Imaging findings in encephalocraniocutaneous lipomatosis

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منابع مشابه

Characteristic imaging findings in encephalocraniocutaneous lipomatosis.

Our patient was worked up for mild developmental delay associated with focal scalp alopecia at age 6, and was diagnosed with encephalocraniocutaneous lipomatosis (also known as Haberland or Fishman syndrome), a rare neurocutaneous syndrome characterized by skin lesions (most commonly focal alopecia with or without underlying lipoma), ocular lesions, and CNS anomalies. CNS anomalies are usually ...

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Encephalocraniocutaneous Lipomatosis

Encephalocraniocutaneous lipomatosis or Haberland syndrome is a rare, congenital neurocutaneous syndrome. It is characterized by unilateral lipomatous hamartomata of the scalp, eyelid, and outer globe of the eye and ipsilateral neurologic malformations. We describe the first case from Lebanon, an infant with classical encephalocraniocutaneous lipomatosis characterized by nevus psiloliparus, uni...

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Encephalocraniocutaneous Lipomatosis: Haberland Syndrome

BACKGROUND Encephalocraniocutaneous lipomatosis (ECCL) was first announced as a new type of ectomesodermal dysgenesis in 1970 by Haberland and Perou. ECCL was first described in 1970, and approximately 60 cases have been reported since then. The classic triad of ECCL are skin, ocular, and central nervous system involvement, including conditions such as unilateral porencephalic cyst, ipsilateral...

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Partial Encephalocraniocutaneous Lipomatosis Syndrome

Encephalocranial lipomatosis is a rare disorder that characteristically involves ectomesodermal tissues such as skin, eye, and the central nervous system. Here, we report a 3-year-old girl presented with developmental delay, seizures, limbal dermoid, and weakness of right lower limb. Imaging revealed hemiatrophy, arachnoid cyst, and polymicrogyria. The constellation of clinical finding and imag...

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Encephalocraniocutaneous Lipomatosis without Neurologic Anomalies

Received September 22, 2011, Revised December 26, 2011, Accepted for publication January 18, 2012 Corresponding author: Jeung-Hoon Lee, M.D., Department of Dermatology, Chungnam National University Hospital, Munhwaro 282, Jung-gu, Daejeon 301-721, Korea. Tel: 82-42-280-7706, Fax: 82-42-280-8459, E-mail: [email protected] This is an Open Access article distributed under the terms of the Creative C...

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ژورنال

عنوان ژورنال: Neurology

سال: 2011

ISSN: 0028-3878,1526-632X

DOI: 10.1212/wnl.0b013e3182299fa9