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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Neuroaxonal Leukodystrophy | 3A0 Case 3 History
Case 3 History ---- The patient was a 75 year old man who presented at the age of 65 with personality changes, becoming more withdrawn, short-tempered, forgetful and moody. Neurology consultation noted that he was walking slower with a shuffling apractic gait and that he had urinary difficulties, including urgency and occasional incontinence. An MRI from this time, reported a prominent ventricular system and prominent cortical sulci, although the ventricular system did appear more dilated than the sulci were atrophic. There are numerous high signal MRI areas in the white matter bilaterally consistent with small lacunar infarcts or ischemic changes including sites in the pons and cerebellum. A trial of CSF removal resulted in mild improvement suggesting NPH but improvement did not last and a second trial produced no changes.
Focal, left-sided seizures with secondary generalization developed. A note from less than a year from his death described him as mute, bedridden, and doubly incontinent. He died with a diagnosis of aspiration pneumonia and dehydration. Final clinical diagnoses were vascular dementia and corticobasal degeneration.
