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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Necrotizing Leukoencephalopathy | 6A0 Case 6 History
Case 6 History ---- The patient was a 52 y/o woman diagnosed with poorly differentiated stage IIIa invasive ductal carcinoma in June 2006. Treatment included mastectomy and chemotherapy. Ten days after completing first round of chemotherapy the patient developed fever (at times spiking), neutropenia, thrombocytopenia, and later fatigue. Admitted for workup; 4 days post admission the patient developed new changes in mental status consistent with encephalopathy. The hospital course included ongoing fever, headache, and progressive dyspnea leading to respiratory failure. CSF revealed a mild lymphocytic pleocytosis, but infectious workup of blood and CSF were both negative. Imaging was said to be unremarkable. Treatment included antibiotics, acyclovir and steroids, and while the fever resolved temporarily, the encephalopathy remained unchanged. Prior to death the patient developed fever and septic shock. General autopsy findings included pneumonia.
Neuropathologic findings
Leukoencephalopathic changes were seen in the left hypothalamus/basal ganglia, basis pontis, and cerebellum. Foci of pale vacuolated white matter are accompanied by axonal spheroids, reactive astrocytes, foamy macrophages, and activated microglia. Mineralized neuronal processes are only occasionally seen. The white matter pathology here is consistent with a leukoencephalopathy that may be chemotherapy associated. This patient was being treated with doxorubicin and cyclophosphamide, both of which have been reported to be rarely associated with a toxic leukoencephalopathy. This is not the typical pathology of DNL, but may be related.
