Neurocutaneous melanosis

Case contributed by Dr Konrado Tenório

Presentation

Walking difficulty and skin findings.

Patient Data

Age: 9 years
Gender: Female
MRI

T1 hyperintense signal in the bilateral amygdaloid nuclei and pons, probably due to melanin/melanocyte deposits.These areas also demonstrate hyperintensity on FLAIR. No susceptibility artifact within the amygdala, with subtle hyposignal susceptibility of the pons are shown on SWI. There is no enhancement after contrast.

Photo

Picture demonstrate a large pigmented nevus in left lumbar region

Case Discussion

Neurocutaneous melanosis clinical presentation is multifactorial and can include skin and neurological findings, such as hydrocephalus, neuropsychological development delay, psychiatric disorders, cranial nerve palsies, intracranial haemorrhage and myelopathy.

Although classically the imaging features are dominated by diffuse leptomeningeal enhancement of the brain and spine due to leptomeningeal/subarachnoid spread of melanocytic cells, with associated hydrocephalus, in a small number of cases melanin-containing parenchymal lesions have been described 1,2

The lesions usually exhibit a high signal intensity on T1-weighted images and do not show enhancement after contrast. They typically occur in the amygdaloid nuclei, cerebellum or pons 1.

PlayAdd to Share

Case information

rID: 52251
Case created: 29th Mar 2017
Last edited: 14th Jun 2017
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.