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myotonia congenita; Thomsen disease; Becker disease; myotonia levior

Pathology: - skeletal muscle stiffness (delayed relaxation), due to membrane hyperexcitability Genetics: - autosomal dominant (Thomsen disease) - autosomal recessive (Becker disease) - associated with defects in CLCN1 Clinical manifestations: - muscle rigidity Notes: - a variant form of Thomsen disease is myotonia levior that is characterized by milder symptoms, later onset & absence of muscle hypotrophy & hypertrophy

General

myotonic disorder genetic disease of muscle (inherited myopathy)

Database Correlations

OMIM correlations

References

  1. OMIM :accession 160800
  2. OMIM :accession 255700