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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Neuroaxonal Leukodystrophy | 2A0 Case 2 History
Case 2 History ---- This patient complained of an inability to focus and difficulty comprehending newspaper articles about one year before he was evaluated at the Washington University ADRC at 36 years of age. He had a number of cognitive problems according to his spouse who also noted difficulty with gait and weakness, particularly in his upper extremities. Psychometric testing showed mild impairment in executive function and speeded psychomotor performance. His head MRI demonstrated diffuse atrophy. The differential diagnosis included dominantly inherited neurodegenerative dementing disorder including atypical Alzheimer’s disease, frontotemporal dementia (specifically Pick’s disease), and corticobasal degeneration. He was admitted to a residential care facility in 1999 and transferred to a nursing home in 2001. His sister stated he had seizures during the last year of his life. He was placed under hospice care and died of respiratory failure at age 43. He had been mute for 3 years, required total care, and had severe dementia.
This man had a strong family history of dementia. His mother had a dementing illness, age of onset 49, and his sister (previous case #1) had severe dementia.
