Table of Contents
Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Neuroaxonal Leukodystrophy | 1A0 Case 1 History
Case 1 History --- This 49 year old woman had a medical history significant for a questionable (likely not) left cerebral CVA, hypertension, dementia, psychosis and epilepsy. Her family history is significant for early onset dementia in her mother at 49 and her brother at 36 (see next case). The patient had a significant psychiatric history complicated by major affective disorder beginning at age 12, requiring 10 years of psychiatric hospitalization. She developed hallucinations. The patient's speech became slurred and progressed to mutism. She was discharged to a nursing home where her dementia gradually progressed. She expired due to complications of pneumonia and cachexia. Her brain specimen, weighing 1245 grams unfixed, received a diagnosis of Alzheimer disease. With the later recognition of her brother’s illness (see Case #2), her autopsied brain was re-reviewed. Her brain showed minimal amyloid deposition in the neocortex and tangles limited to the entorhinal cortex, findings which did not fulfill the criteria of Alzheimer's disease. Instead she received the diagnosis of “Adult onset leukoencephalopathy with neuroaxonal spheroids”.
