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Washington University Experience | NEOPLASMS (EMBRYONAL) | Medulloblastoma, Histologically Defined | Large cell-Anaplastic (LCA) | 6A0 Case 6 History
Case 6 History ---- The patient was a 14 year old male with a history of Moyamoya syndrome diagnosed in early 11/97. He initially presented from an OSH to the ER at SLCH with a 3 week history of new onset slurred speech associated with headaches. A CT scan showed a mass in the cerebellar vermis. An MRA was performed showing occlusion of the circle of Willis, which was followed by emergency angiography prompting a diagnosis of Moyamoya syndrome. The patient was discharged but returned 10 days later with vomiting and a TIA with residual right-sided weakness. He was treated with aggressive pressure management in order to maintain systolic blood pressure over 70 mm Hg. The patient developed multiple infarcts with altered mental status and a decrease in spontaneous movement of the extremities over his remaining course. In 12//97 encephalodural arteriosynangiosis (ECAD) was performed in the left temporal area. A brain CT, following this procedure showed a new lacunar infarct in the right caudate nucleus and right putamen. An old left frontal infarct and old right high frontal infarct were also visualized. In the following days he developed ileus, fluctuating hypertension, diaphoresis, hyperpyrexia with posturing, lasting between 15 minutes and 18 hours. The diagnosis of exclusion at this time was diencephalic storm unresponsive to Morphine, Bromocriptine, Propranolol, and Tegretol. The abnormal posturing was consistent with bilateral basal ganglia infarction. There was worsening of his neurological status with loss of spontaneous movement and increased opisthodomos. In 1/98, Glasgow coma scale (GCS) was 7 (4-eye movement, 2-motor, I-visual). His course progressed relentlessly with loss of upper brainstem function and hypothalamic function causing central diabetes insipidus on 1/98. A CT brain at this time showed massive infarction of the supratentorial brain. The gravity of the situation was discussed with the parents who declined aggressive intervention and tissue donation. The patient expired in January 1998. ---- At autopsy the weight of the unfixed brain was 1,650g. The gyri were flattened, the sulci narrowed and the brain edematous.
