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Dandy-Walker Malformation Associated with Neurocutaneous Melanosis

Cho IY, Hwang SK, Kim SH

Neurocutaneous melanosis associated with Dandy-Walker malformation is a rare dysmorphogenesis that is associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the central nervous system. In...
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Neurocutaneous Melanosis: A Case Report

Seo YN, Jeong HW, In HS

Neurocutaneous melanosis is a rare disorder characterized by the presence of a large or multiple congenital melanocytic nevus with proliferation of melanocytes in the central nervous system. The prognosis of...
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A Case Report of Giant Pigmented Nevus Associated with Porencephaly: A Variant of Neurocutaneous Melanosis

Choi NC, Cho HS, Ha CK, Lim BH

  • KMID: 1957455
  • J Korean Neurol Assoc.
  • 1990 Dec;8(2):366-371.
Giant pigmented nevus is a variety of congenital melanocytic nevi, and may be associated with various abnormalities, such as skeletal malformation, lipoma, vascular nevi, melanosis in the meninges, meningocele, and...
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Clinical Study on 19 Cases of Neurocutaneous Syndrome in Children

Lee SH, Kim SJ, Cha HD, Kim HS, Kwon TC, Kang CM

  • KMID: 1682559
  • J Korean Pediatr Soc.
  • 1988 Mar;31(3):355-361.
No abstract available.
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Drowning Death due to an Epileptic Attack of a Patient with Sturge Weber syndrome: An Autopsy Report

Kang H, Park J, Kwon TJ, Choi YS, Koo HN, Kim SH, Kim TK, Kim M

  • KMID: 2305490
  • Korean J Leg Med.
  • 2010 Nov;34(2):136-139.
Sturge Weber syndrome (SWS) is a rare, congenital neurocutaneous syndrome. We rarely experienced neurocutaneous disorders in forensic practice but there are unexpected sudden deaths of patients with SWS due to...
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Encephalocraniocutaneous Lipomatosis without Neurologic Anomalies

Kim DH, Park SB, Lee Y, Im M, Seo YJ, Choi SH, Lee JH

No abstract available.
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Congenital Melanocytic Nevus with Asymptomatic Neurocutaneous Melanosis

Hong SK, Hwang S, Kang MS, Lee YS, Seo JK, Kim H, Lee D, Sung H

  • KMID: 1427469
  • Korean J Dermatol.
  • 2013 May;51(5):339-342.
Congenital melanocytic nevi (CMN) are benign pigmented lesions that are defined as a tissue malformation of the neuroectoderm. Giant melanocytic nevi with multifocal involvement show significantly greater risk of developing...
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A Case of Neurocutaneous Giant Melanosis Associated with Dandy-Walker Syndrome

Hong SY, Ban JE, Rhu JH, Choi SS, Hwang KG

  • KMID: 2137177
  • J Korean Child Neurol Soc.
  • 2001 May;9(1):171-177.
Neurocutaneous melanosis is a rare congenital phacomatosis, characterized by the presence of multiple pigmented skin nevi at birth. Meningeal melanosis tending to become malignant and seizure. Dandy-Walker syndrome is a...
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Neurocutaneous Melanosis Presenting as Chronic Partial Epilepsy

Ye BS, Cho YJ, Jang SH, Lee BI, Heo K, Jung HH, Chang JW, Kim SH

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...
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Multiple Congenital Melanocytic Nevi Associated with Developmental Delay

Kwon SH, Na SJ, Yeom KB, Huh SY, Na JI

  • KMID: 2088246
  • Korean J Dermatol.
  • 2011 Aug;49(8):753-756.
Multiple congenital melanocytic nevi (MCMN), defined as the distribution of more than three small- or medium- sized congenital melanocytic nevi (CMN) on the body without a giant CMN, is a...
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Giant Congenital Melanocytic Nevus with Multiple Satellite Lesions in a Korean Woman with Mental Retardation

Son HO, Go JW, Lee SY, Kim SH, Cho HK

  • KMID: 2247235
  • Korean J Dermatol.
  • 2013 Mar;51(3):185-188.
Giant congenital melanocytic nevi (GCMN) are defined as melanocytic nevi that are greater than 20 cm in largest dimension at birth. GCMN is a relatively rare condition, affecting approximately 1...
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Rapidly progressing neurocutaneous melanosis NCM in a 49-year-old woman

Kim HJ, Kim MJ, Kim YJ, Han JH, Huh CH, Lee HS, Lee JS

  • KMID: 2258138
  • Korean J Med.
  • 2009 Aug;77(Suppl 1):S183-S187.
Symptomatic neurocutaneous melanosis (NCM) rarely presents in adulthood. NCM is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi (CMN) combined with benign or malignant melanocytic proliferation...
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A Case of Sjogren-Larsson Syndrome

Lee JH, Lee J, Choi GS, Koo SW, Kim YK

  • KMID: 2230539
  • Korean J Dermatol.
  • 1998 Dec;36(6):1078-1082.
Sjogren-Larsson syndrome is a rare autosomal recessive neurocutaneous disorder due to a deficiency of the fatty aldehyde dehydrogenase. It consists of ichthyosis, spastic di- or quardriplegia and mental retardation. We...
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A Case of Symptomatic Neurocutaneous Melanosis Associated with the Dandy Walker Complex and Excellent Response of Seizures to Topiramate

Cho KH, Sun YH, Ryoo E, Tchah H

  • KMID: 2329416
  • J Korean Child Neurol Soc.
  • 2008 Nov;16(2):235-240.
Neurocutaneous melanosis is a rare non-familial congenital neurocutaneous syndrome characterized by the presence of large or multiple congenital melanocytic nevi in association with benign or malignant proliferation of melanocytes in...
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Neurocutaneous Melanosis in Association with Dandy-Walker Complex with Extensive Intracerebral and Spinal Cord Involvement

Sung KS, Song YJ

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...
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A Case of Nevus Sebaceus Syndrome Considered as a Neurocutaneous Syndrome

Kim JG, Lee CH, Kim HJ

  • KMID: 1678222
  • Korean J Dermatol.
  • 1981 Apr;19(2):221-226.
Nevus sebaceus is an ozganoid nevus of sebaceous differentiation first described by Jydassohn in 1895 and commonly develops on scalp or face as a single lesion. But it may be...
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A Case of Encephalocraniocutaneous Lipomatosis (ECCL)

Choi SH, Kim JY, Kim YD

  • KMID: 2337209
  • J Korean Ophthalmol Soc.
  • 2005 May;46(5):904-909.
PURPOSE: Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndromes characterized by unilateral scalp and facial lesions and multiple anomalies involving the eye and central nervous system. To our knowledge, this...
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A Case of Tuberous Sclerosis Associated with Multiple Tumorous Condition

Kim MJ, Chang MY, Lee KS, An RZ

  • KMID: 1544437
  • J Korean Child Neurol Soc.
  • 2000 May;8(1):126-126.
Tuberous Sclerosis is a neurocutaneous syndrome, which is characterized by seizure, mental retardation, angiofibroma and various tumorous conditions. We report a case of 15 year old girl with Tuberous Sclerosis...
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Malignant degeneration in neurofibromatosis: 3 case report

Lee JM, Kang EY, Lee NJ, Chung KB, Suh WH

Von Recklinghausen's neurofibromatosis is a kind of neurocutaneous syndrome characterized by skin, nervoussystem, neuromusculoskeletal manifestations. Most central and peripheral nervous neoplastic lesions in neurofibromatosis are benign but have a significant...
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Aesthetic Consideration of Management in Encephalocutaneous Lipomatosis: How we do it

Kim JM, Kim SJ, Song SH, Kang NH

  • KMID: 1917448
  • Arch Aesthetic Plast Surg.
  • 2012 Oct;18(3):147-150.
Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital neurocutaneous syndrome that was characterized by unilateral, smooth, hairless fatty tissue nevi of the scalp, termed nevus psiloliparus, facial lesions, multiple anomalies involving...
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