Neurocutaneous melanosis

Case contributed by Dr Konrado Tenório

Presentation

Walking difficulty and skin findings.

Patient Data

Age: 9 years
Gender: Female
Modality: MRI

T1W: Hyperintense signal in the bilateral amygdaloid nuclei and pons, probably due to melanin/melanocyte deposits.

FLAIR: Shows amygdalae are hyperintense to the surrounding brain.

Gradient Echo: No susceptibility artifact within the amygdala, with subtle hyposignal susceptibility of the pons are shown.

T1 C+: There is no enhancement after contrast.

Modality: Photo

Picture demonstrate 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 hemorrhage and myelopathy.

The classic MRI finding include hyperintense areas in the temporal lobes on T1-weighted images. Diffuse leptomeningeal enhancement of the brain and spine, and mass of malignant melanoma can also be demonstrated.

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

Leptomeningeal carcinomatosis, meningitis, subarachnoid hemorrhage, non-melanocytic hemorrhagic tumors and other melanin-containing lesions are potential differential diagnoses that can be characterized by MRI images of the brain.

 

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Case Information

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

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