Table of Contents
Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Necrotizing Leukoencephalopathy | 1A0 Case 1 History
Case 1 History ---- The patient was a 9 year-old boy with a history of cerebellar medulloblastoma diagnosed in May 2004 at the age of 7. He initially presented with a two week history of intermittent headache, nausea and vomiting and was treated with antibiotics for a presumed sinus infection, but his symptoms progressed. At that time, an MRI of the brain showed a cystic 5 cm in diameter contrast enhancing mass in the left cerebellar hemisphere. There was associated mild hydrocephalus. He underwent suboccipital craniotomy with resection of the mass at Saint Louis Children’s Hospital. Pathologic analysis of the mass was consistent with medulloblastoma with desmoplastic and anaplastic features. MRI of the entire spine was negative and CSF was negative for tumor cells. A ventriculoperitoneal shunt was placed for hydrocephalus. Chemotherapy with lomustine (CCNU) and vincristine was commenced. By November of 2005 surveillance imaging showed thickening and enhancement of the cauda equina suggesting leptomeningeal metastasis. Salvage chemotherapy including cyclophosphamide and temozolomide resulted in decreased enhancement of the nerve roots. In May of 2006, he was admitted for autologous bone marrow transplant in an attempt to better control his disease. Unfortunately, his post-transplant course was complicated by progressive respiratory failure requiring mechanical ventilation. He also developed elevated liver enzymes with cholestasis as well as acute renal failure necessitating hemodialysis. The clinical concern was for veno-occlusive disease. Within several days his clinical status had deteriorated such that the treating physicians felt there was little hope of survival. After they discussed this with his family, he was removed from the ventilator and subsequently expired.
