Neurocutaneous melanosis

Case contributed by Konrado Tenório


Walking difficulty and skin findings

Patient Data

Age: 9 years
Gender: Female
  • Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

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.


Picture demonstrate a large pigmented nevus in left lumbar region

Case Discussion

The neurocutaneous melanosis clinical presentation is multisystemic and may include skin and neurological findings, such as hydrocephalus, neuropsychological development delay, psychiatric disorders, cranial nerve palsies, intracranial hemorrhage 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.

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