Dominant limb-girdle muscular dystrophy

Description

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 vary 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.

Index terms

Dominant limb-girdle muscular dystrophy
LGMD1 - [limb-girdle muscular dystrophy] 1
Limb-girdle muscular dystrophy 1A, myotilin myopathy

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LGMD 1A - [Limb-girdle muscular dystrophy 1A, myotilin myopathy]
Limb-girdle muscular dystrophy 1B, lamin myopathy
LGMD 1B - [Limb-girdle muscular dystrophy 1B, lamin myopathy]
Limb-girdle muscular dystrophy 1C, caveolin myopathy
LGMD 1C - [Limb-girdle muscular dystrophy 1C, caveolin myopathy]
Limb-girdle muscular dystrophy 1D, gene mapped to 6q23
Limb-girdle muscular dystrophy 1E, gene mapped to 7q
Limb-girdle muscular dystrophy 1F, gene mapped to 7q32.1-32.2
Limb-girdle muscular dystrophy 1G, gene mapped to 4q21
Limb-girdle muscular dystrophy 1H, gene mapped to 3p25.1p23
LGMD1H - [Limb-girdle muscular dystrophy] 1H

Excludes

http://id.who.int/icd/entity/1159616108 – Secondary myopathies
http://id.who.int/icd/entity/1425591047 – Myasthenia gravis or certain specified neuromuscular junction disorders

Exclusions from above levels

http://id.who.int/icd/entity/155258022 – Metabolic disorders
LD26.41 – Arthrogryposis multiplex congenita
05 – Endocrine, nutritional or metabolic diseases

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18 – Complications of pregnancy, childbirth and the puerperium
19 – Certain conditions originating in the perinatal period
22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

8C70.41 – Recessive limb-girdle muscular dystrophy
8C70.4Y – Other specified limb-girdle muscular dystrophy
8C70.4Z – Limb-girdle muscular dystrophy, unspecified

Foundation entity

Dominant limb-girdle muscular dystrophy – http://id.who.int/icd/entity/537908479

Allowed postcoordination axes

Part of: 8C70.4: Limb-girdle muscular dystrophy › 8C70: Muscular dystrophy › 08: Diseases of the nervous system