ICD-11 classes
08 Diseases of the nervous system
Motor neuron diseases or related disorders
8B61 — Spinal muscular atrophy

ICD-11 8B61 — Spinal muscular atrophy

Spinal muscular atrophy (SMA) is a progressive disorder with loss of anterior horn cells leading to muscle weakness and wasting. The weakness is typically symmetrical. Typically, upper motor neuron signs are absent and there is no sensory deficit. Feeding and swallowing can be affected, and involvement of respiratory muscles may occur. SMA is an autosomal recessive disorder linked to chromosome 5q13 and the disorder is caused by deletion or mutation of SMN 1 (spinal motor neuron 1) gene. The four types of SMA I, II, III and IV are categorised based on the age of onset of the disease and the ability to achieve motor milestones.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 8B61 contains 7 clarifying diagnoses:

1. 8B61.0 — Infantile spinal muscular atrophy, Type I
2. 8B61.1 — Late infantile spinal muscular atrophy, Type II
3. 8B61.2 — Juvenile form spinal muscular dystrophy, Type III
4. 8B61.3 — Adult onset spinal muscular atrophy, Type IV
5. 8B61.4 — Localised spinal muscular atrophy
6. 8B61.Y — Other specified spinal muscular atrophy
7. 8B61.Z — Spinal muscular atrophy, unspecified