A Case of Sjogren-Larsson Syndrome / 대한피부과학회지
Korean Journal of Dermatology
;
: 1078-1082, 1998.
Article
in Korean
| 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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Rehabilitation
/
Cerebral Palsy
/
Sjogren-Larsson Syndrome
/
Rhizotomy
/
Neurocutaneous Syndromes
/
Aldehyde Dehydrogenase
/
Ichthyosis
/
Macular Degeneration
/
Intellectual Disability
/
Muscle Spasticity
Limits:
Child
/
Humans
/
Male
Language:
Korean
Journal:
Korean Journal of Dermatology
Year:
1998
Type:
Article
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