This protocol covers patients with hereditary spinocerebellar ataxia who present with generalised spasticity — a functionally significant complication characterised by increased muscle tone arising from upper motor neurone involvement.
Spasticity is the presence of increased muscle tone or hypertonia, caused by a lesion of the upper motor neurones which can cause muscle stiffness, spasms and pain.
Spasticity has also been described more rarely in a range of other autosomal recessive ataxias, spastic paraplegias and autosomal dominant ataxias including SCAs 1–3, 6–8, 10–12, 17, 23, 28, 30, and 35.
The initial approach in this setting involves physiotherapy — with a focus on patient and carer education regarding posture, muscle use, and avoidance of spasticity triggers.
Reduction of spasticity, aiming for complete benefit through the physiotherapy approach before further management steps are considered.