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Washington University Experience | VASCULAR | Subcortical Arteriosclerotic Encephalopathy | 7A0 Case 7 History
Case 7 History ---- This 77 year old woman had been seen for possible parkinsonism and dementia. She had a history of hypertension and recurrent depression with hospitalizations and ECT with good benefit. She experienced visual hallucinations post-ECT treatment (“flames”). At that time, her neurological examination showed cogwheel rigidity in bilateral upper extremities as well as diminished rapid fine finger movements bilaterally and a parkinsonian gait. She also had complaints of small handwriting. She had a syncopal reaction to a trial of Sinemet. Psychometric testing done in October of 1997 showed global mild impairment with deficits in episodic memory, executive function, and speeded psychomotor performance. She recalled none of the five item memory phrase. The clinician rated her CDR 0.5 and diagnosed DAT with depression contributing. At her final visit, her neuropsychiatric problems were the major symptoms. In summarizing her history, the clinician stated that she had a slowly progressive cognitive decline for 6 years prior to her final admission. Since at least 9 years prior she experienced visual hallucinations, frequent falls, extrapyramidal signs, and a fluctuating cognitive status which would meet criteria for DLBD. Her neuropsychiatric difficulties (multiple hospitalizations for psychotic depression and ECT beginning in 1961) overshadowed her memory problems and complicated the differential diagnosis. Her CDR was 3 at the time of death. In 8/2007, she was hospitalized following a grand mal seizure and possible aspiration. She was discharged to a nursing home with hospice care. She died at age 87 of aspiration pneumonia with an advanced dementia (CDR 3). Her primary neurologist thought her neuropathological diagnosis would be DLBD with possible Alzheimer’s disease as a concomitant disorder. She also had several lacunar infarcts as seen on her 8/06 brain MRI. Overlying these neurodegenerative diseases was her long psychiatric history of psychotic depression which may have contributed to her dementia. ---- At autopsy her brain weighed 1220g. The main neuropathological findings of this case are Alzheimer’s disease neuropathologic change and small vessel disease with multiple infarcts. No to focally numerous diffuse beta-amyloid plaques are present in the neocortex, but there is an absence of neuritic plaques, and there are only sparse neurofibrillary tangles which are largely confined to the medial temporal lobe. These findings indicate the presence of Alzheimer's disease (AD) by Khachaturian criteria, but the absence of neuritic plaques does not fulfill CERAD, NIA-Reagan Institute or NIA/AA criteria for the neuropathological diagnosis of AD. Vascular pathology in the form of marked arteriolosclerosis and infarction is also a feature of this brain and probably contributed to the motor and other deficits. Lewy and Pick bodies were not present. No other neurodegenerative diseases were identified. The cognitive deficits experienced during life are not explained by the modest Alzheimer's disease pathology. The two types of lesions, Alzheimer-type changes and vasculopathy, in combination may have produced cognitive deficits beyond what would have resulted from either alone."
