Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

Sir, We read with great interest the case report by Ahuja et al of a child, 6 weeks old, with multiple giant congenital melanocytic nevi and central nervous system melanosis. Neurocutaneous melanosis has been reported to manifest itself most commonly within the first two years of life and children with this entity have been born as still births or have been reported at as early as one month of ...

Tuberous sclerosis complex (TSC) is a rare, auto-somal dominant, hereditary neurocutaneous syndrome, in which hamartomas develop in various organs. Cutaneous features and computed tomography (CT) findings are of great value in the diagnosis of TSC. The present study reported an unusual case of multiple organ abnormalities in a patient with TSC. Radiological findings indicated the presence of ce...

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder that causes congenital glaucoma. Previous experiences have shown that drainage procedures are often required to control associated glaucoma. The conventional surgical approach in trabeculectomy carries a significant risk of intraoperative expulsive hemorrhage. Here, we describe a modified approach of the conventional trabe...

Unidad Académica de Medicina de la Universidad Autónoma de Nayarit, México; Departamento de Neuropatología. Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México; Servicio de Neurocirugía, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México; Servicio de Neuropsiquiatría, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Méxi...

Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ven...

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi with either benign or malignant pigmented cell tumors of the leptomeninges. We report a case of NCM in a patient with previously robust health. No neurological deficit was noted in the patient. At the age of 21 years old, blurred vision initially appeared. ...