Disorder - Spinal Muscular Atrophy

Organ Systems Involved
Musculoskeletal
Nervous/sensory

Alternative Names
Hereditary Canine Spinal Muscular Atrophy (HCSMA)
Canine Motor Neuron Disease

Brief Description

Spinal muscular atrophy affects nerves that control movement throughout the spinal cord and brain, leading to weakness and wasting of the affected muscles.

Presenting Signs

This condition can have an accelerated, intermediate or long term course. In the accelerated condition, first signs appear in puppies between six and eight weeks of age. In this form there is general weakness with poor weight gain and often a mild twitching of the head. These symptoms progress rapidly to a severe wasting of muscles of the limbs, especially the hind legs, neck and head muscles as well as the muscles for breathing, leaving the animal unable to move and breathe properly. The intermediate form begins later and has a slower progression but shows similar signs. The chronic course has its onset in adulthood with only mild clinical signs and slow progression, and dogs can survive several years. A muscle biopsy is used to give the most certain confirmation of diagnosis in the adult dog.

Groups Affected
Gundog
Terrier
Utility
Working dog


Breeds Affected
Brittany
Cairn terrier
English pointer
German shepherd dog
Rottweiler


Related Disorders

Progressive neuronopathy
Focal spinal muscular atrophy
Neuronal abiotrophy

PubMed References
Spinal Muscular Atrophy

Further Reference Material [OMIA Number]
939


Contributor
Nina Lorenz