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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Necrotizing Leukoencephalopathy | 4A0 Case 4 History
Case 4 History ---- The patient was a 26 year old female admitted for evaluation of intracranial masses. Her past medical history was completely benign until December of 1977, when symptoms of weakness and malaise with weight loss led to a diagnosis of acute myelocytic leukemia. She was initially treated with Adriamycin and ARA-C with remission. She had consolidation therapy in January and February of 1978 with the same drugs. In March of 1978 she had bone marrow cryopreservation done at Barnes Hospital. In September of 1978 while still on maintenance chemotherapy a surveillance LP revealed leukemic cells. She was treated with intrathecal ARA-C and methotrexate and 3,000 rads of whole brain irradiation. The systemic chemotherapy was discontinued in April, 1979. However, intermittent intrathecal therapy was still being performed when, in late April of 1979, the patient complained of visual problems and had decreased mental status and memory deficits. CT scan showed two lesions in the suprasellar cistern, hypothalamus and the right medial temporal lobe. She was given an additional 2,200 rads of whole-brain irradiation with no improvement on examination or CT appearance. Shortly after admission angiography showed evidence of mass affect bilaterally, involving the right uncus and the left basal ganglia. Bone marrow biopsy showed recurrent, acute, myelomonocytic leukemia. Because of the finding of recurrent disease in the bone marrow the brain biopsy was cancelled and it was elected to treat the patient with high dose BCNU therapy and autologous bone marrow transplant. The neurologic examination showed only bitemporal hemianopsia, bilateral optic pallor and apparent distal peripheral neuropathy. She received additional BCNU on three successive days and an autologous bone marrow transplant. Shortly after, she exhibited decreased mental status and memory with loss of vision in the left eye. A CT scan done on 7/2/79 showed lucency of the white matter which was more marked on the left hemisphere than the right, with possible involvement of the inferior border of the thalamus and the medial temporal lobe on the left. The masses in the suprasellar region appeared to be less distinct in this study. The patient's condition continued to deteriorate. Blood cultures showed gram negative rods. Shortly thereafter the patient was found without vital signs.
