Abnormal Enhancement of the Left Putamen on Brain MRI in a Case of Proven Creutzfeldt-Jakob Disease

Abnormal Enhancement of the Left Putamen on Brain MRI in a Case of Proven Creutzfeldt-Jakob Disease Dr. Horng-Huei Liou, MD, Department of Neurology, National Taiwan University Hospital, No. 7 Chung-Shan South Road, Taipei 100 (Taiwan) Creutzfeldt-Jakob disease (CJD) is caused by unusual protein-aceous infectious particles, or prions [1]. Criteria for the clinically definite diagnosis of CJD include rapid-evolving dementia, myoclonus, periodic electroencephalographic activity (1–2 Hz) and finally death within 12 months [2]. Magnetic resonance imaging (MRI) of CJD cases show increased signal intensity in the bilateral basal ganglia on T2-weighted images [3–6]. We report here a case of pathologically proven CJD, showing abnormal enhancement in left putamen on godolinum-diethylenetriamine pentaacetic acid (Gd-DTPA) Tr weighted MRI (T1WI), which has not been reported before. A 52-year-old male truck driver became progressively forgetful and aphasic over a 6-week period. He then gradually developed dressing and gait apraxia; urinary incontinence and visual hallucinations were also reported. Startle myoclonus induced by auditory or visual stimulation and spontaneous generalized myoclonus or focal seizure were seen during this period. He became bedridden and detached from others 10 weeks later. On examination 11 weeks after the onset of symptoms, the patient was in a vegetative state with stable vital signs. Neurological examination revealed preserved brain stem reflexes and obvious primitive reflexes. There was generalized hyperreflexia with bilateral ankle clonus and Babinski signs. No focal weakness was detected. In addition, he had episodic generalized myoclonus, generalized tonic-clonic seizures and focal seizures, which occurred separately. Normal values were reported for the blood cell count, chemistry profile, thyroid function, tumor marker screen, ceruloplasmin, and lumbar puncture. Immunological profiles, serologic test for syphilis and screening for AIDS were also normal. EEG on admission showed generalized continuous periodic sharp-wave complexes with an interval of 1–2 s. Two weeks later, EEG showed almost continuous periodic generalized stereotypic sharp waves, with a recurrent rate of 0.5–0.6 Hz, and short periods of suppression burst pattern. Computerized tomography, 12 weeks after onset, was unremarkable. MRI (1.5 T, Signa, General Electric), 14 weeks after onset, showed multiple abnormal signal lesions bilaterally in the putamen, caudate nucleus, and hippocampus. These appeared bright on the T2-weighted images (T2WI) (fig. 1), and the lesion in the inferior aspect of the left putamen showed abnormal enhancement using Gd-DTPA (fig. 2). The ventricles and sulci were dilated. No mass effect was seen. A biopsy of the right frontal lobe, performed 3 months after onset, showed cerebral atrophy. Microscopically, diffuse neuronal loss, striking vacuolation, and hypertro-phied astrocytes of the gray matter were seen. No known mutation of the prion protein (Prp) gene [7] was found in the DNA analysis of either the patient or his family members. In spite of intensive treatment, the patient died 4.5 months after the onset of symptoms.