ICD-11 classes
08 Diseases of the nervous system
Diseases of neuromuscular junction or muscle
Primary disorders of muscles
8C70 — Muscular dystrophy
8C70.4 — Limb-girdle muscular dystrophy
8C70.40 — Dominant limb-girdle muscular dystrophy

ICD-11 8C70.40 — Dominant limb-girdle muscular dystrophy

The Limb Girdle Muscular Dystrophies (LGMD) are a group of genetic disorders characterised predominantly by progressive wasting and weakness of proximal limb girdle muscles, including pelvic, shoulder, upper arm and thigh muscles. The onset symptoms usually varies from early childhood to late adulthood, and the progression rate and distribution of weakness and wasting also varies considerably among individuals and genetic subtypes. There are currently 8 autosomal dominant LGMDs (LGMD1), linked to specific gene mutations. Dominant LGMDs are often allelic with other clinical disorders, including the myofibrillar myopathies or dilated cardiomyopathy.

The diagnosis includes nothing.

It excludes 2 items.

  • Secondary myopathies (8C80-8C8Z)
  • Myasthenia gravis or certain specified neuromuscular junction disorders (8C60-8C6Z)

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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