Treatment of Hereditary Spinocerebellar Ataxia in Generalised Spasticity

This protocol addresses the management of hereditary spinocerebellar ataxia in the specific context of co-occurring generalised spasticity — increased muscle tone that compounds the neurological burden and requires dedicated treatment alongside the underlying ataxia.

Clinical scenario Generalised spasticity — characterised by increased muscle tone or hypertonia arising from upper motor neurone involvement — can accompany hereditary spinocerebellar ataxia across multiple subtypes. It contributes to muscle stiffness, spasms, and pain, and demands a targeted management approach tailored to this combination.

For this clinical scenario, the structured protocol incorporates a specialised intrathecal delivery approach, undertaken only in an expert centre with careful patient selection and follow-up. The complete evidence-based regimen — including criteria, sequencing, and monitoring — is available via the link below.

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References

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.
If these are not successful or not tolerated, greater CNS concentrations of baclofen can be achieved with reduced peripheral exposure to side effects by the use of an intrathecal baclofen infusion, although this requires careful patient selection, planning and an expert centre to undertake the procedure and follow-up.

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