Malignant meningeal melanomatosis with possible case of neurocutaneous melanomatosis.

Headache and an abnormal brain MRI in a patient with history of seizure disorder can have a large differential diagnosis. We report a rare case of malignant meningeal melanomatosis with associated neurocutaneous melanomatosis presenting as such. A 35-year-old male with history of seizure disorder was admitted with continuous headache, nausea, vomiting, and significant weight loss of about 100 pounds over a period of 3 months. His past history was significant for longstanding seizure disorder, noncompliance, several ER visits, drug abuse and recent hospitalization with diagnosis of intracerebral hemorrhage, which was complicated by hydrocephalus requiring VP-shunt. Brain MRI was abnormal showing high signal intensity in the right temporo-parietal area. A brain biopsy and CSF studies showed inflammatory cells, lymphocytosis, gliocytosis and negative for malignancy at the time. He was presumed to have neurosarcoidosis and was started on high dose prednisone. He was on prednisone, temazepam, promethazine and divalproex. On initial evaluation his vital signs were stable; he was restless and cachectic. There was hyperextension, arching of his neck and significant neck rigidity but otherwise alert and oriented and seemed in moderate distress secondary to pain. Laboratory data were normal and it was difficult to do imaging of his head because the patient was unable to lie flat and was restless. Hospital course: His previous brain biopsies and CSF were reviewed by another pathologist and found that the patient had either carcinoma melanomatosis or high-grade glioma. It was decided to have palliative chemotherapy and radiation therapy especially to improve his neck extension, opisthotonos and to make him more comfortable. The patient had complicated hospital course. Although his neck extension improved significantly with radiation therapy and chemotherapy, the patient deteriorated, requiring mechanical ventilation, developed syndrome of inappropriate ADH and subsequently died of respiratory failure. The final diagnosis was revealed on autopsy, which showed malignant meningeal melanomatosis with involvement of spinal cord and also involving his right parietal area and cerebellum with recent hemorrhage. Multiple melanotic skin lesions were noted, however no cutaneous malignant melanoma was observed. This may represent a case of neurocutaneous melanomatosis, a rare condition, mostly fatal in nature and diagnosed often on autopsy, without any treatment options.

Khalil A. Amir, Gary Harpold, Linda Thomas, M. Mir-Kazimov, and Edward Arsura. University of Virginia School of Medicine, Roanoke Salem Program, Veterans Affair Medical Center, Salem, VA.