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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Necrotizing Leukoencephalopathy | 5A0 Case 5 History
Case 5 History ---- The patient was admitted to the hospital at age 16, 33 months after acute lymphoblastic leukemia was diagnosed, for evaluation of a 3 day history of left-sided weakness. His previous course had been complicated by two instances of CNS relapse, and one bone marrow relapse; he had been treated with systemic, intrathecal and intraventricular chemotherapy and radiation therapy. At the time of initial diagnosis, neurologic examination was normal. Induction was achieved with prednisone, vincristine, and L-asparaginase. He was treated prophylactically with intrathecal methotrexate and 2,400 rads of whole brain radiation. For the following 20 months he was doing well on oral methotrexate and 6-mercaptopurine.
A routine lumbar puncture on 4/78 revealed a CNS relapse, although the bone marrow was normal. The spinal fluid was cleared of blast cells with intrathecal injections of methotrexate, hydrocortisone, and cytosine-arabinoside, in addition to systemic administration of vincristine, prednisone, and doxorubicin. Seventy-two hours after the last intrathecal injection, he suffered a single generalized tonic seizure. Lumbar puncture at that time showed 2 mononuclear cells, protein 60, sugar 54, negative cultures, and negative cryptococcal antigen. CT examination at that time was normal; EEG showed excessive delta and theta slowing which predominated in the left frontal area. A second course of radiation was given, 2,400 rads to the brain and 1,400 rads to the spine. He had no further seizures while being treated with phenytoin, and continued his normal activities. Routine studies in 12/78 showed both bone marrow and CNS relapse. General neurologic examinations remained normal. An Ommaya Reservoir (i.e., a catheter in one lateral ventricle attached to a reservoir implanted under the scalp) was placed. CNS remission was achieved with intraventricular methotrexate, hydrocortisone, and cytosine-arabinoside; bone marrow remission was achieved by rotating 9 different systemic chemotherapeutic agents.
In 2/79, he had a deterioration of his school grades, appeared depressed and became lost several times. Following a 3 day period of left-sided weakness, he was readmitted in March 1979. The optic discs were sharp. He was alert and oriented but had poor short term memory. A CT scan showed a medium sized area of contrast enhancement in the white matter of the right frontal lobe surrounding the tract of the ventriculostomy tube with edema and collapse of the right lateral ventricle. The ventricular catheter was removed and the patient was treated with high dose Decadron and his hemiparesis improved. Two months prior to his final admission, a repeat bone marrow showed relapse and he was restarted on Cytoxan and then did well until one day prior to admission when he developed a severe headache, papilledema and non-projectile vomiting. There was a moderate left hemiparesis with left central facial weakness. A head CT showed marked increase in the mass affect in the right hemisphere. There was irregular enhancement over the cortical sulci in the parietal lobe. He was treated with high dose Decadron but did not improve., progressively worsening culminating in death.
