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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Binswangers | 1A0 Case 1 History
Case 1 History ---- This woman was first seen in 1997 at age 57 with a two year history of a flat affect and a depressed appearance which was treated with nortriptyline. Soon she began to have difficulty with activities of daily living. In 1996 she was evaluated by a neurologist for the new onset of a seizure disorder with tonic-clonic presentation. She became incontinent of bowel and bladder and was disoriented to time but would usually know the year. Although she was able to dress and feed herself, she could not participate in the community independently. At that time there were mild Parkinsonian symptoms with masked facies, slightly decreased arm swing, and slowed gait. She was rated CDR 2. MRI scans showed evidence of ventricular dilatation in the lateral ventricles and significant white matter abnormalities, both periventricularly and also extending into the subcortical area, particularly in the frontal areas. The patient was thought to have classic symptoms of normal pressure hydrocephalus, improving clinically after a high volume lumbar tap, but subsequent right ventriculoatrial shunt did not result in maintained improvement. CSF analysis of 14-3-3 was negative. By 1998, the patient was severely demented and wheelchair bound. At that time she was thought to have FTD with subcortical features, CDR 3. From 2000-2002 she had several episodes of seizures and was hospitalized twice. The patient was last seen in 2003. At that time she was profoundly demented. She had contractures of her upper extremities. She had a dystonic facial expression and positive suck and grasp reflex. Her muscle tone was rigid with paratonia and cogwheeling. She had marked apraxia of all limbs and she was mute. She expired in 2003 at age 63. Her clinical diagnoses at the time of death were frontotemporal dementia with subcortical features of parkinsonism and seizure disorder. She was CDR 3 (severe dementia).
Her autopsy showed, almost exclusively, devastating white matter pathology and no evidence of Alzheimer Dz, Lewy-body Parkinson Dz or frontotemporal dementia. There was no family history of dementia. The microvascular pathology in the white matter and the infarctive-ischemic nature of the white matter lesions suggested Binswanger’s subcortical leukoencephalopathy and not CADASIL, MS or leukodystrophies. There was no history of hypertension. The severe loss of white matter substance suggests hydrocephalus ex vacuo and not normal pressure hydrocephalus.
