Clinical,cerebrospinal fluid and imaging characteristics of diffuse meningeal melanomatosis:a report of 5 cases / 中华神经科杂志

ABSTRACT

Objective To investigate the clinical, cerebrospinal fluid and imaging characteristics of 5 cases of diffuse meningeal melanomatosis.Methods The clinical manifestation, features of cerebrospinal fluid and image of 5 patients with meningeal melanomatosis diagnosed by biopsy or autopsy were retrospectively summarized.Results Clinical manifestations of these 5 cases included intracranial hyperpressure, meningeal irritation sign, intracranial nerves impairment, root pain of spinal nerve.In all of these 5 cases, retina hyperpigmentation above left discus opticus was found by funduseope in one case, and congenital melanocytic nevi were found in 4 patients, in which 2 cases were giant congenital melanocytic nevi.Increased lumbar puncture cerebrospinal fluid(CSF)pressure occurred in all cases.Subarachnoid hemorrhage was found in 3 cases.Analysis of CSF revealed increased protein in 4 cases and decreased glucose in 3 cases.Cranial MRI obtained after the intravenous administration of Gd-DTPA showed leptomeningeal enhancement.Malignant melanoma cells were found in CSF of 3 cases.Metastatic malignant melanoma cells were found by biopsy of axillary fossa lymph node in one case.Autopsy of one case revealed diffuse black pigmentation of the leptomeninges, especially in base of skull.Two cases were diagnosed as metastatic meningeal melanomatosis and 3 cases were possible primary meningeal melanomatosis. Conclusions Menings, root of cranial nerve and spinal nerve are impaired in meningeal melanomatosis, which is usually accompanied by congenital melanocytic nevi.Subarachnoid hemorrhage implies meningeal melanomatosis.Diagnosis can be identified when malignant melanoma cells are found in CSF.