Myasthenic Syndrome, Congenital, 8

Alternative Names

  • CMS8
  • Myasthenic Syndrome, Congenital, with Pre- and Postsynaptic Defects
  • CMSPPD
  • Myasthenic Syndrome, Congenital, Due to Agrin Deficiency

Associated Genes

Agrin
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WHO-ICD-10 version:2010

Diseases of the nervous system

Diseases of myoneural junction and muscle

OMIM Number

615120

Mode of Inheritance

Autosomal recessive

Gene Map Locus

1p36.33

Description

Congenital myasthenic syndromes are genetic disorders of the neuromuscular junction (NMJ) that are classified by the site of the transmission defect: presynaptic, synaptic, and postsynaptic. CMS8 is an autosomal recessive disorder characterized by prominent defects of both the pre- and postsynaptic regions. Affected individuals have onset of muscle weakness in early childhood; the severity of the weakness and muscles affected is variable. [From OMIM]

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
615120.1Saudi ArabiaFemaleNoYes Distal arthrogryposisNM_198576.4:c.4178C>THomozygousAutosomal, RecessiveMonies et al. 2017
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