Journal of Korean Neurosurgical Society

  • 제56권1호
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  • Pages.61-65
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  • 2014
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  • 2005-3711(pISSN)
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  • 1598-7876(eISSN)
대한신경외과학회 (The Korean Neurosurgical Society)
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Neurocutaneous Melanosis in Association with Dandy-Walker Complex with Extensive Intracerebral and Spinal Cord Involvement

  • Sung, Kyoung-Su (Department of Neurosurgery, College of Medicine, Dong-A University) ;
  • Song, Young-Jin (Department of Neurosurgery, College of Medicine, Dong-A University)
  • 투고 : 2013.10.29
  • 심사 : 2014.07.15
  • 발행 : 2014.07.28
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초록

Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.

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참고문헌

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피인용 문헌

  1. Dandy–Walker Malformation and Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episode Syndrome: Is There a Causal or Coincidental Association? vol.129, pp.5, 2016, https://doi.org/10.4103/0366-6999.176984
  2. Neurocutaneous melanosis presenting with hydrocephalus and malignant transformation: case-based update vol.34, pp.8, 2014, https://doi.org/10.1007/s00381-018-3851-5
  3. Neurocutaneous melanosis is not always a benign disease vol.86, pp.3, 2014, https://doi.org/10.4103/ijdvl.ijdvl_456_18
  4. Clinical Follow-Up of Patients with Neurocutaneous Melanosis in a Tertiary Center; Proposed Modification in Diagnostic Criteria vol.146, pp.None, 2014, https://doi.org/10.1016/j.wneu.2020.11.091