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Washington University Experience | NEURODEGENERATION | Pick Disease | 7A0 Case 7 History
Case 7 History ---- This right-handed male was first evaluated at Memory Diagnostic Center (MDC) in 1988 at the age of 52. The patient had a progressive decline in memory and thinking for about 3 years. In early 1987 he had difficulty with his employment responsibilities with lack of motivation and he was fired. In 1987 a neurologist, aided by a CT showing "selective bifrontal atrophy“, made a diagnosis of Pick’s disease. The patient ceased driving, getting lost in familiar neighborhoods. The patient gained 15 pounds over the course of the year and was described as a compulsive eater and drinker. He had poor responses to judgement and problem solving questions and became agitated. He was unable to write a spontaneous sentence or draw a cube. Physical exam revealed lack of initiation of speech, a "slightly juvenile“ affect with frequent giggling when unable to answer questions. No CDR was assigned. After his November 1988 visit he received the diagnosis of global degenerative dementia of the Pick-Alzheimer type. He had a B-12 deficiency of uncertain etiology, on replacement Rx. The patient had permanent nursing home admission in February 1989 due to escalating violent behavior towards staff and residents. In January 1991 he was getting lost in the facility. He consistently knew family but could not remember events of 15 minutes prior. He had experienced falls in the later months of 1990 and family witnessed a generalized seizure in January 1991. The patient was able to only follow one-step commands, had receptive aphasia and demonstrated a repetitive behavior disturbance. The only positive finding was a marked snout reflex. He was rated a CDR 3, diagnosis frontal lobe atrophy with severe dementia, aphasia and behavioral disturbances of repetitive activity, possible Pick’s disease, AD or "nonspecific“ dementia, and apparent generalized tonic-clonic seizures presumed secondary to advanced dementia. He developed left hemiparesis in May 1991. A CT scan revealed bilateral subdural hematomas for which the family declined surgical treatment. At that time history indicated that he was mute, displayed no facial recognition of family, was fed by staff, and was wheelchair dependent. He died at age 65, in December 2001 due to limited oral intake for 2 months. This gentleman had a 15-year course of a gradually progressive cognitive impairment with insidious onset. His presenting symptoms of apathy, disinhibition, and abulia with symptoms of perseverations and motor impersistence are the clinical picture of a frontotemporal dementia. There is also supportive evidence provided by the 1987 CT scan that demonstrated mild but symmetric disproportionate frontal lobe atrophy. Although language changes were not prominent in the early stages, the patient ultimately demonstrated receptive aphasia and became mute.
