Amygdalar neuromelanosis intractable epilepsy without leptomeningeal involvement Case report

Douglas R. Taylor, Scott D. Wait, James Wheless, Frederick Boop

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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 partialonset 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.

Original languageEnglish (US)
Pages (from-to)21-24
Number of pages4
JournalJournal of Neurosurgery: Pediatrics
Volume12
Issue number1
DOIs
StatePublished - Jul 2013

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Seizures
Epilepsy
Melanosis
Pigmentation
Temporal Lobe
Rare Diseases
Amygdala
Single-Photon Emission-Computed Tomography
Electroencephalography
Quality of Life
Drug Resistant Epilepsy
Neurocutaneous melanosis

All Science Journal Classification (ASJC) codes

  • Clinical Neurology
  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Amygdalar neuromelanosis intractable epilepsy without leptomeningeal involvement Case report. / Taylor, Douglas R.; Wait, Scott D.; Wheless, James; Boop, Frederick.

In: Journal of Neurosurgery: Pediatrics, Vol. 12, No. 1, 07.2013, p. 21-24.

Research output: Contribution to journalArticle

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