Schwartz-Jampel syndrome, abbreviated SJS and also known as chondrodystrophic myotonia, is a rare genetic disease. It is caused by a mutation in the perlecan gene, HSPG2, and produces osteochondrodysplasia, a disorder of bone and cartilage growth, associated with myotonia, which is delayed relaxation of muscle after contraction. Most people with the syndrome have a nearly normal life expectancy. The short source lead supports only these points, so the account here is intentionally brief.
Facts
Classification
ICD-10 ChapterVI: Diseases of the Nervous System 2 Communicable or Non-communicable Transmission Route Causative Agent
Causative Agent (category) Prevalence Band Sources
1. Schwartz-Jampel syndrome (Wikipedia)
Lead paragraph, first sentence
caused by a mutation in the perlecan gene (HSPG2)
Lead paragraph, first sentence [body-system]
osteochondrodysplasia associated with myotonia
View the Source2. Monarch Initiative: Schwartz-Jampel syndrome - Monarch Initiative MONDO:0009717
Monarch InitiativeCross reference: ICD10CMQuote, Cross reference: ICD10CM
ICD10CM:G71.13
View the Source 3. National Cancer Institute (NCI Thesaurus): Schwartz-Jampel syndrome - NCI Thesaurus concept C35008
National Cancer Institute (NCI Thesaurus)DefinitionQuote, Definition
A classification for rare genetic syndromes with an autosomal recessive pattern of inheritance.
View the Source 4. MedlinePlus Genetics (U.S. National Library of Medicine): Schwartz-Jampel syndrome - MedlinePlus Genetics
MedlinePlus Genetics (U.S. National Library of Medicine)Description and InheritanceQuote, Description and Inheritance
This condition is inherited in an autosomal recessive pattern , which means both copies of the gene in each cell have mutations.
View the Source 5. Orphadata (Orphanet): Orphadata epidemiology, ORPHAcode 800
Orphadata (Orphanet)Orphanet epidemiology, ORPHAcode 800Quote, Orphanet epidemiology, ORPHAcode 800
"PrevalenceClass": "\u003c1 / 1 000 000", "PrevalenceGeographic": "Worldwide", "PrevalenceQualification": "Class only", "PrevalenceType": "Point prevalence"
View the Source Reader Challenges (0)
No disputes yet. Spotted an error or a better source? Open the first one.
Sign in to dispute this or suggest a correction.