Premium
[P3–277]: THE ASSOCIATION BETWEEN NEUROPSYCHIATRIC SYMPTOMS, APOE ε4 AND PROGRESSION OF ALZHEIMER'S DISEASE
Author(s) -
Liu YiChien,
Fuh JongLing,
Meguro Kenichi
Publication year - 2017
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2017.06.1491
Subject(s) - apolipoprotein e , clinical dementia rating , memory clinic , medicine , neuropsychiatry , neuropsychology , dementia , psychiatry , alzheimer's disease , disease , logistic regression , rating scale , psychology , cognition , developmental psychology
Background: Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD also may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. We therefore reviewed our extensive experience with patients with SIH and bvFTD. Methods: Using a prospectively maintained registry, we identified all patients with SIH (based on the criteria of the International Classification of Headache Disorders, third edition [ICHD-III]) who met clinical criteria for bvFTD (International Behavioral Variant FTD Criteria Consortium [FTDC]). Patients or their caregivers/family were contacted for follow-up. A modified Migraine Disability Assessment Score (MIDAS) questionnaire was used to assess the severity of the symptoms (Grade I or II: good outcome; grade III or IV: poor outcome). The patients were compared to a cohort of SIH patients without bvFTD. Results:The mean age at onset of SIH symptoms for the 21 men and eight women was 50.6 years (range, 34-65 years) and it was 52.9 years (range, 37-65 years) at the time of onset of bvFTD symptoms. All patients had hypersomnolence. MRI showed brain sagging in all patients, CSF opening pressure was low in about half of patients, but a spinal CSF leak could not be detected in any patient. Three patients only underwent epidural blood patching, with a good outcome in one patient. After unsuccessful directed percutaneous treatments, the remaining 26 patients underwent one or more surgical procedures with a good outcome in 20 patients (77%). Overall, a good outcomewas obtained in 21 patients (72%); 20 (91%) of 22 patients who had not undergone prior Chiari surgery compared to one (14%) of seven patients who did undergo Chiari surgery (p<0.003). Compared to SIH patients without symptoms of bvFTD (n1⁄4547), those with bvFTD symptoms were older, more often male, less often demonstrated a CSF leak on spinal imaging, and more often underwent surgery (p<0.02). Conclusions: bvFTD in SIH is rare and associated with brain sagging and hypersomnolence. Spinal CSF leaks are rarely detected. bvFTD symptoms are often refractory to the usual percutaneous procedures but most patients can be cured, although one or more surgical treatments are generally required.