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Washington University Experience | NEURODEGENERATION | NBIA1 (PKAN, Hallervorden-Spatz Dz) | 3A0 Case 3 History
Case 3 History ---- This man was seen in March 1995 at age 84 years. There was a family history which included his mother with memory problems, a brother with Parkinson’s disease and memory problems, and a sister with dementia at an age of 72. His medical history was significant for a partial colon resection for cancer in 1981, adult-onset diabetes, and a history of orthostatic hypotension which was under control. He had a gradual onset and progression of memory and thinking difficulty for at least 6-9 months. In April 1995, he had a motor vehicle accident without loss of consciousness which was his fault. His wife stated that she noticed memory changes shortly after this, but she had been questioning his judgment while driving before the accident happened. He became more short-tempered and dependent on his wife. There was a very slow progression of memory loss. In January 2003, he had two falls, hitting his head (with possible concussion) and a clear decline since the falls. At 95, his Clinical Dementia Rating (CDR) was 2, dementia of the Alzheimer type. In January 2007, he moved to a nursing home after being hospitalized with delirium for possible pneumonia or UTI. A brain CT scan done at that time showed cerebral cortical and cerebellar atrophy as well as scattered microvascular changes and chronic-appearing ischemic changes. He had an episode of decreased consciousness related to low blood sugar and possible aspiration for which he received IV antibiotics. He died of aspiration pneumonia at age 97. In summary, he showed very slowly progressive cognitive decline over 12 years with no sign of stroke, PD, or seizures.
