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Washington University Experience | NEURODEGENERATION | Alzheimer Disease | Gross Pathology | 10A0 Case 10 History
Case 10 History ---- In September 2002 at age 62 years this female developed and self-reported memory complaints. The patient’s mother developed Alzheimer disease (AD) dementia at age 62 and died of inanition at age 75. The patient had one sibling, a 66-year-old sister, who had no memory difficulty. The patient admitted “concern about my memory” and reported that she had difficulty in recalling conversations. She also reported low mood, irritability and “mood swings”. Her MMSE score was 29. Her physician attributed the patient’s self-reported memory complaints to stress and anxiety and recommended Zoloft. The next year she was rated as cognitively normal (CDR 0). She returned again to the MDC at age 69. Her husband now noted both cognitive and behavioral changes in his wife that he believed had gradually developed and progressed since she was 67yo. She communicated less, was quiet, and was forgetful with frequent repetition. In addition, she had personality changes. She was more irritable and had aggressive “outbursts”; she had struck her husband on several occasions. She was diagnosed with AD dementia with a CDR of 1. A head MRI scan (August 2009) at an outside institution was interpreted as showing “generalized cortical atrophy”. By September 2009 she would not let her spouse leave home. An outpatient psychiatric evaluation elicited a history of increasing hostility over the past two months with agitation, anxiety, and aggression (hitting her spouse). During his office evaluation, she became agitated and profane and began hitting her husband; security was called, and she was admitted to the inpatient geropsychiatry unit at BJH. She was discharged from the geropsychiatry unit to a facility on olanzapine. Due to her apparent rapid progression; a CSF 14-3-3 evaluation was negative for prion disease. An MRI scan showed ventricular dilatation ex vacuo. A physician attending her in the residential home in 9/18/12 indicated that she was asked to evaluate the patient’s “weight loss and change in condition”. The 72yo patient reportedly had a “rapid decline” over the past several months with weight loss, tremors and becoming non ambulatory. She was placed on hospice. The purported cause of her dementia was reported as both AD and Lewy body disease. The patient was unresponsive to commands. Slight contractures of the knees and elbows were noted. The elbows were “difficult to passively extend”. The patient responded occasionally with a single word. She was unable to stand. Repeat evaluation on November 2012 noted pressure ulcers on her buttocks and “failure to thrive”. The patient died in December 2012 at age 72 at home where she had been for slightly more than three years. The presumptive cause of death was inanition. It was thought that the patient’s initial self-reported memory complaints that led to the initial MDC evaluation in September 2002, were the initial expression of her dementing illness, she had symptomatic onset at 61 years of age and a total duration of 11 years. She reported a family history of dementia in her mother with the approximate age of onset at 62 years and a total course of 13 years. The phenotype suggested AD dementia with gradual onset and progression (forgetfulness, repeating questions and statements, forgetting conversations, forgetting appointments, misplacing items) and functional loss (unable to prepare meals at her usual level, learn the operation of a cell phone, or drive). An unusual aspect of her dementia was the prominent agitation, anxiety, and aggression. She also had extrapyramidal signs, including hypomimia, bradykinesia, and cogwheel rigidity. Because the cognitive complaints (even though largely self-reported) began years prior to the onset of extrapyramidal dysfunction, it was thought that the primary diagnosis should be AD dementia with later complications of parkinsonism and atypical behavioral disturbances. These behaviors are more common in more progressed AD patients.
