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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | Inflammatory Demyelination | 7A0 Case 7 History
Case 7 History ---- The patient was a 33 year old man with acutely progressive CNS dysfunction and amblyopia culminating in global aphasia and posturing. The process is characterized by widespread white matter damage on MRI. A trial of high-dose steroids was ineffective. There is no evidence of systemic disease and multiple serologies were non-contributive. At that time the patient was biopsied and a diagnosis made of acute inflammatory demyelination.
There was recent progression over the course of one year since the biopsy with rapid deterioration one month prior to his final admission. At that time the patient presented with severe right hemiparesis, global aphasia, and the inability to follow simple commands. He had decerebrate posturing to pain on the right and normal withdrawal on the left. Pertinent laboratory tests included a negative work-up for adrenoleukodystrophy. During the course of hospitalization, the patient received a total of 10 days of high dose methylprednisolone; however, his course continued to deteriorate and he expired shortly thereafter.
7A-D represent the results of his neurosurgical biopsy
7E-H the results of autopsy.
