title: 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.
authors.affiliation: Radiodiagnosis, Jawaharlal Nehru Medical College, India drazfarsiddiqui@gmail.com.
authors.forename: Mohammed Azfar
authors.initials: MA
authors.lastname: Siddiqui
authors.affiliation: Radiodiagnosis, Jawaharlal Nehru Medical College, India.
authors.forename: Shaista
authors.initials: S
authors.lastname: Siddiqui
authors.affiliation: Radiodiagnosis, Jawaharlal Nehru Medical College, India.
authors.forename: Nuzhat
authors.initials: N
authors.lastname: Zaman
authors.affiliation: Radiodiagnosis, Jawaharlal Nehru Medical College, India.
authors.forename: Ibne
authors.initials: I
authors.lastname: Ahmad
authors.affiliation: Radiodiagnosis, Jawaharlal Nehru Medical College, India.
authors.forename: Ekram
authors.initials: E
authors.lastname: Ullah
mesh_terms: D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D008067:Lipoma / Q000150:complications / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging; D008297:Male; D008548:Melanosis / Q000150:complications / Q000175:diagnosis*; D020752:Neurocutaneous Syndromes / Q000150:complications / Q000175:diagnosis*; D009464:Neuroma, Acoustic / Q000150:complications / Q000175:diagnosis*; D035583:Rare Diseases; D014057:Tomography, X-Ray Computed
keywords: CPA lipoma; Melanocytic naevus; Meningeal melanomatosis; Neurocutaneous melanosis; Neuroectodermal dysplasia