Amygdalar neuromelanosis intractable epilepsy without leptomeningeal involvement

Case report

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Neurocutaneous melanosis (NCM) is a rare, congenital neuroectodermal dysplasia generally resulting in early death from neurological decline due to leptomeningeal involvement. Nonmeningeal CNS epileptogenic lesions presenting in later childhood in the absence of leptomeningeal disease are rare. This report summarizes a rare presentation and curative epilepsy surgery.

The authors discuss the case of a 14-year-old girl with NCM who originally presented with intractable partial-onset seizures. The MRI, PET, and SPECT studies subsequently revealed a focal epileptogenic source in the right temporal lobe. Results of video-electroencephalography monitoring concurred with the imaging findings, and a right temporal lobectomy was performed including the medial structures. Following surgery, histopathological features of the lesion included multiple scattered mononuclear cells with brown pigmentation in the amygdala specimen. The patient remains seizure free 2 years postresection, and no longer needs medication for seizure management.

This patient presented with an atypical CNS manifestation of NCM that is curable by epilepsy surgery. Her intractable epilepsy developed secondary to amygdalar neuromelanosis, which had no associated leptomeningeal melanosis, an uncommon occurrence. As evidenced by the lack of seizure activity following resection, the patient's quality of life greatly improved after neurological surgery.

Abbreviations used in this paper:CMN = congenital melanocytic nevi; NCM = neurocutaneous melanosis.

Article Information

Current affiliation for Dr. Wait: Levine Children's Hospital/Carolinas Medical Center, Carolina Neurosurgery and Spine Associates, Charlotte, North Carolina.

Address correspondence to: Frederick A. Boop, M.D., Division of Pediatric Neurosurgery, LeBonheur Children's Hospital, 6325 Humphreys Boulevard, Memphis, Tennessee 38120. email:

Please include this information when citing this paper: published online May 3, 2013; DOI: 10.3171/2013.3.PEDS12502.

© AANS, except where prohibited by US copyright law.



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    Photograph showing cutaneous findings in neuromelanosis. This patient has multiple, large, confluent melanocytic nevi covering large portions of her trunk.

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    Coronal FLAIR MRI study demonstrating right amygdalar hyperintensity (red arrow).

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    Intraoperative photograph showing a gross anatomical view of the surgical field. The temporal lobe (asterisk) and leptomeninges are normal in appearance. The typical neuromelanosis findings of leptomeningeal involvement are absent. Temporal lobectomy was demarcated posteriorly by the vein of Labbé (arrow).

  • View in gallery

    Photograph showing a gross anatomical amygdalar specimen. Prominent brown pigmentation of the amygdala is present in this en bloc specimen.

  • View in gallery

    Photomicrograph of an H & E–stained amygdalar section demonstrating diffuse infiltration of melanin pigment interspersed among the neurons of the nucleus. Original magnification ×40.



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