Neurocutaneous melanosis is an uncommon disorder that is classified among the phakomatoses. Fully expressed, it consists of multiple cutaneous nevi and a proliferation of melanocytes in the leptomeninges.1 A forme fruste is isolated benign melanosis of the meninges, which may undergo malignant transformation, most often during adult life.2 We report the unusual clinical course and computed tomographic (CT) findings in a case of presumed primary malignant melanomatosis of the meninges without skin nevi in a child.
REPORT OF A CASE
A 41/2-year-old girl had recurrent headaches in July 1979, and a tentative diagnosis of migraine was made. Recurrence of symptoms in September led to complete examination that included a CT scan, which was normal. Soon afterward, she was admitted to a hospital following a seizure. Lumbar puncture (LP) revealed a pressure of 300 mm H2O, 30 WBCs, 20 RBCs, a glucose level of 30 mg/dL
Crisp DE, Thompson JA. Primary Malignant Melanomatosis of the Meninges: Clinical Course and Computed Tomographic Findings in a Young Child. Arch Neurol. 1981;38(8):528–529. doi:https://doi.org/10.1001/archneur.1981.00510080090015
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: