Baseline Imaging of a Transplant Candidate Sharmineh Shams, Kai Lin, Nerses Sanossian University of Southern California, Los Angeles, CA, United States Background: Fluid‐Attenuated‐Inversion‐Recovery Vascular Hyperintensity (FVH) are well described in the setting of acute stroke, but may occur in chronic arterial disease. The presence of FVH may indicate a high risk of stroke, especially in the face of a stressor such as surgery. We describe FVH noted during the pre‐surgical evaluation of a renal transplant patient to highlight how this neuro‐imaging marker may provide additional information about stroke risk. Methods: Case Report Results: A 40 year old man with multiple vascular risk factors including diabetes, hypertension, dyslipidemia and previous stroke for preoperative stroke evaluation. He denies any recent episodes of neurological symptoms. The patient was being evaluated for had been on dialysis for many years and was considered having renal transplantation. His neurological examination was significant only for some difficulty with frontal executive function, and sensory polyneuropathy. His brain CT scan showed an old left frontal infarction and extensive calcific atherosclerosis. The MRI of the brain revealed border zone infarctions predominantly in the left side basal ganglia lacunes. FLAIR images revealed extensive FVH in the bilateral insula and fronto‐parietal lobes. The MR angiogram of the brain was consistent with cerebral atherosclerosis. Conclusion: FVH are seen in the setting of disordered flow, for example in the setting of leptomeningeal collaterals. They are often transient in acute stroke because of progression to infarction or re‐establishment of normal flow patterns. The presence of chronic FVH may be an indicator of severe flow disturbance and high risk of stroke. 18.  Transient Ischemic Attacks in a Young Female with Takayasu's Arteritis Mary Catherine Mayo, May Kim University of Southern California, Los Angeles, CA, United States Introduction: Takayasu's arteritis (TA) is a large vessel vasculitis primarily affecting the aorta and its proximal branches and commonly occurring in Asian females aged 15–35 years. The mechanism of stroke and Transient Ischemic Attacks (TIA) in TA is likely due to decreases or shifts in blood flow secondary to steno‐occlusive disease and/or thromboembolism exacerbated by hypertension. Patients (or Materials) and Methods: We present a 37 year old Hispanic female with history of TA diagnosed two years prior to presentation with ten episodes of right amarousis fugaux lasting 10–60 seconds as well as one episode of left sided weakness and dysarthria lasting several minutes. She had no residual symptoms after the episodes and her symptoms were consistent with a TIA. She received MR imaging of her brain and neck and was referred to cardiothoracic surgery. Results: MR imaging of the neck demonstrated a complete occlusion of the left subclavian artery and bilateral common carotid arteries. MR imaging of the brain revealed no evidence of acute infarction on diffusion weighted imaging. Blood supply to her brain came from the right vertebral artery and from collateral flow to the left side. Conclusion: This patient underwent surgical replacement of the ascending aorta and aortic valve as well as a carotid bifurcation graft. She has continued treatment with steroids for her TA and is doing well as an outpatient without recurrent TIA symptoms. This patient represents a dramatic case of occlusive disease in TA causing TIA, and demonstrates the benefit of surgical intervention. 19.  Reversible Vasoconstriction in a Migraineur Possibly Triggered by Phentermine Elliot Hogg 1 , Andres Gonzalez 2 , Nerses Sanossian 2 
 1 Keck School of Medicine, Los Angeles, CA, United States, 2 Department of Neurology, University of Southern California, Los Angeles, CA, United States Introduction: Reversible Cerebral Vasoconstriction Syndrome (RCVS) is characterized by multi‐vessel stenosis/occlusion leading to ischemic stroke. We present a report of a young woman using the appetite suppressant phentermine who was initially misdiagnosed and untreated. The case demonstrates the importance of considering RCVS in the setting of atypical headache, and the role of neuroimaging in prompt diagnosis. Patients (or Materials) and Methods: Case Report Results: A 33 year old woman migraineur presented with severe atypical headache and right‐sided facial numbness. Seven days after discharge she returned to the ER with aphasia and right‐sided hemiparesis. A secondary hospital's MRI revealed an acute left anterior cerebral artery (ACA) stroke. LP was unremarkable. She was treated with clopidogrel and Vicodin. After her condition worsened she was anticoagulated. An MRI and catheter angiography demonstrated multi‐vessel stenosis and left subclinoid ICA occlusion leading to a diagnosis of vasculitis and treatment with intravenous steroid. She was transferred for tertiary care with a misdiagnosis of Moyamoya disease. On transfer additional history indicated premorbid use of oral contraceptives, sumatriptan, promethazine and phentermine‐based diet pills. Treatment was begun in the ICU with a standard vasospasm protocol of hypervolumia with normal saline, and hypertension with dopamine and nimodipine. High dose magnesium oxide was given for its vasodilative effects. Over the next week, cerebral arterial velocities on transcranial Doppler paralleling clinical improvement. Conclusion: An environment of migraine‐related vascular hyperreactivity and phentermine use may have predisposed to RCVS. Multimodal neuroimaging, and an absence of vascular risk and inflammation, were keys to diagnosis. 20.  Primitive Neuroectodermal Tumor (PNET) of the CNS in a 73 Year Old Man: A Case Report
 Remia Paduga, Saud Khan University of Kansas Medical Center, Kansas City, MO, United States Introduction: PNET is primarily a childhood neoplasm, it is histologically similar to medulloblastoma but occur in locations other than the cerebellum. It is very rare, esp in adults. We report a 73 y/o man who presented with PNET. Patients (or Materials) and Methods: The patient has been previously healthy until he developed tremors followed by a rapid decline in his memory and cognition. He became weak in his legs, lost significant weight in a span of three months. He had an extensive imaging study of his neuraxis which showed diffuse meningeal enhancement. Results: He had a tenuous and one month long work‐up which includes basic and elaborate rheumatologic, infectious, metabolic, oncologic and toxic investigations. His whole body was imaged for presumed CNS metastases. He had four serial lumbar taps. He had numerous biopsies namely, thyroid, skin (3 sites), meninges and colon (2 sites). His fourth lumbar tap showed malignant cells and biopsies of the sites mentioned above turned out negative except for the meninges. After numerous histopathologic stains and markers of the meninges and review of his images, he was finally diagnosed with PNET of the CNS. Patient and family then decided to pursue palliative care as his prognosis is dim. Conclusion: Since 1987 only 24 cases of CNS PNET in adults have been reported in the literature, 21 supratentorial cases, 2 cases localized in the spinal cord and 1 case in the posterior fossa. These included 12 men and 8 women with age ranged from 7–69. Our patient will be the 25th case and the oldest so far seen. 21.  Superficial Siderosis of the CNS: Clinical and Imaging Features Lama Chahine, Alexander Rae‐Grant Cleveland Clinic, Cleveland, Ohio, United States Introduction: Superficial Siderosis (SS) of the CNS is a rare disorder characterized by repeated microhemorrhages into the subarachnoid space with resultant subpial cerebral and spinal hemosiderin deposition. We present a case of SS and discuss the imaging features. Patients (or Materials) and Methods: patient's medical record, laboratory tests, and imaging studies were reviewed. Results: 49 year old male presented with subacute bilateral sensorineural hearing loss (SNHL). MRI showed diffuse hypointense signal along the surface of the inferior aspect of the cerebellum, brain stem, and spinal cord. Lumbar puncture showed xanthochromia. Cerebral and spinal angiograms did not show any source of hemorrhage. 9 years later, he had developed leg stiffness, gait instability, and dysarthria. Physical examination showed decreased auditory acuity, severe dysarthria, normal motor power in the upper extremities, and no antigravity power in the lower extremities with bilateral spasticity. MRI showed thoracic spine cyst that was decompressed. Spasticity, dysarthria, and spastic paraparesis progressed; at last follow‐up he is no longer able to ambulate. Conclusion: SS of the CNS is caused by recurrent subarachnoid hemorrhages (SAH) with accumulation of hemosiderin and iron‐containing pigments in the leptomeninges, brain surface, brainstem, cerebellum, cranial nerves, and spinal cord. The cause of hemorrhage can not always be identified but in some cases there may be dural pathology or tumors. Classic clinical features include progressive SNHL, cerebellar ataxia, pyramidal signs, ataxia, and headache. MRI findings are pathognomonic and include hypointense signal on the surface of the cerebral hemispheres, brainstem, cerebellum, and spinal cord. 22.  Selective Motion Perception Deficit Following Cerebellar Stroke Sharmineh Shams, Adrian Burgos, Nerses Sanossian University of Southern California, Los Angeles, CA, United States Background: Motion perception is inferring the speed and direction of objects based on visual, vestibular and proprioceptive inputs and is mediated by the cerebellum. We present a case where a cerebellar stroke produced motion perception deficits by disrupting visual‐motor integration mechanisms involved in perceptual stabilization. Methods: Case report Results: A 42 year‐old woman presents with a chief complain of trouble with her vision. She is able to see stationary objects but is not able to recognize them either she is moving or the object is. She is able to track a moving object, such as a finger with her eye, but as she is tracking it, if another moving object comes into her field of vision, she is not able to recognize and identify it. She experienced a subarachnoid hemorrhage secondary to posterior inferior cerebellar artery aneurysm about 9 years ago and received endovascular embolization of the PICA aneurysm. Neuroimaging revealed an infarction of the inferior midline cerebellum and an occluded aneurysm without any visualization of flow in the left PICA artery. Her symptoms were present since the embolization, without significant improvement, and had led to severe disability. Conclusions: The cerebellum contains representations for pursuit in the flocculus most likely involved in the coordination of pursuit and in the posterior vermis precise adjustments of the eye movements such as adaptation of pursuit initiation. Defective ocular motor control including the ability to track moving targets and motion direction discrimination can result from midline cerebellar stroke and lead to significant disability. 23.  Higher Prevalence of Silent Lacunes In Stroke Patients With Moderate and Severe Sleep Apnea Mohammed Al‐Amoodi 1 , Wahid Rashidzada 2 
 1 University of Missouri, Kansas City, MO, United States, 2 Headache and Pain Center, Leawood, KS, United States Introduction: We investigated the relationship between subclinical ischemic lesions and obstructive sleep apnea (OSA) in a population of patients presenting to the hospital with stroke. We examined the association of OSA with the prevalence of silent lacunar infarction, periventricular hyperintensity (PVH), and cerebrovascular ischemic disease (CVID). Patients (or Materials) and Methods: 65 subjects presented to our hospital with a stroke. Three types of silent lesions were assessed by MRI. OSA was assessed using ResMed ApneaLink. Cross‐sectional study was performed on OSA severity and the prevalence of silent cerebrovascular lesions detected by brain MRI. Data were subdivided into 4 levels of OSA severity; None, Mild, Moderate, and Severe based on measured respiratory disturbance index (RDI) values. Data was collapsed into a low RDI (None and Mild) group and a high RDI (Moderate and Severe). Results: Independent samples t‐tests revealed that there was a statistically significant difference between low and high RDI patients on mean PVH (p < 0.001), and number of Lacunes (p = 0.001). Chi‐square revealed a difference between low and high RDI in the CVID (p < 0.001) with a correlation of r = 0.498 between level of RDI and CVID. Conclusion: Results indicate that stroke patients with moderate to severe OSA (RDI greater or equal to 15/h) have a higher prevalence of silent lacunar infarctions, periventricular hyperintensity and cerebrovascular ischemic disease than stroke patients with less OSA (RDI less than 15/h). 24.  Quantitative Analysis of MRI for Evaluation of Brain Iron Deposition in Alzheimer Disease Yuri Suleimanov 1 , Sergiy Radchenko 2 , Vasyl Sava 1 , Juan Sanchez‐Ramos 1 , Leon Prockop 1 
 1 University of South Florida, Tampa, United States, 2 National Taras Shevchenko University, Kyiv, Ukraine Introduction: The increased iron deposition in brain associated with shortening of T2 relaxation times on MRI can be used to track the development of AD. Recently, high field MRI was employed (Schenk et al., 2006) as a trustworthy method for iron assessment in brain. However, 3 T MRI is not always available. In our study we offer a novel method based on routine 1.5 T MRI protocols. Patients and Methods: Brain imaging protocols were developed and applied to 42 MCI and AD patients and 20 age‐matched controls using 1.5 T MRI. Dual spin echo images with TR = 2.666 sec; TE = 30 & 92 msec; FOV = 22 × 22 cm; matrix 256 × 256 and slice thickness of 4 mm were acquired on 1.5 T scanner (General Electric, Waukesha, WI). Results: The region of interest (ROI) was the corpus striatum (putamen and globus pallidus). It was segmented on coronal slices and T2‐weighted images were computed to generate series of contour lines representing equal T2 values for hypothetical surfaces containing multiple planes. Computer analysis involved the selection of base contour representing identical T2 value in all slices. The iron indices were computed by combining data obtained from sequential slices within ROI. Total iron index was plotted against diagnosis of normal, MCI and AD. One‐way ANOVA revealed significant differences (P < 0.05) between three groups. Conclusion: Standard 1.5 T MRI can be used for quantitative determination of iron accumulation in both MCI and AD subjects and may serve as a biomarker of disease progression. 25.  MRI (DWI, T2, Flair Sequences) Findings of an Acute Ischemic Stroke in a 61 Year Old Patient Taken at Least 30 Minutes BEFORE Symptom Onset: A Case Report Remia Paduga, Gary Gronseth University of Kansas Medical Center, Kansas City, MO, United States Introduction: MRI DWI sequence has been reported in the literature to detect ischemic changes in the brain as early as 3–5 minutes of symptom onset. Here we report a case of a 61 year old veteran patient who demonstrated clinical stroke shortly after his MRI was taken. Patients (or Materials) and Methods: A 61 year old patient with a small cell cancer of the lung was seen with multiple neurological complaints of four month duration that comes and goes consistent with a paraneoplastic process. An MRI of the head was recommended by the neurology consult team performed a day after he received chemotherapeutic agents. His MRI showed acute stroke in the left perirolandic cortical area and posterior MCA distribution in the T2, flair and DWI sequences. After the MRI was taken, patient went to the parking lot to smoke and was seen by family members a few minutes later slumped on the wheelchair with complete right sided weakness and burnt fingers from smoldering cigarette butt. Results: The patient was given IV TPA within 3 hours from the last time he was seen normal and had marked improvement in his dysarthria, facial droop and dense hemiplegia. He had a post ictal CT scan done six days after which showed subtle hypodense areas in the left MCA distribution. His paraneoplastic process turned out to be a Lambert‐Eaton myasthenic syndrome which is another rare entity. Conclusion: MRI (DWI, T2, Flair sequences) findings in an ischemic stroke can antedate symptom onset. There has been no case report of similar event in the literature. 26.  A Severe Case of Bilateral Striatopallidodentate Calcinosis, Treated with a Dopamine Agonist – A Case Report Sara Khan, Jinny Tavee Cleveland Clinic, Cleveland, OH, United States Introduction: Bilateral symmetric striatopallidodentate calcinosis (BSPDC), commonly known as Fahr's disease is a progressive neurodegenerative disorder that presents with neuropsychiatric and extrapyramidal symptoms. As there is no known treatment that limits calcium production or deposition in the brain, most therapeutic modalities are aimed at symptomatic relief. We report a case of severe BSPDC which symptomatically responded to Ropinirole. Patients (or Materials) and Methods: A 36‐year‐old female presented with progressive slurred speech, gait imbalance, choreoathetosis, blephrospasm, mood lability and cognitive decline. Exam was significant for frontal lobe signs, upper extremity dysmetria, dysarthric speech, ataxic gait, hyperreflexia and choreoathetosis. Results: CT and MRI brain demonstrated diffuse symmetric calcium deposition in bilateral basal ganglia, thalamic, subcortical, cerebellar, and central pontine regions. Extensive metabolic and genetic testing were negative. Wilson's disease, parathyroid, thyroid, calcium metabolism and infectious disorders were also excluded. The patient was treated with Ropinirole, a dopamine agonist, with marked improvement in speech and limb ataxia. Conclusion: Idiopathic BSPD calcification or Fahr's disease is a rare disorder that clinically manifests as a spectrum of movement disorders and seizures. CT remains the most effective screening tool. While varying signal intensities on MRI may correlate with different stages of the disease, it remains unclear whether or not the severity of symptoms corresponds with the degree of calcification. Part of the symptom complex may be attributed to reduced levels of dopamine with in the basal ganglia secondary to calcification. Our patient's symptoms responded to a dopamine agonist, perhaps identifying a class of drugs which can be used for the management of severe Fahr's disease. Much work still needs to be done to define neuroprotective agents to prevent or reverse calcification. 27.  Multimodal Neuroimaging in Pediatric Stroke Extends the Treatment Window: A Case Report Waimei Tai, Nerses Sanossian, Arthur Partikian University of Southern California, Los Angeles, CA, United States Multimodal Neuroimaging in Pediatric Stroke Extends the Treatment Window: A Case ReportIntroduction: Pediatric stroke is rare and difficult to diagnose. Given the devastating neurologic sequelea of ischemic stroke in children, it is important to recognize and treat aggressively. Recent clinical trials in adults have demonstrated efficacy of intravenous TPA up to 4.5 hours after onset and smaller series have described efficacy up to 6 hours using neuroimaging. We report the case of a 13 year‐old boy in whom neuroimaging led to use of intravenous TPA five hours after symptom onset. Patient: A 13 year old boy was found down, alert, but unable to speak or move his right side by teachers during a field trip with a last know well time (LKWT) of 09:30. He was helicopter‐transported for urgent evaluation by the neurology team at 11:30. Initial exam revealed right homonymous hemianopia, severe dysarthria, expressive aphasia, right hemiplegia and sensory loss with an initial NHISS of 23. Non‐contrast head CT was unremarkable and was followed by urgent MRI/A with perfusion‐weighted imaging. MR demonstrated diffusion‐perfusion mismatch of >50% and lack of flow signal in the left intracranial carotid on MRA. The decision for treatment with IV TPA was made and a 0.9 mg/kg dose was administered at 4.75 hours after LKWT. Repeat head CT in 24 hours showed no hemorrhage and patient's NIHSS was 12. During hospitalization he was found to have a hypercoagulable condition and he continued to improve to an NIHSS of 7. Conclusion: Multimodal neuroimaging can help diagnose and treat pediatric stroke outside of previously established time windows. 28.  Retrospective Case Series of Reversible Posterior Leukoencephalopathy: Typical and Atypical Etiologies and Neuroimaging Features Vikram Penumalli, Kelly Blend, Kristen Gruenther, Patrick Capone Winchester Neuroimaging, Virginia Commonwealth University, Winchester, VA, United States Introduction: Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical and neuroimaging diagnosis that is commonly seen in the setting of uncontrolled hypertension, ecclampsia, renal failure or immunosuppressive therapy. We present a case series of patients diagnosed with RPLS at a tertiary care hospital and review the clinical presentation, medical comorbidities and neuroimaging findings of these patients. Patients (or Materials) and Methods: 16 patients with RPLS were collected retrospectively over the past 9 years. Hospital charts and neuroimaging findings were reviewed. Results: We identified 16 patients with RPLS (14 females and 2 males) with a mean age of 43.6 years (range 20 – 67 years). Associated medical conditions included 3 patients undergoing narcotic withdrawal which to our knowledge has not been described, 1 patient with Guillain‐Barre syndrome, and previous well characterized etiologies such as ecclampsia, renal insufficiency, hypertensive emergency, and immunosuppressive agents. Atypical neuroimaging features included hemorrhage, cortical lesions, and frontal lobe edema. Conclusion: RPLS is a heterogenous clinical and neuroimaging diagnosis that can be seen with a variety of medical comorbidities leading to a failure of cerebral autoregulation and resulting in variable patterns of cerebral edema. Recognition of the spectrum of associated medical comorbidities and neuroimaging findings is the key to making this diagnosis which usually has a favourable prognosis. 29.  A Case of Familial Creutzfeldt‐Jakob Disease Presenting as an Acute Ischemic Stroke Esteban Cheng‐Ching, Sara Khan, Muhammad S. Hussain, Jinny Tavee The Cleveland Clinic Foundation, Cleveland, OH, United States Introduction: Creutzfeldt‐Jakob Disease (CJD) is a fatal condition characterized by a rapidly progressive dementia and neurological decline. We report a case of familial CJD presenting with stroke‐like episodes and preserved cognition that manifested as evolving areas of restricted diffusion on serial MRI studies. Patients (or Materials) and Methods: We reviewed the patient's medical record, laboratory tests and imaging studies. Results: 60‐year‐old woman presented with sudden onset of right upper extremity weakness. On examination, she had focal proximal weakness, increased tone, and brisk reflexes in her right arm. The first MRI demonstrated restricted diffusion in the left caudate. A month later, the patient developed acute right leg weakness. Followup MRI showed restricted diffusion in the left medial frontal and frontoparietal cortex suggestive of embolic infarcts, although the ADC map showed no abnormality. This prompted a CSF analysis, which demonstrated a positive 14–3‐3 protein. One month later, the patient developed quadriparesis and diffuse myoclonic jerks with intact cognition. She expired shortly afterwards. Autopsy confirmed spongiform changes of the brain. It was later discovered that her father had also died of autopsy‐confirmed CJD although he had presented with dementia. Conclusion: Familial CJD is typically characterized by a rapidly progressive encephalopathy of earlier onset and more protracted clinical course than sporadic CJD. Although stroke‐like progression has occasionally been seen with the sporadic variant, it has never been described in familial CJD. This case is the first description of familial CJD presenting as multiple stroke‐like episodes. 30.  Magnetic Resonance Imaging of Corticothalamic Connectivity in a Case of Focal Status Epilepticus Vikram Penumalli, Radoslav Raychev, M. Kelly Nicholas, Fernando Goldenberg, James Tao Department of Neurology, University of Chicago Medical Center, Chicago, IL, United States Introduction: Transient periictal neuroimaging findings such as cortical edema and gyriform enhancement in the setting of status epilepticus have been well described and are thought to be the result of neurovascular coupling, hyperperfusion and glutamate excitotoxicity involving predominantly cortical structures. In this case of focal status epilepticus, a less common pattern of restricted diffusion in the thalamus as well as the cortical ribbon is described and highlights the role of corticothalamic pathways in focal seizure disorders. Patients (or Materials) and Methods: Case presentation of a 68 year old female with focal status epilepticus treated in a neurointensive care unit. Results: A 68 year old female had an unwitnessed fall at home and was found to have a left hip fracture. During her emergency room evaluation, her exam showed left gaze preference and left upper extremity weakness. The neurology consult team witnessed a focal seizure involving forced left gaze deviation and left sided myoclonus. EEG revealed focal electrographic status epilepticus involving the right posterior quadrant. MRI demonstrated areas of restricted diffusion involving the right temporo‐occipital cortex and right posterior thalamus that mimicked a right posterior cerebral artery infarction. Lumbar puncture revealed concomitant non‐herpetic viral meningitis. After treatment of focal status epilepticus, the patient had follow‐up neuroimaging which demonstrated near complete resolution of the lesions, suggesting that these were the result of excessive seizure activity rather than the cause of it. Conclusions: 1. The pattern of transient cortical and subcortical lesions seen in this case demonstrates that corticothalamic pathways may be involved in reinforcement and propagation in focal seizure disorders. 2. Periictal neuroimaging in the setting of status epilepticus may reveal a variety of transient lesions that may mimic stroke, tumor or infection. Accurate differentiation of these entities is important when attempting to predict a patient's neurological prognosis.1[ Anatomic MRI (A&B) and fiber tracking images (C&D) showed that rat model (B&D) of spinal cord chronic compression was successfully established to mimic the disruption of spinal cord tract continuity (Arrow), which was observed in patients of cervical myelopathy (A&C). ] 31.  Classic ‘Open Ring Sign’ Not Necessary for Diagnosis of Tumefactive Demyelination Johanna Morton, Daniel Ontaneda, Steven Shook Cleveland Clinic, Cleveland, OH, United States Introduction: The diagnosis of tumefactive multiple sclerosis (MS) is generally defined as demyelinating lesions greater than 2 cm in diameter. These lesions create a diagnostic challenge due to their infrequent presentation and presence in multiple demyelinating disorders. Often, an ‘open ring sign’ on post‐contrast T1 weighted imaging aids in the diagnosis of tumefative demyelination. Patients (or Materials) and Methods: A 30 year‐old right‐handed female presented with progressive left‐sided weakness after sustaining a fall. She has a history of being diagnosed with a ‘demyelinating condition’ at age 12 that presented with visual loss and right hemiparesis. Her diagnosis was reportedly confirmed by brain biopsy at that time. Eighteen years later, without interim followup, she presented with the above picture. Results: MRI showed extensive bilateral periventricular deep white matter and subcortical T2 hyperintense lesions with T1 hypointensity. Many of the lesions demonstrated ring enhancement, the largest consistent with a closed‐ring appearance. Several large necrotic white matter lesions were suggestive of a long‐standing demyelinating process. Conclusion: Although the ‘open ring sign’ is classically characteristic of demyelinating disease, the presence of an enhancing closed ring should not preclude the diagnosis of demyelination. Often such a closed ring of enhancement is thought to represent other pathologies, such as lymphoma, neoplasm, or infectious etiologies. The pattern of enhancement, either open or closed ring, should not be used as the sole predictor of etiology in enhancing lesions. 32.  MRI Findings in Cryptococcus Meningitis Rani Sarkis, Maryann Mays Cleveland Clinic, Cleveland, OH, United States Introduction: Cryptococcus meningitis is an opportunistic infection affecting predominantly immunosuppressed patients. MRI findings in the HIV population have been reported in the literature however little has been reported about the non‐HIV population. Patients (or Materials) and Methods: A retrospective chart review of MRIs of the brain of patients with cryptococcal meningitis (based on positive CSF) over a ten year period was performed. All MRIs performed during the active phase of the disease were included. Results: 35 patients with cryptococcal meningitis were identified. All except one patient received contrast for their MRIs. Twenty seven of the patients were males, and mean age at presentation was 52 years. Clinically, 14 were HIV positive, 8 were being actively treated for cancer, 5 were transplant patients, four had idiopathic CD4 lymphocytopenia, two had hepatitis C cirrhosis, and two had systemic lupus erythematosus. MRI findings in the HIV subgroup included meningeal enhancement (43%), ventriculomegaly (14%), strokes (14%), and abscesses (14%). MRI findings in the non‐HIV group included meningeal enhancement (57%), ventriculomegaly (33%), intracranial hemorrhage (14%), pseudocysts (14%), and strokes (9.5%). One patient also developed sinus venous thrombosis. Ten patients had otherwise non specific findings on MRI. Conclusion: MRI is an important tool in assessing patients with cryptococccal meningitis and helps in the diagnosis of complications of this disease which include abscesses, hemorrhages, sinus thromboses, and strokes both in the HIV and non‐HIV population. 33.  Multiple Bio‐imaging Evaluations of Spinal Cord Chronic Compression in Rat C.Y. Wen, Y. Hu, T.H. Li, K.O. Lam, Ed. X. Wu, D.K. Luk 1 Department of Orthopaedics and Traumatology, the University of Hong Kong, Hong Kong, Hong Kong, 2 Department of Electrical and Electronic Engineering, the University of Hong Kong, Hong Kong, Hong Kong Aims: Spinal cord chronic compression‐induced cervical myelopathy is a common cause of spinal cord dysfunction. The disease generally leads to impairment of the sensory and motor function of the cord progressively. However, the precise mechanism and the underlying pathophysiology of myelopathy remain under investigated [1–3]. The establishment of animal model which reproduce the clinical condition of cervical myelopathy would be helpful for better understanding to the disease [3]. This study aims to establish a rat model of spinal cord chronic compression. MethodsAnimal model Total 15 adult Sprague‐Dawley rats were used. After general anaesthesia, the laminae of C3‐C7 were exposed and a small space around facet was opened. A water‐absorbing urethane‐containing polymer, which would expand upon absorbing tissue fluid, was inserted into the spinal canal at lateral side of the C5‐C6 region. Post‐operative evaluationMagnetic Resonance Imaging: Axial T2W and diffusion tensor images (DTI) of the C3–7 spinal cord were acquired in vivo using a 7 T Bruker PharmaScan 70/16 scanner. Respiration gated 4‐shot SE‐EPI sequence with navigator echo was used with the following parameters: TR/TE = 3000/29 ms, δ/Δ= 3.5/17 ms, slice thickness = 2 mm with 0.2 mm inter‐slice gap, FOV = 30 mm, data matrix = 128 × 128, NEX = 4. Diffusion encoded gradients with b = 0.8 ms/μm 2 were applied along 30 directions. Fiber tracking were performed by DTI Studio with FA threshold = 0.2, tracking was stopped if FA <0.15 or turning angle >450. Micro‐Computed Tomography: After harvest of rat spinal cord, the specimens were processed for micro‐CT scanning at the isotropic pixel size of 9 μm (Skyscan‐1076, Belgium). The 3D images of spinal cord were reconstructed. Histology : rat spinal cord was embedded in wax and sectioned to 8 μm for H&E and luxol fast blue staining. Results: Micro‐CT and DTI images demonstrated the distortion of white matter of spinal cord and disruption of spinal tract continuity. Under histological evaluations, the decreased number of neuron, increased blood vessels with loosely organized spinal cord after chronic compression were found after chronic compression. Conclusion: This is the first study to successfully establish a rat model of spinal cord chronic compression to mimic clinical scenario of cervical myelopathy. 34.  A Rapidly Enlarging Pontine Perivascular Space May Kim, Meng Law University of Southern California, Los Angeles, CA, United States Introduction: Perivascular spaces (PVS) are pial lined structures that are filled with interstitial fluid. Fluid changes between the vessel and pia, or within the interpial space, may cause the PVS to enlarge. Normal PVS that expand may exert mass effect and therefore neurologic impairment. It is unclear how quickly these spaces can enlarge. Patients and Methods: We report a case of a 60 year old male with a two week history of progressive right hand and foot numbness, tingling, and diplopia. Magnetic resonance imaging (MRI) was performed and reviewed with a neuroradiologist. Results: MRI of the brain with and without contrast revealed a T2 hyperintense round cystic appearing lesion in the pons measuring 15.3 mm by 11.5 mm. A second MRI was performed 2 weeks later which revealed a larger T2 hyperintense cystic lesion measuring 18.5 mm by 14.6 mm. A tiny artery was seen within the cystic lesion on both MRI's. Gradient echo imaging did not reveal any blood products within the lesion. The MRA and diffusion sequences were unremarkable. Conclusion: The mechanisms by which PVS enlarge are unknown. The time in which a PVS enlarges is also unknown. PVS with cystic components should be differentiated from cystic neoplasms and other CNS diseases using multi‐modal imaging with MRI and MR spectroscopy (MRS). It is important to closely follow patients with PVS, as mass effect due to rapidly enlarging lesions may require neurosurgical intervention.2[ Micro‐CT images show the distortion of gray and white matter of rat spinal cord. ]3[ The representative images show the intact (left) and compression (right) part of rat spinal cord (H&E, luxol fast blue staining). ] 35.  Blood‐Brain Barrier Disruption is Involved in Seizure and Hemianopsia in Nonketotic Hyperglycemia Dong Wook Kim, Jeeyoung Oh, Hong Gi Roh, Jin Woo Choi Konkuk University Hospital, Seoul, Korea, Republic of Introduction: Nonketotic hyperglycemia (NKH) is a clinical syndrome consisting of severe hyperglycemia, hyperosmolarity and intracellular dehydration without ketoacidosis. Diverse neurological symptoms have been described in patients with NKH, including hemichorea, seizure, hemianopsia and coma, but the pathogenic explanation for these neurological symptoms remains unclear. Patients (or Materials) and Methods: We performed serial MRI scans for a 65‐year‐old female presented with recurrent seizures and hemianopsia as presenting symptoms of NKH. MRI was obtained on the second hospital day, 15 days later, and two months later. Results: Initially, the T1 weighted images failed to demonstrate any abnormality, but focal cortical hyperintensity and subcortical hypointensity around the right parietal area was noted on T2 weighted images. Diffusion weighted imaging (DWI) demonstrated restricted diffusion in the right parietal area. Following intravenous gadolinium enhancement, minimal overlying CSF space enhancement around the right parietal area was noted. Delayed FLAIR images, obtained 12 hours later without additional gadolinium, showed prominent CSF space enhancement without parenchymal changes, which has been considered a marker of BBB disruption. The patient improved slowly, and MRI performed two weeks later revealed that T2 hypointensity and restricted diffusion became less intense and delayed CSF space enhancement was reduced. The patient became completely asymptomatic two months later, and follow‐up MRI at that time showed no abnormality with the disappearance of subcortical T2 hypointensity, restricted diffusion and delayed CSF space enhancement. Conclusion: we suggest reversible BBB disruption as a cause of hyperglycemia‐induced seizures and hemianopsia in NKH patients. 36.  Differentiation of Glioma Progression or Recurrence from Treatment‐Induced Changes Using a Combination of Diffusion, Perfusion and 3D‐MR Spectroscopy: A Prospective Study Ajit Goenka, Atin Kumar, Raju Sharma, Ashu Seith, Rakesh Kumar, Pramod Julka All Inda Institute of Medical Sciences (AIIMS), New Delhi, India Introduction: Conventional MR differentiation of glioma progression or recurrence from treatment‐induced changes is a formidable challenge. We hypothesized that combination of diffusion‐weighted imaging (DWI), perfusion MR and multivoxel 3D‐MR spectroscopy (MRS) may enable this distinction with greater confidence. Patients (or Materials) and Methods: Sixty‐five consecutive patients with treated high‐grade gliomas were examined prospectively with DWI, perfusion‐sensitive contrast‐enhanced MR and 3D‐MRS on a 1.5 T scanner. Thirty‐six lesions in 32 patients could not be characterized on conventional MR. Normalized rCBV and metabolite ratios (nCho/NAA, nCho/Cr and nNAA/Cr) were computed for these lesions. Restricted diffusion was inferred from hyperintensity on trace DWI and corresponding hypointensity on ADC maps. Composite gold standard consisting of histopathology, clinico‐radiological follow‐up and glucoheptonic acid (GHA)‐SPECT was used for final categorization. Results: 21 lesions were due to tumor progression and 15 lesions were due to treatment‐induced changes. Normalized rCBV in progression group (3.82) was significantly higher than in non‐progression group (0.99) (p < 0.05). At rCBV higher than 2.3, 85% lesions could be correctly classified (71.43% sensitivity, 100% specificity). MRS ratios were higher in tumor group; however, the difference was not statistically significant. Restricted diffusion was detected in 10 lesions from progression group but only in one lesion from non‐progression group. Conclusion: Diffusion and perfusion MR enable non‐invasive differentiation of tumor progression from treatment‐induced changes in most cases with high accuracy. Restricted diffusion is highly specific for recurrent tumor. MRS was found not to contribute significantly to overall diagnostic accuracy due to frequent overlap of metabolite ratios, which is likely to be due to inherent heterogeneity of these lesions. 37.  Cryptococcosis of the CNS Presenting with Disseminated White Matter Lesions Daniel Ontaneda, Steven Shook Cleveland Clinic, Cleveland, Ohio, United States Introduction: Cryptococcus neoformans is an encapsulated yeast that produces a picture of meningo‐encephalitis, most commonly seen in immunosupressed patients. Typical MRI imaging findings in Cryptococcal meningo‐encephalitis include mass lesions, pseudo cysts, radiologic meningitis, and hydrocephalus. Patients (or Materials) and Methods: 69 year old male with PMH significant for oral candidiasis, GE leiomyoma, prostate cancer, and hypertension presented to the clinic with a 6 month history of progressive gait dysfunction and lower extremity weakness. Neurological examination showed paraparesis, dysmetria in the upper and lower extremities. Gait was markedly ataxic. Results: MRI of the brain showed confluent T2 hyperintensities involving bilateral sub cortical structures, thalamus, midbrain, and pons. These lesions were hypointesne on T1 imaging. CSF examination showed 19 WBC and 18 RBC, protein was 496 and glucose was 10. Fungal culture grew Cryptococcus neoformans, latex agglutination for Cryptococcus was also positive. HIV serology was negative. CD4 count was 94. All other CSF studies were normal. Conclusion: Our case illustrates a unique presentation of Cryptococcal meningo‐encephalitis in a patient with idiopathic CD4 lymphocytopenia. Diffuse white matter lesions are rare in this entity. Demyelination has been proposed as the pathological basis of these changes. It is felt that cryptococcal infections may produce a radiological picture similar to ADEM. Leukoencephalopathy should be considered along with the more classic imaging features of Cryptococcosis such as cryptococcomas, hydrocephalus, and meningeal enhancement in CNS. 38.  Multiple Subarachnoid Abscesses due to Group B Streptococcal Meningitis Andrea Synowiec, Sandeep Rana Allegheny General Hospital, Pittsburgh, PA, United States Introduction: We present a case of Group B Streptococcus (GBS) meningitis in an elderly male with rare imaging findings of multiple subarachnoid abscesses. Case Presentation: A 70‐year‐old Caucasian male with a history of well‐controlled diabetes and hypertension presented to the emergency department after acute onset of diaphoresis, flu‐like symptoms, fever, and rapid decline in mentation. He had no history of recent infections or travel. MRI was performed at admission and again one week later. Results: Initial MRI revealed a widened CSF space at the vertex in the frontal and parietal regions, with a diffusely septated appearance of the subarachnoid space containing fluid/fluid levels within a few of the loculations. There was notable opacification of the mastoid air cells bilaterally. Lumbar puncture revealed 750 WBC, glucose of 56 mg/dL, and protein of 856 mg/dL. Group B Streptococcus antigen was positive in the CSF. CSF gram stain revealed few gram‐positive cocci, although blood and CSF cultures showed no growth. The patient was initially treated with broad‐spectrum antibiotics. A second MRI revealed increased debris in the subarachnoid loculations, consistent with multiple subarachnoid abscesses. Gadolinium was not administered due to renal insufficiency. Conclusion: GBS is a frequent cause of meningitis in neonates, and less commonly can cause meningitis in peri‐partum women and immunosuppressed adults. We present the unusual imaging finding of multiple subarachnoid abscesses due to GBS meningitis in a diabetic elderly male. 39.  Radiographic Characteristics of a Case of Collision Tumor Comprised of Primary B‐cell CNS Lymphoma and Anaplastic Astrocytoma Lara Kunschner, Frank Pu, Robert Williams Allegheny General Hospital, Pittsburgh, PA, United States Introduction: The juxtaposition of dural‐based tumor and malignant glioma has been infrequently reported. Occasional metastatic rests of systemic malignancies have been identified within primary brain tumors, the so‐called collision tumor. Much less common, though, is the collision of 2 primary central nervous system malignancies, of which only one is gliomatous in origin. The exception, of course, is the co‐occurrence of glioblastoma and sarcoma. Trivial numbers of non‐malignant inflammatory cells within a glioma is fairly common. We present a case of a collision tumor consisting of primary B‐cell CNS lymphoma and anaplastic astrocytoma. Patients (or Materials) and Methods: A healthy, 29 yo man presented with a generalized seizure. Initial evaluation revealed a complex, left parietal mass on brain CT and MRI. The tumor was a collision tumor due to meeting diagnostic criteria for both primary B‐cell CNS lymphoma and Anaplastic astrocytoma within the solitary tumor. Results: The tumor had heterogeneous characteristics pre‐contrast, enhanced post‐contrast, and involved both grey and white matter. No dural thickening or enhancement was noted. The radiographic and pathological images are presented. Radiographic findings from recent cases of malignant glioma in juxtaposition with dural–based malignancy are also presented, as well as typical MRI findings in both primary lymphoma and AA as isolated tumor. Conclusion: Central nervous system collision tumors are rare entities, especially when comprised of 2 primary brain malignancies. The presence of an identifiable tissue plane is helpful in the radiographic diagnosis, but overlapping characteristics make radiographic diagnosis very difficult. 40.  MECP2 Duplication Associated with Dysgenesis of the Corpus Callosum Ngoc Minh Le, Manikum Moodley Cleveland Clinic, Cleveland, OH, United States Introduction: Mutations in methyl‐CpG‐binding protein 2 (MECP2) on chromosome Xq28 is a well known cause for classical Rett syndrome, an X‐linked dominant developmental disorder, predominantly seen in females. Mutations may also cause a more severe neurodevelopmental phenotype in rare surviving males. MECP2 duplication causes a different phenotype which includes severe mental retardation, hypotonia, recurrent respiratory infections, and clinical features of Rett syndrome. There have been no reports in the literature of brain abnormalities on MRI in Rett syndrome, except for diffuse atrophy as measured by volumetric studies. One prior case report has associated MECP2 duplication with periventricular nodular heterotopia. Patients (or Materials) and Methods: A 5 year old boy with severe global developmental delay since 9 months of age presented mild hypotonia, stereotypies, optic atrophy, plagiocephaly, and hypertelorism. He was adopted at birth. Results: At 1 year of age, chromosome karyotype was normal (46XY) and brain MRI showed dysgenesis of the corpus callosum (absence of rostrum and markedly thinned genu body and splenium) with repeat MRI 2 years later showing additional diffuse white matter volume loss. Chromosomal microarray (CMA) revealed chromosome Xq28 duplication of 877 Kb in size which contains the region for MECP2. Conclusion: Unlike Rett syndrome, MECP2 duplications can be associated with brain malformations, including malformations of cortical development and midline defects. Therefore in patients with developmental delay without clear etiology, CMA may provide a definitive diagnosis and in those with MECP2 duplications, brain MRI may reveal associated intracranial abnormalities. 41.  MRI Findings of an Unusual Case of Hemorrhagic Primary CNS T‐cell Lymphoma – A Case Report Daniel Thielemann, Jon Brillman, Sandeep Rana Allegheny General Hospital, Pittsburgh, Pennsylvania, United States Introduction: We present MR findings of malignant primary CNS T‐cell lymphoma. Case Presentation: A 42 year old Kuwaiti female with no significant past medical history presented to an outside hospital with a chronic history of headache, altered mental status, and dizziness. Results: Based on original MRI and CSF findings she was initially treated for encephalitis. She returned one month later with worsening symptoms and new‐onset generalized seizures. Repeat MRI scanning demonstrated interval increase in lesion burden and hemorrhagic changes throughout, at which point she underwent treatment with intravenous steroids for presumed cerebral vasculitis. The patient worsened, and follow‐up MRI demonstrated multiple focal supratentorial hemorrhagic lesions with surrounding vasogenic edema, diffuse areas of restricted diffusion, and subfalcine herniation. MRA demonstrated widespread irregularities of the intracranial vasculature. The patient underwent brain biopsy which revealed the diagnosis as malignant primary CNS T‐cell lymphoma with extensive hemorrhage and necrosis. HIV antibody type 1 and 2 were negative. She underwent left‐sided decompressive hemicraniectomy for amelioration of increasing intracranial pressure and was treated with intravenous steroids. Ultimately, she succumbed to her illness. Conclusion: Primary CNS T‐cell lymphoma is an extremely rare disorder with an incidence of 3.6–8.5% of lymphomas and has a poor prognosis. We present unusual MR imaging of primary CNS T‐cell lymphoma with hemorrhagic changes in an HIV negative patient. 42.  Midbrain Stroke Causing Pupil Sparing Oculomotor Nerve Palsy in Patient with Diabetes Parastou Shilian, Adel Olshansky, Nerses Sanossian, Said Beydoun USC, Los Angeles, CA, United States Introduction: Pupil sparing oculomotor nerve palsy (PSOMNP) in patients with diabetes is commonly due to ischemic neuropathy in the subarachnoid space. Evaluation of PSOMNP often relies on clinical symptoms/signs and often does not utilize brain MRI. We present a case of PSOMNP secondary to brainstem infarct. Patients (or Materials) and Methods: Case report and neuroimaging Results: 72 year old woman with diabetes mellitus, congestive heart failure, hypertension, and aortic valve stenosis developed left ptosis and diplopia after aortic valve replacement surgery. Her post‐operative course was complicated by one episode of atrial fibrillation with rapid ventricular response. Patient was started on anticoagulation. Neurological examination revealed complete ptosis of the left eye that was depressed and abducted on primary gaze. Patient was unable to cross midline on adduction or elevate her left eye. Pupils were symmetrically reactive. MRI of the brain demonstrated DWI hyperintensity in the area of left paramedian midbrain consistent with acute infarct. Conclusion: Both diabetic ischemic mononeuropathy and stroke can lead to PSOMNP. Most cases of diabetic patients with PSOMNP are not evaluated with appropriate neuroimaging and may, in fact represent ischemic stroke. This case demonstrates the importance of considering ischemic midbrain infarction in the differential diagnosis of PSOMNP and obtaining appropriate neuroimaging. 43. “Hot Cross Bun” Sign and Other Neuro‐imaging Findings in a Patient with Multisystem Atrophy: Case Report Arash Taavoni, Jon Brillman, Sandeep Rana Allegheny General Hospital, Pittsburgh, PA, United States Introduction: Multisystem atrophy (MSA) consists of a group of disorders characterized by neuronal degeneration mainly in the substantia nigra, striatum and cerebellum. Although MSA is distinct from Parkinson's disease it shares many aspects, such as tremors, cogwheel rigidity, bradykinesia, ataxia and autonomic findings. Our case report underscores the importance of neuro‐imaging in diagnosing MSA. Patients (or Materials) and Methods: 57 year‐old, female, past medical history of hypertension, diabetic neuropathy, status post gastric feeding tube, and a three year history of progressive lower extremity “weakness” to the point of being wheelchair bound. She had severe cognitive impairment and could not care for herself. Patient presented to the hospital with fever and diarrhea. On physical exam the patient was orient only to self and place. Positive Cogwheel rigidity and orthostatic hypotension. Mild resting tremor. Results: MRI demonstrated a very prominent “hot cross bun” sign in which there is a cruciform linear area of high signal on T2WI in the pons with tiny round darker areas within the checkerboard of the cross. Atrophy of the cerebellum and pons is also seen. Conclusion: In our case report, we describes a patient whose MRI findings demonstrate recently described finding of “hot cross bun” sign in MSA. This finding is highly specific to this disorder. Although neuro‐imaging is not necessary to make the diagnosis of MSA, MRI findings can be crucial to making the correct diagnosis. 44.  DWI‐Negative Stroke and Pure Hypoperfusion: An Imaging Indication for Hemodynamic Therapies? Sarah Song, Ichiro Yuki, Satoshi Tateshima, Doojin Kim, David S. Liebeskind UCLA Stroke Center, Los Angeles, CA, United States Introduction: Acute stroke therapy focuses solely on arterial recanalization, yet reversal of hypoperfusion is the ultimate therapeutic goal. Although the underlying stroke etiology is rarely established in the acute phase, compromised hemodynamic status and risk of evolving infarction may be dissimilar in progressive stenosis compared with thromboembolic occlusion. Imaging of collateral perfusion and infarct patterns may provide clues for optimal treatment in a given patient. Methods: Case report. Results: A 68‐year‐old woman with diabetes presented with acute expressive aphasia, right facial paresis and right hemiparesis (NIHSS 12). CT confirmed no hemorrhage, and the patient received intravenous tPA. Repeat exam 6 hours after onset showed improvement in strength but persistent aphasia (NIHSS 7). MRI revealed no DWI hyperintensity but extensive areas of perfusion delays in the left MCA territory. MRA showed severe flow attenuation of the left M1 and distal branches. Angiography revealed elongated stenosis of the left M1. Collateral flow was supplied via leptomeningeal anastomses from ipsilateral ACA and PCA, leaving a vulnerable area in the left frontal operculum consistent with localization of her aphasia. Treatment with IV fluids and permissive blood pressures were initiated and the patient was normal within days. Serial multimodal MRI revealed no subsequent DWI lesion despite persistent perfusion delays. Conclusion: DWI‐negative stroke and pure hypoperfusion may be a marker of transient failure in established collaterals. Stroke and neurological deficits may result from pure hypoperfusion. Multiparametric characterization of collateral perfusion may chronicle restoration of cerebral blood volume and neurological improvement despite persistent delays in perfusion. 45.  Superior Sagittal Sinus Thrombosis – Imaging Pitfalls Marcella A. Wozniak 3 , Sandra Ruby 1 , Alexander Mark 2 
 1 University of Maryland School of Medicine, Baltimore, MD, United States, 2 University of Maryland Medical Center, Baltimore, MD, United States, 3 Baltimore VHA Medical Center, Baltimore, MD, United States Introduction: Patients with superior sagittal sinus (SSS) thrombosis (SSST) can present with cerebral venous infarction, intracerebral hemorrhage, headache or increased intracranial pressure. Methods: Three clinical cases. Results: 1. 53 yo woman on hormone replacement complained of headache then developed left weakness and left visual field loss. Head CAT revealed a region of hypodensity in right parieto‐occipital region with hemorrhage. Magnetic resonance (MR) venography (MRV) sagittal time of flight sequences suggested partially occlusive SSST, but phase contrast sequences revealed a patent SSS. 2. 38 yo woman with prior DVT and migraines had refractory headache and opening pressure of 34 on lumbar puncture. Head MR suggested increased signal on T1 sequences in the entire SSS. MRV was initially reviewed as “flow’ in SSS. Review of T1 sequences revealed subacute thrombus in the entire SSS resulted in increased signal that mimicked the “flow” on the MRV. 3. 34 yo morbidly obese woman presented with the sudden onset of left hemiparesis, normal head CAT and received IV tPA. Her weakness resolved. Repeat head CAT and CAT angiogram were normal. She had a focal then generalized seizure beginning in the left arm. A diagnosis of “transient tumor attack” was considered. A head CAT with contrast revealed a new area of hypodensity in right frontal lobe and decreased enhancement in the SSS. Conclusion: MRV and CAT venography can facilitate the diagnosis of SSS thrombosis. Correlation of imaging with clinical presentation and careful review of all available imaging is needed when assessing patients for SSS thrombosis. 46.  Combined Lateral Pontine and Extrapontine Myelinolysis Ehsan Malek, Alireza Negahban, Jamshid Ahmadi University of Southern California, Keck School of Medicine, Radiology Department, Lose Angles, CA, United States Objective: To illustrate detailed MR imaging of extensive myelinolysis associated with rapid correction of severe hypernatremia. Findings: After three weeks binge drinking, a 40 year old alcoholic man admitted in stupor with a serum sodium level of 190 mmol/L. He progressed to coma with flaccid quadriplegia following correction of serum sodium to 155 mmol/L over 3 days. MRI showed extensive bilateral involvement of the ventrolateral aspect of the rostral pons with sparing of the central pons and pyramidal tract. The lesions were hypointense on T1, and hyperintense on T2. These MR findings show a butterfly appearance of the pons. The extrapontine lesions were in globus pallidi, putamen, claustri, thalmai and hypothalami. Repeat MRI four weeks later showed slight improvement. Three months later MRI shows development of minimal atrophy in the involved areas of pons and deep brain tissue. Conclusion: The MRI findings correlate closely with a postmortem neuropathologic description of a single previous case report in 1989 without MRI. 47.  Bilateral Sturge‐Weber Syndrome Georg Evers, Tobias Loddenkemper Children's Hospital, Boston, MA, United States Introduction: Sturge‐Weber‐syndrome (SWS) is a sporadic congenital neurocutaneous syndrome presenting with unilateral facial vascular nevus and ipsilateral leptomeningeal angiomatosis. We present an infant boy with bilateral facial nevus, bilateral cerebral leptomeningeal enhancement, and calcifications. Methods: Case study including history, MRI, CT and EEG‐data. Results: This infant boy presented at birth with a bilateral facial vascular malformation extending also over parts of the trunk and extremities. Exam revealed spastic quadriparesis. Seizures occurred on day of life three. Brain MRI at one month of age showed diffuse leptomeningeal enhancement over both cerebral hemispheres, enhancing bilateral ischemic white matter changes as well as atrophy. Bifrontal white matter calcifications were confirmed on subsequent head‐CT at 2 month of age. EEG showed asymmetry of the background and sleep structures with higher amplitudes on the right and bilateral posterior spikes. EEG‐seizures arose independently from both hemispheres. Clinical seizures consisted of left clonic, tonic, and eye versive seizures. Follow up MRI scan at 3 month of age revealed progressive bilateral atrophy of both cerebral hemispheres, worsening ischemic white matter changes, and increasing calcifications. Seizures remained refractory to treatment with phenobarbital, phenytoin, topiramate, clonazepam and levetiracetam. Conclusion: The case illustrates a rare bilateral manifestation of SWS. Bilateral SWS may represent an extreme variant of the SWS‐spectrum or even a separate syndrome. Calcifications and meningeal angiomatosis are typical features of SWS but bilateral calcifications are rarely seen. Additionally, calcifications are unusual under one year of age. Hemispherectomy is usually not a treatment option in patients with severe bilateral SWS. 48.  Orbital Apex Syndrome: A Rare Case of Perineural Invasion of Skin Cancer Sara Khan, Charles Bae Cleveland Clinic, Cleveland, United States Introduction: Perineural invasion and orbital extension is a rare manifestation of skin cancer. Initial neuroimaging is often negative which can delay diagnosis. We present a case of this rare condition, with an emphasis on the clinical presentation and neuroimaging findings. Patient and Methods: A 57 year old man presented with progressive symptoms over 3 months. He initially noticed right forehead numbness followed by double vision. He quickly developed a complete right ptosis. He also had a severe right‐sided headache and ocular pain. Past medical history was significant for Squamous cell carcinoma of his forehead (had resection four years prior) without recurrence. Neurological exam was normal except for right ptosis, fixed and dilated right pupil, complete right opthalmoplegia and numbness in the right V1 distribution. Results: A brain MRI performed two months after symptom onset was normal. Cerebrospinal fluid was normal. Brain MRI a month later was still normal. However, a dedicated orbit MRI demonstrated an enhancing right orbital apex mass which involved the right cavernous sinus and superior oblique muscle. Open biopsy of the mass revealed a poorly differentiated squamous cell carcinoma with perineural invasion. Conclusion: Orbital apex syndrome resulting from perineural spread of squamous cell carcinoma often presents in the setting of a previously treated head or neck skin cancer. The diagnosis can be missed without local recurrence of the skin cancer. It can be confused with other conditions (e.g. trigeminal neuralgia, herpes zoster). Imaging may not pick up an abnormality until macroscopic disease develops in the orbit. 49.  Reversible Posterior Leukoencephalopathy Syndrome Due to Ephedrine After Elective Cesarean Section Qing Hao, Matthew Tenser, Radoslav Raychev, David Liebeskind UCLA Stroke Center, Los Angeles, United States Introduction: Reversible posterior leukoencephalopathy syndrome (RPLS) manifests neuroimaging findings similar to hypertensive encephalopathy and eclampsia, including predominantly bilateral subcortical lesions and angiographic features of vasoconstriction. Sympathomimetic agents related to ephedrine may cause vasoconstriction, yet RPLS due to ephedrine has not been described. Patients and Methods: Case report. Results: A 31‐ year‐old woman with no past medical history suddenly developed right upper quadrant visual deficit, short‐term memory loss and dull headache associated with nausea, vomiting and photophobia at 24 hours after elective cesarean section. She was not hypertensive or eclamptic, but received ephedrine to avoid hypotension after spinal anesthesia. Her symptoms lasted only 40 minutes, yet MRI on the next day revealed DWI and FLAIR hyperintensity in the left posterior temporo‐occipital lobe. MRA was unrevealing. Two months later, follow‐up MRI showed complete resolution of the previously noted abnormality. She remained asymptomatic without neurological sequelae. Conclusion: RPLS and transient neurological deficits lasting only minutes in duration may occur shortly after ephedrine use. RPLS due to ephedrine for potential hypotension after cesarean section should be recognized as a potential complication. Subtle vasoconstrictive effects of ephedrine may not be apparent on MRA. Overlap with postpartum angiopathy or reversible cerebral vasoconstrictive syndrome, however, suggests a common vascular pathophysiology that may be discerned with serial MRI. 50.  Evolution of FLAIR Vascular Hyperintensities in Acute Ischemic Stroke Nerses Sanossian 1 , Qing Hao 2 , David Liebeskind for the UCLA Collateral Inversigators 2 
 1 University of Southern California, Los Angeles, CA, United States, 2 University of California Los Angeles, Los Angeles, CA, United States Background: FLAIR vascular hyperintensities (FVH), the relatively subtle serpentine early vessel signs in acute ischemic stroke, are caused by slow retrograde flow from leptomeningeal collaterals. Angiographic correlative studies reveal that FVH are highly specific for collaterals and hypoperfusion, changes in FVH associated with revascularization have not been chronicled. Methods: Retrospective review was conducted of consecutive acute ischemic stroke cases at a single institution with serial FLAIR MRI acquired before and after conventional angiography. FVH characteristics on serial MRI were reviewed in blinded fashion. Angiographic patency and perfusion status was separately graded by TIMI score. Results: 632 stroke cases were reviewed to identify 55 with FLAIR prior to and after angiography. Initial MRI revealed FVH in 46/55 or 84% of cases. FVH was noted on only 18/55 or 33% of follow‐up MRI scans. In all cases with FVH on follow‐up MRI imaging there was evidence of FVH on the initial scan. In the interventional cohort (n = 33), cases with persistent FVH were discharged with less severe strokes (NIHSS 4 vs. 11, p = 0.04) despite presenting with equally severe strokes (22 vs. 17) and having equal recanalization rates (final TIMI 1.8 vs 1.5). This was due to the higher percentage of partial territory infarction with persistent FVH (75% vs. 50%) rather than complete territory infarction (10% vs. 25%). In non‐interventional cases (N = 22) persistence of FVH was also associated with partial territory infarction (90% vs. 23%). Conclusions: Persistence of FVH on post‐angiography MRI is associated with partial territory infarction. In cases of complete territory or no infarction, FVH disappear. 51.  Multiple Ischemic Strokes after Transcatheter Arterial Chemoembolization for Hepatocellular Carcinoma with a Radiographic and Pathologic Correlate Victor Zach 1 , Beth Rapaport 1 , Ji Yeoun Yoo 1 , Lara Goldfeder 2 , Jesse Weinberger 1 
 1 The Mount Sinai School of Medicine, New York, NY, United States, 2 New York City Office of Chief Medical Examiner and Department of Forensic Medicine, New York University School of Medicine, New York, NY, United States Introduction: Transcatheter arterial chemoembolization (TACE) is a widely used form of therapy in advanced hepatocellular carcinoma (HCC). We report an autopsy case of multiple cerebral emboli during TACE. To our knowledge, this is the first case with pathological data. Patients (or Materials) and Methods: A 66 year old man with recurrent HCC underwent TACE. Forty‐five minutes into the procedure the patient suddenly became unarousable. On examination, the patient was nonverbal, opened his eyes to voice, and did not follow commands. Right gaze preference, right facial weakness, but otherwise normal brainstem reflexes were present. He localized to noxious stimuli. Ankle clonus was present bilaterally. His fingers and left foot were cold and cyanotic. Results: Diffusion weighted imaging of the brain revealed innumerable punctate areas of restricted diffusion throughout both cerebral hemispheres and cerebellum reflecting small infarcts that appeared embolic. Transesophageal echocardiography and emboli detection transcranial dopplers did not demonstrate a shunt. An abdominal CT revealed bibasilar atelectasis. No significant improvement occurred after 6 weeks. On autopsy multiple microscopic subacute infarcts were found in the cerebrum, midbrain and cerebellum, but no embolic material was seen. Embolic material was noted in dilated vessels throughout the fibrotic right diaphragm and in the upper lobe of the right lung. Conclusion: Cerebral embolism after TACE of HCC is rare, yet devastating. Our patient's post‐TACE abdominal CT revealed bibasilar atelectasis masking a pathologically proven hepato‐pulmonary shunt. We suggest careful evaluation for invasion of HCC into the thoracic cavity prior to TACE. 52.  Fibromuscular Dysplasia Presenting as a Common Carotid Artery Dissection Resulting in Multiple Ischemic Infarcts: Neurosonographic Diagnosis with Angiographic Correlate Victor Zach, Svetlana Zhovtis, Kathryn Kirchoff, Jesse Weinberger The Mount Sinai School of Medicine, New York, NY, United States Introduction: Common carotid artery (CCA) dissection is a rare cause of ischemic stroke. We describe a case of multiple acute infarcts in a patient with CCA dissection, detected initially by carotid duplex (CD.) Patients (or Materials) and Methods: A 50 year‐old Caucasian female with a history of classic migraine developed a rapidly expanding visual aura evolving into an inability to see on her right side, left cervicooccipital pain, and an inability to speak, lasting for 45 minutes. On arrival, the patient was asymptomatic. Her mood was labile, cycling rapidly between inappropriate laughter and inappropriate crying. Object recall was impaired. She had no focal neurological deficits. Results: An urgent CD revealed a circular area of dissection in the distal CCA with some mobility of the dense circular rim of the thrombus and a lucent core. Magnetic resonance imaging (MRI) revealed multiple foci of acute infarction in the left anterior, middle, and posterior cerebral artery distributions, and a carotid (i.e. fetal) origin of the left posterior cerebral artery. Magnetic resonance angiography (MRA) of the neck with gadolinium demonstrated the CCA dissection. Full anticoagulation was started. Conventional angiography confirmed the CCA dissection and was suggestive of fibromuscular dysplasia. At one month follow up she remained asymptomatic. On repeat CD there was a 3.5 mm area of distal CCA dissection with resolution of the circular thrombus. Conclusion: CCA dissection is rare but must be recognized by CD. Axial images are necessary in patients where dissection is suspected to make a definitive diagnosis. 53.  Extra Cranial Carotid Artery Disease in Egypt Foad Abd Allah, Essam Baligh 1 Cairo University‐Neurology Department, Cairo, Egypt, 2 Cairo University‐Cardiovascular Medicine Department, Cairo, Egypt Background and Purpose: The prevalence of carotid artery stenosis has been studied in several cohorts and cross‐sectional studies of populations. Prevalence estimates are mostly based on ultrasound studies, because duplex ultrasound is accurate, reproducible, and inexpensive to diagnose and follow patients with arterial disorders. The purpose of our study is to describe the pattern of a large sample of Egyptians. Methods: We analyzed the data of 4733 Egyptian subjects who underwent extracranial carotid duplex scanning at the vascular laboratories of two large tertiary referral hospitals in Cairo from 1.2.2003 to 1.2.2008. Demographic and clinical data and cause of referral were correlated with ultrasound findings. Results: Atherosclerotic carotid artery disease was present in 40.9% of the study population: Intimal thickening and less than 50% stenosis in 38.34%, 50–69% internal carotid artery stenosis in 1.9%, ≥ 70% stenosis in 0.6% and occlusion in 0.06%. Non‐atherosclerotic disease was detected in 9 subjects (0.19%) only. Multivariate stepwise logistic regression analysis selected age, hypertension and diabetes mellitus as independent predictors of the presence of carotid atherosclerotic disease. Conclusion: Hemodynamically significant and clinically relevant extracranial atherosclerotic carotid disease is rare in Egyptians. Risk factors for carotid atherosclerosis are the same as in societies where carotid disease is more prevalent. 54.  Cerebral Blood Flow Velocity Measurement in Children at Risk for Hypoxic‐Ischemic Brain Injury: Preliminary Results Michele Merat 2 , Audrey Shibata 1 , Jamie Hutchison 1 , Anne‐Marie Guerguerian 1 
 1 Hospital for Sick Children, Toronto, Ontario, Canada, 2 University of Bern, Bern, Switzerland Objectives: To characterize the relationship between cerebral blood flow velocity (CBFV), physiologic variables, and outcome in children at risk for hypoxic‐ischemic (HI) brain injury. Methods: Prospective cohort study of children following cardiopulmonary arrests and return of spontaneous circulation, or cardiac and/or respiratory failure supported by extra‐corporeal mechanical oxygenation (ECMO), associated with coma. Utstein methods were used for definitions. CBFV measured with transcranial Doppler sonography, cerebral oxygen saturation (ScO2) with near infrared spectroscopy, and systemic physiologic variables were measured daily up to 6 days during stable hemodynamic conditions until extubation, decannulation from ECMO, or death. Pediatric Cerebral Performance Category (PCPC) was assessed at 3 and 6 months. Longitudinal data analyses for repeated measures were used to model the relationship between CBFV and physiologic variables. Results: Nineteen comatose subjects were enrolled after informed consent process, aged between 7 days to 17 years. Among subjects supported by ECMO, change in CBFV was associated with flow in ml/min, time after injury and PaO2. CBFV was not independently associated with mean arterial blood pressure (MABP), hematocrit, and PaCO2. ScO2 was associated with MABP and ECMO flow. Probability of survival was not statistically associated with CBFV however a trend for increased or maintained CBFV is shown in the current sample. Conclusions: These preliminary data suggest that the study is feasible. More importantly these data are needed given that the relationship between CBFV and physiologic variables reported (or expected) in healthy children is not immediately found in children with coma following events associated with severe risk for HI brain injury. These preliminary results need to be examined further, for longer periods of time, and focusing on measuring the integrity of cerebral autoregulation. 55.  Cerebrospinal Fluid (CSF) Flow Across the Aqueduct of Sylvius (AS) in Neonates is Induced by Respiration Paul Maertens, Shannon Overs, Daniel Dees, Zachary Funk, Lee Vick University of South Alabama, Mobile, AL, United States Introduction: In adults, it is currently accepted that cardiac cycle‐related variations in cerebral blood volume produce bidirectional oscillatory movement of CSF within AS. To assess if this is also true in neonates we investigated the Doppler spectral waveform of CSF flowing across AS and analyzed its temporal relation to the cardiac cycle and respiration. Patients (or Materials) and Methods: Seven neonates with grade 2 IVHs were first shown to have a flow signal in AS using power Doppler imaging through the anterior and posterior fontanelles using a Phillips 5500. Subsequently, using duplex imaging, Doppler spectral waveform of CSF was displayed simultaneously with EKG tracing and temporal relation to respiration was analyzed using the flow–time display on Dräger ventilators. Results: Doppler spectral waveform of CSF in AS was bidirectional in all neonates. The flow direction of CSF was not affected by cardiac cycle. Instead flow direction of CSF correlated directly with the flow of air in the upper airways during respiration. During inspiration, there was a short caudocranial movement of CSF which was followed by a longer craniocaudal movement of CSF during expiration. Conclusion: In neonates with IVHs, duplex imaging can provide real time information about CSF flow. The bidirectional oscillatory movements of CSF within AS are produced by variations in the cerebral blood volume during the respiratory cycle. During inspiration, the net inflow of blood increases the intracranial volume and produces craniocaudal CSF flow. During expiration, the net outflow of blood decreases the intracranial volume and promotes caudocranial flow.TCD Sensitivity (%) Specificity (%) PPV (%) P valuebMCA MFV≥120 66.7 86.4 47.1 <0.001 mMCA MFV≥120 83.3 63.3 22.7   0.003 Preangio MFV≥150 91.7 85.3 42.3 <0.001 aMCA‐bMCA ratio ≥1.5 91.7 60.3 27.5   0.001 aMCA‐cMCA ratio ≥1.25 75  55.6 14.8   0.042 aMCA‐iACA ratio≥2 36.4 66.3 12.1  0.55 aMCA‐iPCA ratio≥2.5 90   31.1 13.5  0.58 aMCA Lindegaard ratio 91   69   31.2 <0.00156.  Derivation of Transcranial Doppler criteria for Angiographically proven Middle Cerebral Artery vasospasm after Aneurysmal Subarachnoid hemorrhage Joseph Sebastian, Carol Derksen, Khurshid Khan, Bilal Hameed, Mohammad Ibrahim, Muzaffar Siddiqui, Ashfaq Shuaib, Maher Saqqur University of Alberta, Edmonton,Alberta, Canada Introduction : Transcranial Doppler (TCD) has been subjected to many criticisms for its ability to detect impending vasospasm. The purpose of this study is to derive a new criteria of middle cerebral artery (MCA) vasospasm (VSP) based on correlation between TCD and MCA vasospasm after aneurysmal SAH. Patients and Methods : A prospective collected data of 134 patients with aneurysmal SAH admitted to University of Alberta hospital from January 2006 to December 2008. Complete TCD was performed daily from day 2 to 14 from symptoms onset. Follow up DSA was done at day 7 to 9 from symptom's onset. TCD mean flow velocity (MFV) of all vessels at baseline, middle and before DSA were measured.The following MCA ratios were calculated: Affected MCA MFV/ baseline MCA MFV ratio (aMCA/ bMCA ratio), aMCA/ contralateral (c) MCA MFV ratio, aMCA/ ipsilateral (i)ACA MFVratio,aMCA/ iPCA MFV ratio, and aMCA Lindegaard ratio. DSA VSP was defined as > 1/3 luminal narrowing. Univariate and multiple variate analysis were used. Results : Results are preliminary and final results will be notified later. Based on the above ratios, an algorithmic TCD analysis for MCA VSP will be developed. Conclusion: Stringent criteria for MCA VSP based TCD velocities are feasible and applicable in SAH population.ECHO SAV V‐V E/A Ao (VTI) M(VTI)Baseline 180 LV 40 1.3 12.2 12.9 150 1.8 15.6 210 1.8 15.9 200 LV 40 1.6 13.0 15.0 Final 200 LV 20 13.0TCD P AV Range of flow V‐V Δ Baseline 200 105–111 0 90–106 100 101–113 LV 10 104–113 150 101–110 LV 20 104–111 160 107–111 LV 30 104–111 180 105–115 LV 50 100–10957.  Usefulness of TCD as a Diagnostic Tool to Evaluate Intracranial Hemodynamic Status: Five Years Experience Methil Pradeep, Sowntharya Yashoda, Mazharudeen Mohammed KG Hospital & Post Graduate Institute, Coimbatore, Tamilnadu, India Background & Purpose: TCD is useful for understanding cerebral hemodynamics.We analyzed TCD data performed over last 5 years to evaluate the usefulness of TCD as a diagnostic tool. Methods: We studied 500 cases. To evaluate alteration of cerebral hemodynamics the blood flow velocity of each artery was classified into marked decrease, decrease, normal, increase & marked increase. Results: Incomplete insonaton was performed in 22% of cases. In blood flow velocity evaluation not insonated was mainly in the anterior circulation. The incomplete studies were significantly higher in women (60%) compared to men (30%). Most of the incomplete studies were in elderly patients with cerebro vascular disease. Normal range of velocities was seen in 40% of cases in the anterior circulation. Remarkable hemodynamic alterations were observed only in a small proportion of cases .8% showed marked increase& 3% showed marked decrease in velocities. In the posterior circulation not insonated vessels were < 3% compared to the anterior circulation. In the posterior circulation normal range of blood flow velocity was more frequently observed than in the anterior circulation. Conclusion: In 5 year experience of TCD not insonated vessels was a major problem to evaluate the anterior circulation, especially in the elderly stroke patients. Small proportion of remarkable blood flow alterations was another limitation for TCD. 58.  Feasibility of Transcranial Doppler to Perform Atrioventricular and Ventriculoventricular Optimization in Patients who have a Biventricular Pacemaker Defibrillator Rachel Horne, Sadaf Ashfaq, Mariam Ishaque, Moses Mulindi, Wes Alexander, Reza Ahmadian, Amer Suleman 1 The Heartbeat Clinic, Dallas, Texas, United States, 2 Medtronic USA, Minneapolis, United States Introduction: Traditional echocardiographic atrioventricular optimization (AVO) for patients with biventricular devices requires diastolic filling and ventricular systole be adjusted to improve stroke volume hence tissue perfusion. Since TCD measures brain perfusion directly, it theoretically can be used to measure tissue perfusion and perform AVO. Patient and Method: A 55 year old man with a biventricular defibrillator was studied. Echocardiographic AVO was performed. Optimal stroke volume resulted from a ventriculoventricular (VV) interval adjustment of LV 20 (LV 20 = LV activated before RV 20 ms). In a double blind manner another optimization was performed with TCD using head frame to insonate middle cerebral artery. At a VV interval of 0 and AV interval of 200, Delta button was pressed to set baseline at 100%. Mean velocity > 100% means positive and < means negative trend. First VV than AV intervals were adjusted. During adjustments, patient alternated between 15 seconds of rest and data recording, determining range of flow. Results:Conclusion: Results show that gold standard of echo optimization is comparable with TCD optimization. This maybe a useful future technique, but the relevance would require more clinical studies. 59.  Color Doppler Flow Imaging of the Ophthalmic Artery Flow in Moyamoya Disease Shoichiro Kawaguchi, Takeshi Matsuyama, Hideaki Mishima, Yasushi Shin, Hiroaki Tanaka Nara Prefectural Nara Hospital, Nara, Japan Introduction: The authors discussed and analyzed the ophthalmic artery (OA) flow in moyamoya disease. The OA of moyamoya disease works as the important collateral pathway for intracranial blood flow. Patients and Methods: The 43 patients (mean age: 36 years) with moyamoya disease were evaluated. The patients were divided into two groups according to the initial clinical symptoms. Group A consisted of the 12 patients presenting with an initial hemorrhagic episode, and Group B consisted of the 31 patients presenting with an initial ischemic episode. There was no significant difference of the demographic data between two groups. In each group, the OA flow of the symptomatic side was examined using the OA color Doppler flow imaging (CDFI) revealing the peak systolic flow velocity (Vs), time‐averaged maximum flow velocity (TAMX) and pulsatility index (PI) of the OA. Results: 1) The average Vs was 0.43 m/sec, and the average TAMX was 0.26 m/sec. These values were significantly (p < 0.05) high compared to the normal controls. The average PI was 1.18, which was significantly low compared to the controls. 2) The OA CDFI findings of Group A showed the significantly (p < 0.05) high Vs (mean: 0.66 m/sec), high TAMX (mean: 0.41 m/sec), and low PI (mean: 0.89) compared to the OA CDFI values of Group B. Conclusion: The OA CDFI findings such as high Vs, high TAMX and low PI significantly related to the intracranial hemorrhage in moyamoya disease. 60.  Unusual Transcranial Doppler Findings in Rabies Encephalitis Aarti Sarwal, Joshua Botdorf, Manjamalai Sivaraman, Yuji Oba University of Missouri, Columbia, MO, United States Rabies encephalitis is a uniformly fatal disease. Transcranial Doppler(TCD) studies in known cases have described global vasospasm. We present TCD findings in one such case managed with Milwaukee Protocol. 55 year‐old male presented with hydrophobia, ataxia and paresthesias,five weeks after exposure. Patient was intubated & sedated. Milwaukee Protocol was initiated. CT head was unremarkable. Amantadine, Nimodipine, tetrahydrobiopterin, CoenzymeQ10, Vitamins, levocarnitine started. TCDs (pCO2 30.6, MAP 96) showed LMCA peak‐systolic velocity (PSV) 108, end‐diastolic velocity (EDV) 28, mean velocity (MV) 47, Resistivity index (RI) 0.74 and Pulsatility Index (PI) 1.7; RMCA PSV 83, EDV 23, MV37, RI 0.72, PI 1.62. Autonomic failure ensued, sedatives were tapered;burst suppression persisted on EEG. Follow up TCDs (pCO2 43.4, MAP 71) showed LMCA PSV 181, EDV 39, MV 70, RI 0.78, PI 2.03; RMCA PSV 161, EDV 39.5, MV 65, RI 0.75, PI 1.87 .CTangiogram showed effacement of sulci but no vasospasm; opening pressure 41 cm on lumbar puncture. Next TCD (pCO2 54.3, MAP 69) showed LMCA PSV141, EDV 37.6, MV 70.1, RI 0.73, PI 1.48; RMCA PSV 114, EDV 32, MV 55.3, RI 0.72, PI 1.48. Patient then developed acute renal failure, severe acidosis, hypotension refractory to vasopressors and transtentorial herniation. Patient expired on Day 8 of hospitalization. Steadily increasing velocities & indices in this patient represented increasing cerebral edema with no vasospasm on CTA. PI/RI represent increase in peripheral vascular resistance & correlate well (r = 0.99). Increased intracranial pressures in this case may have been side effect of therapy or be part of natural history of rabies adding to spectrum of Doppler findings in this rare disease. 61.  The Progression of Middle Cerebral Artery Velocities on Transcranial Doppler in Children with Sickle Cell Disease Barbara Voetsch 1 , Feliks Koyfman 1 , Haroot Artinian 1 , Philippa Sprinz 2 , Karla Fuentes 2 , Pikula Aleksandra 1 , Michael Winter 3 , Christopher Kelly 1 , Michael Murray 1 , Matthew Ogrodnik 1 , Viken Babikian 1 
 1 Department of Neurology – Boston Medical Center, Boston, United States, 2 Department of Pediatrics – Boston Medical Center, Boston, United States, 3 Boston University School of Public Health, Boston, United States Introduction: Sickle cell disease (SCD) carries a high risk of stroke in children. Based on the STOP trial, transcranial Doppler (TCD) is recommended to identify children with increased stroke risk, defined sonologically as mean middle cerebral artery velocity (MCAv) ≥200 cm/sec. However, the rate of disease progression as measured by TCD and the effect of therapeutic interventions on its values has not been extensively studied. Patients (or Materials) and Methods: We performed a retrospective study of 79 children with homozygous SCD (53.2%M; age at first TCD: 7.4±4.1 yrs; 86.1% African‐American) followed at Boston Medical Center's Pediatric Hematology program from 2004–2008. Treatment with hydroxyurea and/or exchange transfusion was initiated as clinically indicated. Serial TCDs were performed, and mean MCAv change over time was analyzed by linear regression. Results: All children had at least 2 TCD studies (53%: 3 studies; 32%: ≥ 4 studies). The average MCAv on first study was 134.1±27 cm/sec (reference for unaffected children: 80±15 cm/sec). Four children had MCAv ≥200 cm/sec on first TCD, two of which improved with transfusions. Three children (4%) with non‐critical values initially, progressed to MCAv≥200 cm/sec. Overall, there was a non‐significant increase in MCAv to 153.2±39.9 cm/sec (p = 0.22). In treatment‐stratified analyses, the rate of progression of MCAv also did not change significantly. Conclusions: Although MCAvs did not change significantly overall, 4% of children developed critical levels. Determinants of this progression remain unclear. Further studies are required to better understand the natural history of the disease and the rate of MCAv change on TCD. 62.  Reversible Congestive Venous Myelopathy due to Spinal Dural Arterio‐venous Fistula Munish Goyal, Aarti Sarwal, Mason Cummins, Niranjan Singh University of Missouri, Columbia, MO, United States Introduction: Dural vascular malformations compose 3–4% of spinal cord masses. These are often not considered in differential diagnosis of progressive myelopathy. We present a case of reversible venous congestive myelopathy due to spinal dural arterio‐venous fistula (DAVF). Results: 64‐year‐old male with chronic low back pain presented with 3‐month history of relapsing remitting weakness of both lower extremities. MRI lumbosacral spine and CT myelogram showed changes of previous lumbar diskectomy and scoliosis. He developed acute right sciatic pain, deteriorating asymmetric paraparesis with bladder retention. Examination showed areflexic paraplegia, extensor plantars, T10 sensory level, absent cremasteric and anal reflex with disability grade, Aminoff & Logue score 8. MRI thoracolumbar spine revealed non‐enhancing diffuse T2 signals from T6 through T12; prominent intradural extramedullary veins suggesting DAVF. Patient was given intravenous methylprednisone and transferred to another facility for spinal angiogram which showed spinal DAVF at right L4 with intradural vein draining into spinal canal upto mid‐thoracic level. Transarterial embolization was performed with no residual DAVF on follow up. He started improving immediately after embolization. He was discharged to rehabilitation where he continued to improve, regained bladder function and ability to walk with walker. Conclusion: This case illustrates rare natural history of spinal DAVF with relapsing remitting subacute course. Most literature reports poor outcomes in patients with DAVF with high Aminoff and Logue score and late intervention. Our patient showed significant improvement despite high disability score suggesting that myelopathy in spinal DAVF is caused by reversible venous congestion hence AVF should be treated aggressively regardless of presentation. 63.  Neuroimaging in Children with Tongue Fasciculations Izabela Koper‐Skladaniec, Paul Maertens, Jennifer Currie, Sherryl Falkos, Rosa Vidal University of South Alabama, Mobile, AL, United States Introduction: Bilateral tongue fasciculations are a hallmark of spinal muscular atrophy (SMA). The onset is usually subacute with bilateral fasciculations and atrophy. The vast majority of acute unilateral tongue fasciculations are due to idiopathic hypoglossal nerve palsies (HNP) that resolve spontaneously without atrophy. Neuroimaging findings in pediatric patients presenting with tongue fasciculations due to other causes than SMA and HNP have not been systematically reviewed. Materials and Methods: Records of all children presenting to our hospital with tongues fasciculation between 2004 and 2009 were reviewed. Ten patients between 15 years of age and early infancy were identified. Mean age of our population was 2.1 years old. None of these patients had SMA or HNP. Brain MRI, CT scan and/or ultrasound were done in 7, 8 and 4 patients respectively. Results: Neuroimaging studies were abnormal in all except one neonate with diagnosis of congenital hypoplasia of left hypoglossal nucleus. In three neonates with diagnosis of Möbius syndrome, cranial ultrasound and CT scan showed brainstem calcifications. In one neonate with spinocerebellar degeneration, CT scan and ultrasound showed progressive spinocerebellar atrophy. In two infants with spinocerebellar ataxia type 7, CT scan and MRI showed cerebellar atrophy. In one infant, MRI showed syringobulbia. In one older child with acute demyelinating rhombencephalitis, MRI showed abnormal signal in medulla. In a teenager with acute brainstem infarction, CT and MRI showed basilar artery thrombosis. Conclusion: We conclude that neuroimaging is extremely useful in distinguishing various etiologies of tongue fasciculations. Abstract 64 is intentionally omitted.65.  Venous Collaterals and Perfusion in Acute‐on‐Chronic Cerebral Venous Thrombosis Qing Hao, Neal Rao, Suman Kalanithi, David Liebeskind UCLA Stroke Center, Los Angeles, United States Introduction: The depiction of cerebral venous thrombosis, associated venous congestion and collaterals have been revolutionized by the advent of multimodal CT/MRI. Thrombolysis is often pursued in extensive thromboses as in the superior sagittal sinus (SSS), yet parenchymal lesions and capacity of myriad venous collateral outflow routes are drawing increasing attention beyond delineation of thrombosis itself. Methods: Case report of acute‐on‐chronic previously undiagnosed SSS thrombosis. Results: A 61‐year old man suffered from acute onset of severe headache and CT was performed without any diagnosis established or treatment initiated. The headache waxed and waned, particularly exacerbated by dependent head position. Intermittent, severe bouts of headache occurred every 1–2 months for 1 year until they became constant for 2 weeks before culminating with a generalized tonic‐clonic seizure. MRI revealed FLAIR hyperintensity in the left fronto‐parietal region and petechial hemorrhage in the right caudate nucleus. DWI did not demonstrate ischemia yet perfusion images displayed bilateral centrum semiovale lesions. MRV, CTV, and conventional angiography all showed extensive SSS with marked diminution of bilateral transverse sinuses and prominent venous collaterals. Review of the original CT from 1 year earlier showed the right caudate lesion and hyperdensity throughout the SSS. Anticoagulation was started in the ICU, yet potential thrombolysis was never needed and he was discharged without neurological sequelae. Conclusion: Extensive thrombosis in the SSS and transverse sinuses may cause isolated headache due to acute‐on‐chronic venous congestion. Venous collaterals and not necessarily clot extent may be critical neuroimaging features to consider in management of cerebral venous thrombosis. 66.  Changes of Cerebral Glucose Metabolism in Subcortical Aphasia after Hemorrhagic Stroke Yong Wook Kim, Ji Hoon Jang, Yu Hee Won Yonsei University College of Medicine, Seoul, Korea, Republic of Objective: The functions of subcortical structures in language are still controversial, but the several roles that basal ganglia and thalamus are essential components of the brain networks have been suggested. Subcortical hemorrhagic stroke in dominant hemisphere has known to induce aphasia by structural disconnection or by compressive ischemia among language related cortical structure. But, the functional neuroimaging researches which wish to clarify the relationship between subcortical aphasia and secondary cortical dysfunction are limited. The aim of this study was to investigate the changes of cerebral cortical metabolism in subcortical aphasia after hemorrhagic stroke in dominant hemisphsere and assess the relationship between the severity of subcortical aphasia and the associated cortical dysfunction using brain positron emission tomography (PET). Subjects and Methods: 16 right handed patients (10 men, 6 women; mean age, 52.4 years with range, 36–79 years; mean duration from onset to study, 4.1 month with range 1–7.5 momths) with left subcortical hemorrhage, who spoke Korean as their first language, were enrolled in this study. All patients underwent an aphasia evaluation using the Korean version of the Western Aphasia Battery (K‐WAB). The severity of aphasia was evaluated by calculating the aphasia quotient (AQ) for each patient by summing the score of the 4 subtests. Measurement of cerebral glucose metabolism using Brain 18 F‐fluorodeoxyglucose positron emission tomography ( 18 F‐FDG PET) was performed in the control and the all patients. Statistical analysis of PET images in the control and all patients were performed using statistical parametric mapping software (SPM2, Institute of Neurology, University College London, UK) by a voxel‐by‐voxel basis using two sample t‐test. Additionally, the correlation between the changes of cerebral glucose metabolism and the AQ was assessed using a single‐subject covariate model. Results: SPM analysis of 18 F‐FDG PET images showed that significantly decreased cerebral glucose metabolism in the subcortical aphasic patients, compared to the healthy control, was in the left superior frontal gyrus, the middle frontal gyrus, the left inferior parietal gyrus and the right cerebellar cortex ( P correctecd < 0.005). In contrast, the right inferior frontal gyrus, the right middle temporal gyrus, the right inferior temporal gyrus, the right hippocampal gyrus, the right postcentral and the right supramarginal gyrus, the right supplementary motor area and the left cerebellum ( P correctecd < 0.005). In voxel‐based SPM correlation analysis between the changes of cerebral glucose metabolism and the AQ of each patients, the most significant positive correlations were observed in the both rectal gyri and the right hippocampal and parahippocampal gyri ( P uncorrectecd < 0.01). Conclusion: Subcortical aphasia after hemorrhagic stroke showed increased metabolism in the non‐dominant hemisphere that is a homologue of expressive and comprehensive area in dominant hemisphere and showed cerebral metabolic correlation with neural networking cortex which is involved in semantic fluency and memory. These findings may support the understanding of mechanism for language recovery. 67.  Cerebral Metabolic Patterns in Permanent Vegetative State after Acquired Brain Injury Yong Wook Kim, Ji Hoon Jang, Mi Hee Park Yonsei University College of Medicine, Seoul, Korea, Republic of Objectives: Vegetative state is defined as preservation of spontaneous eye opening, sleep wake cycle, but there is complete absence of behavioral evidence for awareness of self and environment. Particularly, permanent vegetative state exhibit sustained impaired level of consciousness longer than 12 months in traumatic causes and 3 months in non‐traumatic causes of brain injury. Even though there were many studies to assess cerebral metabolic patterns in acute and persistent vegetative state, researches for cerebral metabolic patterns in permanent vegetative state there were limited. For that reason, we evaluated the changes of cerebral glucose metabolism in permanent vegetative state after acquired brain injury in this study. Subjects and Methods: 12 patients with permanent vegetative state in level of consciousness were consecutively enrolled in this study. Three patients were caused by traumatic brain injury, nine patients by non‐traumatic brain injury. We measured the level of consciousness by JFK‐Coma Recovery Scale (JFK‐CRS), and evaluated cerebral glucose metabolism by positron emission tomography scanner (GE. Milwaukee, Wisconsin, USA). Statistical analysis of cerebral metabolism in permanent vegetative state was performed by statistical parametric mapping software (SPM2, Institute of Neurology, University College London, UK) comparing to 12 normal controls and correlation analysis between cerebral metabolism and level of consciousness state measured by JFK‐CRS was evaluated by single‐to‐single covariate analysis in SPM2. Results: 1. SPM analysis showed that significantly decreased cerebral glucose metabolism in permanent vegetative state was in the left precuneus, both middle cingulum, both thalamus, the left superior parietal and the left occipital cortices, the right middle and the right inferior frontal cortices compared to normal controls ( P corrected < 0.005). 2. Statistically significant Increase of cerebral metabolism in permanent vegetative state was in both cerebellum, the right supramarginal and the left inferior temporal cortices ( P corrected < 0.005). 3. In correlation analysis between changes of cerebral glucose metabolism and level of consciousness measured by JFK‐CRS, improved level of consciousness was significantly associated with increased metabolism in both middle cingulum ( P uncorrected < 0.005). Conclusions: Our findings suggested that permanent vegetative state after acquired brain injury showed significant decrease of diffuse cerebral metabolism in the neural network of consciousness, which could be helpful to study for neural mechanism for consciousness recovery. 68.  Preserved GABA‐A Binding on 11C‐flumazenil Positron Emission Tomography in Alzheimer Disease Belen Pascual 1 , Elena Prieto 3 , Josep M. Marti‐Climent 3 , Javier Arbizu 3 , Jose C. Masdeu 2 
 1 Department of Psychiatry, Massachusetts General Hospital, Boston, Massachusetts, United States, 2 Section in Integrative Neuroimaging, National Institute of Mental Health, National Institutes of Health, Bethesda, Maryland, United States, 3 Department of Nuclear Medicine, University of Navarra Medical School, Pamplona, Navarra, Spain Introduction: [11C]flumazenil is a specific positron emission tomography (PET) tracer for the GABA‐A receptor, abundant in the medial temporal lobe, an early target of Alzheimer's disease (AD) pathology. [11C]flumazenil PET has been reported to be more sensitive and specific than diffusion‐weighted MRI for the detection of neuronal loss in stroke. We conducted a pilot study to evaluate the potential of flumazenil imaging to detect neuronal loss in AD. Patients (or Materials) and Methods: Eight AD patients and 9 matched healthy controls were studied. Mean Mini‐Mental Examination score for the AD patients was 20.57 (SD, 4.67). An arterial input function was applied to derive both the total tissue distribution volume and the binding potential of flumazenil, using the graphic plot method of Logan (2001). The distribution volume of flumazenil was analyzed using both regions‐of‐interest (ROIs) and voxel‐based analysis methods (SPM). Results: We did not find significant differences in GABA‐A receptor density between AD patients and healthy controls. The presence of dementia was not associated with a reduced distribution volume of flumazenil. Conclusion: 11C‐flumazenil PET demonstrates no apparent loss of GABA‐A receptor density in early Alzheimer disease. 69.  FDG‐PET Changes in Brain Glucose Metabolism in Patients with White Matter Disease with and without Dementia: Differentiation from Alzheimer Disease Belen Pascual 1 , Elena Prieto 3 , Josep M. Marti‐Climent 3 , Javier Arbizu 3 , Jose C. Masdeu 2 
 1 Department of Psychiatry, Massachusetts General Hospital, Boston, Massachusetts, United States, 2 Section in Integrative Neuroimaging, National Institute of Mental Health, National Institutes of Health, Bethesda, Maryland, United States, 3 Department of Nuclear Medicine, University of Navarra Medical School, Pamplona, Navarra, Spain Introduction: The boundary between vascular dementia and Alzheimer's disease (AD) continues to be unclear. Some posit that progressive vascular dementia, as with small‐vessel disease, is simply vascular brain disease plus AD. As AD presents a characteristic pattern on fluoro‐deoxyglucose (FDG) PET, we sought to determine whether the FDG pattern of vascular dementia resembled more AD or the pattern in patients with severe microvascular disease but no dementia. Patients (or Materials) and Methods: Vascular disease patients were selected on the basis of confluent white matter lesions on both hemispheres. Among them, with a similar degree of vascular disease on MRI, neuropsychological testing separated groups with dementia (WMD‐D, N = 8) and without dementia (WMD‐nD, N = 9). In addition, 8 patients with AD, and 9 healthy controls were studied. Groups were matched by age, sex and education level. FDG distribution was analyzed using voxel‐based analysis methods (SPM). Results: The AD group showed the characteristic pattern of bilateral hypometabolism in temporo‐parietal association cortex and cuneus. By contrast, patients with vascular dementia had a similar pattern to the vascular patients without dementia. However, a comparison between the two groups of vascular patients showed a greater decrease in frontal metabolism in the demented patients, a pattern quite distinct from the metabolic pattern of AD. Conclusion: FDG‐ PET seems to suggest that, at least in some instances of vascular dementia, the metabolic abnormality defining the presence of dementia involves the frontal lobe, a pattern quite different from AD and concordant with the neuropsychological findings of impaired executive function characteristic of vascular dementia.