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Published on: January 29, 2018
Neurocutaneous melanosis presenting as chronic partial epilepsy
Byoung Seok Ye1, Yang-Je Cho, Sang Hyun Jang
1Department of Neurology, Yonsei University College of Medicine, Seoul, Korea.
Journal of Clinical Neurology (Seoul, Korea)
|June 11, 2009
Summary
Neurocutaneous melanosis (NCM), a rare syndrome, can manifest as chronic partial epilepsy. Surgical removal of a melanocytic lesion in the amygdala successfully resolved seizures in one patient.
Area of Science:
- Neurology
- Dermatology
- Pathology
Background:
- Neurocutaneous melanosis (NCM) is a rare syndrome involving congenital melanocytic nevi (CMN) and central nervous system melanocyte proliferation.
- Chronic partial epilepsy is an uncommon presentation of NCM.
Purpose of the Study:
- To report a case of NCM presenting solely as chronic partial epilepsy.
- To highlight the successful surgical management of epilepsy in NCM.
Main Methods:
- A 32-year-old male with multiple CMN and chronic partial epilepsy underwent brain MRI and EEG.
- Anterior temporal lobectomy was performed, with pathological examination of the amygdala lesion.
- Post-operative seizure freedom was assessed.
Main Results:
- Brain MRI revealed a focal lesion in the right amygdala correlating with EEG findings.
- Pathology confirmed the presence of melanophages in the amygdala.
- The patient achieved complete seizure control after surgery.
Conclusions:
- NCM can present with chronic partial epilepsy as its primary manifestation.
- Anterior temporal lobectomy is an effective treatment for epilepsy in NCM patients with focal lesions.
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