For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
39
views
11
references
 Top references
cited by
2
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      449
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: not found

      Neurocutaneous Melanosis Presenting as Chronic Partial Epilepsy

      case-report

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          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.

          Related collections

          Most cited references11

          • Record: found
          • Abstract: found
          • Article: not found

          Neurocutaneous melanosis: definition and review of the literature.

          Julie N. Kadonaga, Ilona Frieden (1991)
          Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The syndrome is thought to represent an error in the morphogenesis of the embryonal neuroectoderm. We review 39 reported cases of neurocutaneous melanosis and propose revised criteria for diagnosis. Most patients with neurocutaneous melanosis presented in the first 2 years of life with neurologic manifestations of increased intracranial pressure, mass lesions, or spinal cord compression. Leptomeningeal melanoma was present in 62% of the cases, but even in the absence of melanoma, symptomatic neurocutaneous melanosis had an extremely poor prognosis. Useful diagnostic procedures include cerebrospinal fluid cytology and magnetic resonance imaging with gadolinium contrast. Patients may be aided by palliative measures such as shunt placement to reduce intracranial pressure. Dermatologists in their follow-up of patients with large or multiple congenital melanocytic nevi should be aware of this condition, to aid in prompt diagnosis and because the treatment of cutaneous lesions may be altered in the presence of symptomatic neurocutaneous melanosis.
          Article 0 comments Cited 60 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Neurocutaneous melanosis: clinical features of large congenital melanocytic nevi in patients with manifest central nervous system melanosis.

            Q Wasti, Raj Rao, Katie Provost-Javier … (1996)
            Patients with a large congenital melanocytic nevus (LCMN) may have associated leptomeningeal melanocytosis with or without central nervous system (CNS) melanomas. These patients are considered to have neurocutaneous melanosis, a disorder that, when symptomatic or otherwise manifest neurologically, carries a poor prognosis even in the absence of malignancy. Our purpose was to identify typical clinical features in patients who have manifest CNS melanosis in association with LCMN. The records of 117 patients with LCMN in the New York University Registry of LCMN and the reports of 172 cases of LCMN in the world literature were included for features that might signal a high risk for the development of manifest CNS involvement. Of the 289 patients with LCMN, 33 had manifest CNS melanosis. In all 33 in whom symptomatic neurocutaneous melanosis was diagnosed, the LCMNs were present in a posterior axial location on the head, neck, back, and/or buttocks. "Satellite" nevi were known to be present in 31 of the 33 patients. Patients with LCMN in a posterior axial location, especially when associated with "satellite" melanocytic nevi, are at greater risk for the development of manifest neurocutaneous melanosis than patients with LCMN limited to the extremities or those who are lacking satellite nevi.
            Article 0 comments Cited 31 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Giant congenital melanocytic nevi: the significance of neurocutaneous melanosis in neurologically asymptomatic children.

              A. Barkovich, I Frieden, Frances M. Williams … (2001)
              Patients with a giant congenital melanocytic nevus can develop melanotic tumors characterized by central nervous system involvement, termed leptomeningeal melanocytosis or neurocutaneous melanosis. Although symptomatic neurocutaneous melanosis is rare, we previously reported distinct magnetic resonance (MR) findings of T1 shortening, strongly suggestive of neurocutaneous melanosis, in 30 percent (6 of 20) of children with giant congenital melanocytic nevi who presented initially without neurological symptoms. The purpose of this study was to determine the incidence of neurocutaneous melanosis in high-risk patients and its long-term clinical significance. Magnetic resonance imaging was recommended for all 46 patients with "at-risk" giant congenital melanocytic nevi involving the skin overlying the dorsal spine or scalp. The clinical histories and follow-up of these patients were evaluated by retrospective chart review. Forty-two underwent MR imaging of the brain and 11 underwent additional MR scanning of the spinal cord. Abnormalities were identified in 14 of 43 MR studies, and 23 percent (n = 10) had T1 shortening indicative of melanotic rests within the brain or meninges. None had associated masses or leptomeningeal thickening. The most common areas of involvement in these 10 included the amygdala (n = 8), cerebellum (n = 5), and pons (n = 3). In the group of 11 patients with spinal MR scans, a tethered spinal cord was demonstrated in one. Additional abnormalities were detected by MR scanning, including a middle cranial fossa arachnoid cyst, a Chiari type I malformation, and a crescentic enhancement that subsequently resolved. Clinical follow-up averaging 5 years (range, 2 to 8 years) revealed that only one of the 46 patients evaluated developed neurological symptoms, manifested as developmental delay, hypotonia, and questionable seizures but no other signs of neurocutaneous melanosis. No patient has developed a cutaneous or central nervous system melanoma. Magnetic resonance findings of neurocutaneous melanosis are relatively common, even in asymptomatic children with giant congenital melanocytic nevi. Although these findings suggest an increased lifetime risk of central nervous system melanoma, they do not signify the eventual development of symptomatic neurocutaneous melanosis during childhood.
              Article 0 comments Cited 25 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Journal
                Journal ID (nlm-ta): J Clin Neurol
                Journal ID (publisher-id): JCN
                Title: Journal of Clinical Neurology (Seoul, Korea)
                Publisher: Korean Neurological Association
                ISSN (Print): 1738-6586
                ISSN (Electronic): 2005-5013
                Publication date (Print): September 2008
                Publication date (Electronic): 30 September 2008
                Volume: 4
                Issue: 3
                Pages: 134-137
                Affiliations
                [a ]Department of Neurology, Yonsei University College of Medicine, Seoul, Korea.
                [b ]Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea.
                [c ]Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
                Author notes
                Correspondence: Kyoung Heo, MD, PhD. Department of Neurology, Yonsei University College of Medicine, 134 Sinchon-dong, Seodaemun-gu, Seoul 120-752, Korea. Tel +82-2-2228-1607, Fax +82-2-393-0705, kheo@ 123456yuhs.ac
                Article
                DOI: 10.3988/jcn.2008.4.3.134
                PMC ID: 2686876
                PubMed ID: 19513317
                SO-VID: c262122a-1bab-4826-9ece-05b7e3f22659
                Copyright © Copyright © 2008 Korean Neurological Association
                History
                Date received : 09 April 2008
                Date revision received : 13 June 2008
                Date accepted : 23 June 2008
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Neurology
                Keywords: epilepsy surgery,partial epilepsy,neurocutaneous melanosis
                Data availability:
                ScienceOpen disciplines: Neurology
                Keywords: epilepsy surgery, partial epilepsy, neurocutaneous melanosis

                Comments

                Comment on this article

                scite_

                Similar content449

                See all similar

                Cited by2

                See all cited by

                Most referenced authors85

                See all reference authors