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Autosomal recessive myogenic arthrogryposis multiplex congenita

MedGen UID:
1647450
Concept ID:
C4707155
Disease or Syndrome
Synonyms: Autosomal recessive myogenic AMC; autosomal recessive myogenic AMC; autosomal recessive myogenic arthrogryposis multiplex congenita; SYNE1 (spectrin repeat containing nuclear envelope protein 1) related arthrogryposis multiplex congenita; SYNE1-related AMC; SYNE1-related arthrogryposis multiplex congenita
SNOMED CT: Autosomal recessive myogenic arthrogryposis multiplex congenita (764812008); SYNE1-related arthrogryposis multiplex congenita (764812008); SYNE1 (spectrin repeat containing nuclear envelope protein 1) related arthrogryposis multiplex congenita (764812008)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0017892
Orphanet: ORPHA319332

Definition

A rare inherited neuromuscular disease with prenatal presentation (usually in the second trimester) of reduced fetal movements and abnormal positioning. This results in joint abnormalities that may involve both lower and upper extremities and is usually symmetric. Also associated with severe hypotonia at birth, bilateral club foot, motor development delay, mild facial weakness without ophthalmoplegia, absent deep tendon reflexes, normal motor and sensory nerve conduction velocities, no cerebellar or pyramidal involvement and progressive disease course with loss of ambulation after the first decade of life. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Autosomal recessive myogenic arthrogryposis multiplex congenita

Recent clinical studies

Etiology

Attali R, Warwar N, Israel A, Gurt I, McNally E, Puckelwartz M, Glick B, Nevo Y, Ben-Neriah Z, Melki J
Hum Mol Genet 2009 Sep 15;18(18):3462-9. Epub 2009 Jun 19 doi: 10.1093/hmg/ddp290. PMID: 19542096
Ho NC
Neuropediatrics 2000 Oct;31(5):252-6. doi: 10.1055/s-2000-9238. PMID: 11204282

Diagnosis

Kamal NM, Alzeky AM, Omair MR, Attar RA, Alotaibi AM, Safar A, Alosaimi NS, Abosabie SAS
Ital J Pediatr 2022 Jun 23;48(1):107. doi: 10.1186/s13052-022-01301-x. PMID: 35739559Free PMC Article

Prognosis

Kamal NM, Alzeky AM, Omair MR, Attar RA, Alotaibi AM, Safar A, Alosaimi NS, Abosabie SAS
Ital J Pediatr 2022 Jun 23;48(1):107. doi: 10.1186/s13052-022-01301-x. PMID: 35739559Free PMC Article
Shashi V, Geist J, Lee Y, Yoo Y, Shin U, Schoch K, Sullivan J, Stong N, Smith E, Jasien J, Kranz P; Undiagnosed Diseases Network, Lee Y, Shin YB, Wright NT, Choi M, Kontrogianni-Konstantopoulos A
Hum Mutat 2019 Aug;40(8):1115-1126. Epub 2019 May 5 doi: 10.1002/humu.23760. PMID: 31264822Free PMC Article
Ho NC
Neuropediatrics 2000 Oct;31(5):252-6. doi: 10.1055/s-2000-9238. PMID: 11204282

Clinical prediction guides

Kamal NM, Alzeky AM, Omair MR, Attar RA, Alotaibi AM, Safar A, Alosaimi NS, Abosabie SAS
Ital J Pediatr 2022 Jun 23;48(1):107. doi: 10.1186/s13052-022-01301-x. PMID: 35739559Free PMC Article
Shashi V, Geist J, Lee Y, Yoo Y, Shin U, Schoch K, Sullivan J, Stong N, Smith E, Jasien J, Kranz P; Undiagnosed Diseases Network, Lee Y, Shin YB, Wright NT, Choi M, Kontrogianni-Konstantopoulos A
Hum Mutat 2019 Aug;40(8):1115-1126. Epub 2019 May 5 doi: 10.1002/humu.23760. PMID: 31264822Free PMC Article
Attali R, Warwar N, Israel A, Gurt I, McNally E, Puckelwartz M, Glick B, Nevo Y, Ben-Neriah Z, Melki J
Hum Mol Genet 2009 Sep 15;18(18):3462-9. Epub 2009 Jun 19 doi: 10.1093/hmg/ddp290. PMID: 19542096
Ho NC
Neuropediatrics 2000 Oct;31(5):252-6. doi: 10.1055/s-2000-9238. PMID: 11204282

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