Structured Summary
Abstract
An autosomal recessive neurocutaneous disorder characterized by severe ichthyosis MENTAL RETARDATION; SPASTIC PARAPLEGIA; and congenital ICHTHYOSIS. It is caused by mutation of gene encoding microsomal fatty ALDEHYDE DEHYDROGENASE leading to defect in fatty alcohol metabolism.
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Classification
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Synonyms
9 entry terms
- Congenital Icthyosis Mental Retardation Spasticity Syndrome
- FALDH Deficiency
- Fatty Alcohol:NAD+ Oxidoreductase Deficiency
- Fatty Aldehyde Dehydrogenase Deficiency
- Fatty Aldehyde Dehydrogenase Deficiency Disease
- Ichthyosis Oligophrenia Syndrome
- Ichthyosis, Spastic Neurologic Disorder, and Oligophrenia
- Sjögren-Larsson Syndrome
- Sjogren Larsson Syndrome
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Aspects Covered
34 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
Indexing Annotation
do not confuse with SJOGREN'S SYNDROME; in titles & translations use diacritic: Sjögren-Larsson
MeSH Record
History Note
1991; use ICHTHYOSIS 1984-1990
MeSH Record
Previous Indexing
- Ichthyosis (1966-1990)
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Tree Numbers
AMA Style
References
- National Library of Medicine. Sjogren-Larsson Syndrome. Medical Subject Headings (MeSH). 2026. Unique ID D016111. http://id.nlm.nih.gov/mesh/2026/D016111
- Sjogren-Larsson Syndrome. In: Wikipedia. https://en.wikipedia.org/wiki/Sj%C3%B6gren%E2%80%93Larsson_syndrome
- Sjogren-Larsson Syndrome. In: Wikidata. https://www.wikidata.org/wiki/Q2291208