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A Case of Sjogren-Larsson Syndrome / 대한피부과학회지
Korean Journal of Dermatology ; : 1078-1082, 1998.
Artículo en Coreano | WPRIM | ID: wpr-35777
ABSTRACT
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 report a case of a 9-year-old boy who was suspected to have Sjogren-Larsson syndrome. He had congenital ichthyosis, spastic diplegia and mental retardation. In addition, there was macular degeneration on the fundus. His neurological symptoms were improved after selective posterior rhizotomy and rehabilitation therapy. However, the dermatological problem did not subside with conservative treatment.
Asunto(s)

Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Rehabilitación / Parálisis Cerebral / Síndrome de Sjögren-Larsson / Rizotomía / Síndromes Neurocutáneos / Aldehído Deshidrogenasa / Ictiosis / Degeneración Macular / Discapacidad Intelectual / Espasticidad Muscular Límite: Niño / Humanos / Masculino Idioma: Coreano Revista: Korean Journal of Dermatology Año: 1998 Tipo del documento: Artículo

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Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Rehabilitación / Parálisis Cerebral / Síndrome de Sjögren-Larsson / Rizotomía / Síndromes Neurocutáneos / Aldehído Deshidrogenasa / Ictiosis / Degeneración Macular / Discapacidad Intelectual / Espasticidad Muscular Límite: Niño / Humanos / Masculino Idioma: Coreano Revista: Korean Journal of Dermatology Año: 1998 Tipo del documento: Artículo