J Clin Neurol.  2008 Sep;4(3):134-137. 10.3988/jcn.2008.4.3.134.

Neurocutaneous Melanosis Presenting as Chronic Partial Epilepsy

• Ye BS
• Cho YJ
• Jang SH
• Lee BI
• Heo K
• Jung HH
• Chang JW
• Kim SH

• KMID: 2287678
• DOI: http://doi.org/10.3988/jcn.2008.4.3.134

Abstract

BACKGROUND: Neurocutaneous melanosis (NCM) is a rare neurocutaneous syndrome characterized by the presence of multiple congenital melanocytic nevi (CMN) and the proliferation of melanocytes in the central nervous system, usually involving the leptomeninges. Chronic partial epilepsy as a sole manifestation is rare in NCM.
CASE REPORT
A 32-year-old man suffering from chronic partial epilepsy presented with multiple CMN on his trunk and scalp. Brain MRI demonstrated a focal lesion in the right amygdala that was consistent with interictal epileptiform discharges in the right temporal region on electroencephalography (EEG). An anterior temporal lobectomy was performed, and the pathology investigation revealed numerous melanophages in the amygdala. The patient was seizure-free after surgery.
CONCLUSIONS
We report a patient with NCM presenting as chronic partial epilepsy who was successfully treated by anterior temporal lobectomy.

Keyword

Neurocutaneous melanosis; Partial epilepsy; Epilepsy surgery

MeSH Terms

Adult
Amygdala
Anterior Temporal Lobectomy
Brain
Central Nervous System
Electroencephalography
Epilepsies, Partial
Humans
Melanocytes
Melanosis
Neurocutaneous Syndromes
Nevus, Pigmented
Scalp
Stress, Psychological
Melanosis
Neurocutaneous Syndromes

Figure

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  Fig. 1 Brain MRI. T2-weighted images (A, B), and T1-weighted gadolinium-enhancement images (C, D) show a focal lesion (arrow head) with hyperintensity and hypointensity without enhancement in the right amygdala, respectively.

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  Fig. 2 Photograph showing the gross operative finding (A) of a grayish-to-black friable soft tissue without definite mass formation in the right amygdala. The specimen from the lesion (B) contained numerous melanophages in the cortex, which is consistent with neurocutaneous melanosis. The specimen from the scalp nevus (C, D) was compatible with intradermal nevus. Hematoxylin and eosin staining. Scale bars=50 µm.

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