Neurocutaneous melanosis: Review of a rare non-familial neuroectodermal dysplasia with newer association of cerebellopontine angle cistern lipoma

Abstract

Neurocutaneous melanosis is a rare neuroectodermal dysplasia with a grave prognosis. It is actually a disorder of neuronal migration at the time of the embryogenesis hence classified as a neurocristopathy. The patients are initially identified by the skin manifestations of the disease in the form of melanocytic naevus which can be hairy or non-hairy. These patients may or may not present with neurological symptoms but often show CNS abnormalities especially on MRI of the brain and the spine. A lot has been described about the disease since the first case described by Rokitansky in 1861, but every time a new CNS pathology is being added to the long list of currently documented pathologies. Herein we describe a case of a 5 yr old boy with seizures and hairy melanocytic naevus over the trunk and back who was diagnosed as a case of Neurocutaneous melanosis on subsequent evaluation by CT and MRI. We also describe the new association of CP angle cistern lipoma with neurocutaneous melanosis.

Keywords: CPA lipoma; Melanocytic naevus; Meningeal melanomatosis; Neurocutaneous melanosis; Neuroectodermal dysplasia.