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Washington University Experience | NEURODEGENERATION | Corticobasal Degeneration (CBD) | 6A0 Case 6 History
Case 6 History ---- This retired accountant was seen at the Memory Diagnostic Center (MDC) in 11/2005 at age 67years. His parents both had dementia, age of onset unknown. He had a history of excess alcohol intake and prostate cancer. There was a gradual history of cognitive decline over three years which began as short term memory loss. His family noted behavioral problems of disinhibition, yelling in public, agitation, and arguing. He was hyperactive, would perseverate in doing tasks, and would pace. There was anxiety but no depression. He had expressive language problems but no difficulty with comprehension. There were no hallucinations, delusions, or falls. His neurologic exam was unremarkable. Psychometric testing showed impairments in executive function and semantic memory. His MMSE score was 29, Logical Memory was 3, and Short Blessed Test score was 1. The clinician rated him CDR 1 with a diagnosis of frontotemporal dementia. Razadyne was initiated. The family called the MDC to report increased agitation and outbursts in February 2006. Zoloft was started. In March 2006, he was agitated during daytime hours and up at night pacing. He yelled and refused to cooperate. The clinician ordered Seroquel. When he returned to MDC in 2/2007, the CS stated he was repeating within minutes to hours. He was misplacing items and had decreased attention span and organizational skills. There was language difficulty with stuttering and paraphasic errors. He had trouble with tasks that required several sequential steps. There was a slight resting tremor but no other evidence of extrapyramidal disorder. His MMSE score was 25 and Logical Memory was 0. The clinician rated him CDR 1, FTD. MRI showed atrophy, but it was not noted to be greater in any particular lobes. By his 2/2008 MDC visit, there was a decline in language and memory. He could not be left alone for safety reasons. He could only follow basic directions and would have trouble recalling recent events and conversations. He would have geographic disorientation in familiar areas and sometimes in his own home. He was not socially appropriate and would need help with ADLs. Behavioral problems included agitation and eating compulsively. In testing, he had difficulty following commands and would wander if left unattended. His psychometric scores were consistent with moderate dementia with significant aphasia. The clinician rated him CDR 3, DAT, but stated frontotemporal dementia could not be excluded. He moved to a nursing home in April 2008 and died in 10/2010 at age 72 of inanition while under hospice care. His wife reported that he seemed to know family members but that he had no short-term memory. He was unable to participate in nursing home activities and required total care (CDR 3). He had some parkinsonism including rigidity, shuffling gait, and falls. There was no indication of stroke. Because of his early age of onset (64), fairly rapid decline, and pronounced language and behavior problems, the clinical diagnosis was FTLD,
