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Antenatal multiminicore disease with arthrogryposis multiplex congenita

MedGen UID:
334470
Concept ID:
C1843691
Disease or Syndrome
Synonyms: Minicore myopathy, antenatal onset, with arthrogryposis; Multicore myopathy, antenatal onset, with arthrogryposis; Multiminicore myopathy, antenatal onset, with arthrogryposis
 
Monarch Initiative: MONDO:0015794
Orphanet: ORPHA178148

Definition

Multiminicore disease (MmD) is a clinically heterogeneous condition in which several subgroups can be distinguished (see 255320 and 602771). General features include neonatal hypotonia, delayed motor development, and generalized muscle weakness and amyotrophy, which may progress slowly or remain stable. Muscle biopsy shows multiple, poorly circumscribed, short areas of sarcomere disorganization and mitochondria depletion (areas termed 'minicores') in most muscle fibers. Typically, no dystrophic signs, such as muscle fiber necrosis or regeneration or significant endomysial fibrosis, are present in multiminicore disease (Ferreiro and Fardeau, 2002). [from OMIM]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAntenatal multiminicore disease with arthrogryposis multiplex congenita
Follow this link to review classifications for Antenatal multiminicore disease with arthrogryposis multiplex congenita in Orphanet.

Professional guidelines

Curated

Lillis S, Abbs S, Ferreiro A, Muntoni F, Jungbluth H
Eur J Hum Genet 2012 Feb;20(2) Epub 2011 Oct 19 doi: 10.1038/ejhg.2011.180. PMID: 22009146Free PMC Article

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