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Washington University Experience | VASCULAR | Subcortical Arteriosclerotic Encephalopathy | 3A0 Case 3 History
Case 3 History ---- The patient was in good health with a history of migraine headaches. The family noted a flattened affect and depression in 1995. In 1996 she was evaluated by a neurologist for unwitnessed episodes of loss of consciousness that were considered the onset of a seizure disorder including a tonic-clonic seizure while asleep. She developed a dementing process and had become incontinent of bowel and bladder. Her APOE was 2/3. Her physician’s neurological examination was significant for mild parkinsonism with masked facies, slightly decreased arm swing, and slowed gait. There was some mild cogwheeling in bilateral upper arms and paratonia in all four extremities. Her physician rated her as a CDR 2. MRI scans of the brain were performed in Sept/Oct 1996 and showed ventricular dilatation in the lateral ventricles and white matter abnormalities, both periventricularly and also extending into the subcortical area, mostly in the frontal areas. A cisternogram was compatible with communicating hydrocephalus and her neurosurgeon placed a right ventriculo-atrial shunt in July 1997. The shunt placement did not result in improvement cognitively or physically. By 1998, she was severely demented, wheelchair bound and required total assistance for personal care. Her clinical diagnoses at the time of death were frontotemporal dementia with subcortical features of parkinsonism and seizure disorder (CDR 3, severe dementia). She expired in November 2003 at age 63. ---- At autopsy the fresh brain weighed 1050 grams. No senile plaques, neurofibrillary tangles, or vascular amyloid deposits were present in any neocortical regions using a-beta and PHF tau immunostains. There was no evidence for Lewy bodies or frontotemporal degeneration. ---- This is a most unusual case with almost exclusive devastating white matter pathology with parallel loss of myelin and axons, confluent enough to pass for a leukodystrophy and no evidence of additional neurodegenerative disease in an apoE 2/3 woman with a 7 year course that progressed to severe CDR 3 dementia. There was no family history of dementia. The frontal horns were widely dilated at the time of autopsy in spite of right frontal shunt placement, providing no evidence for normal pressure hydrocephalus (NPH).
