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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Necrotizing Leukoencephalopathy | 3A0 Case 3 History
Case 3 History ---- This was a 64 year-old male with past medical history of Wegener's granulomatosis and rheumatoid arthritis. He was transferred from an OSH in September 2002 to BJC after diagnosis of lymphoma and renal failure. A few days prior to his presentation to the OSH, he had several weeks of fatigue and a few days of change in mental status that led to bone marrow biopsy resulting in the diagnosis of mantle cell lymphoma. He was oriented x 2, somnolent but could be awakened easily. His cranial nerves and motor function were grossly intact. Laboratory results from outside hospital showed: BUN/Cr= 34/2.8, WBC=45 with 62% blasts. The CSF from lumbar puncture showed: 6 white cells and protein of 64, Head CT and EEG were normal. On his first hospital day, he developed fever with platelets decreasing to 97,000 and became less responsive. By next morning, he required intubation for airway protection. An EEG documented moderate to severe generalized slowing. He developed fever up to 40.2oC and he was placed on broad spectrum antibiotic coverage including acyclovir. Repeated lumbar punctures were negative for HSV, CMV, or Cryptococcus. Cytology studies showed acute inflammatory cells in a background of scattered lymphoid and monocytoid cells. Brain MRI was normal. Over the next three days, he became afebrile, his white count decreased and sedation was weaned. However, the patient was not responsive. His brainstem reflexes were intact; however, his sodium was increased to 156. It was unclear at that time if his mental status changes were related to infection, lymphoma and/or elevated sodium. The repeat lumbar puncture was consistent with mantle cell lymphoma with CNS involvement. He was started on intrathecal methotrexate. His examination remained about the same except for some non-purposeful eye opening. He developed fever again without a clear source and his sodium remained elevated. After about a week without improvement, family discussions lead to withdrawing care with the cause of death listed as mantle cell lymphoma with CNS involvement.
