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Benign Samaritan congenital myopathy

MedGen UID:
1666762
Concept ID:
C4749502
Disease or Syndrome
Synonym: benign Samaritan congenital myopathy
SNOMED CT: Benign Samaritan congenital myopathy (770787005)
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:0017936
Orphanet: ORPHA324581

Definition

A rare genetic skeletal muscle disease with characteristics of severe neonatal hypotonia with respiratory insufficiency, delay in motor milestones, and dysmorphic features including bitemporal narrowing, epicanthal folds and hypertelorism. Affected individuals show gradual improvement in hypotonia and muscle weakness within the first two years of life resulting in minimal clinical manifestations in adulthood. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBenign Samaritan congenital myopathy

Recent clinical studies

Etiology

Böhm J, Leshinsky-Silver E, Vassilopoulos S, Le Gras S, Lerman-Sagie T, Ginzberg M, Jost B, Lev D, Laporte J
Acta Neuropathol 2012 Oct;124(4):575-81. Epub 2012 Jul 3 doi: 10.1007/s00401-012-1007-3. PMID: 22752422

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