Neurocutaneous melanosis

Case contributed by Dr Arya Mehta

Presentation

Multiple skin lesions. No CNS complaints at present. No positive familial history.

Patient Data

Age: 20 days
Gender: Female
Modality: MRI

Well defined oval area of altered signal intensity noted in right half of pons which appears hyperintense on T1WI, hypointense on T2WI and FLAIR. No evidence of blooming on gradient images. No restricted diffusion on DWI.

Case Discussion

Neurocutaneous melanosis is a rare nonfamilial syndrome characterized with giant and/or multiple congenial melanocytic nevi of skin and melanocytic tumours in leptomemimges of central nervous system.

It is thought to be neurocristopathy caused by neural crest aberration during early embryonic development.

These lesions may occur in pons,amygdala,cerebrum,cerebellum and spinal cord.

They are asymptomatic generally. Seizures and raised ICP can occur with leptomeningeal melanoma which occurs in almost half of patients.

They generally have poor outcomes with few treatment options.

This patient had multiple congenital pigmented patches over abdomen, back and periorbital regions. Skin biopsy revealed them to be melanocytic nevi.

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Case Information

rID: 27008
Case created: 17th Jan 2014
Last edited: 21st Dec 2016
Inclusion in quiz mode: Included

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