Treatment of Hereditary Spinocerebellar Ataxia in Generalised Spasticity

In hereditary spinocerebellar ataxia, generalised spasticity — increased muscle tone or hypertonia resulting from upper motor neurone involvement — can be a significant and disabling complication, contributing to muscle stiffness, spasms, and pain.

Clinical scenario: A patient with hereditary spinocerebellar ataxia presenting with generalised spasticity. This combination has been documented across a range of autosomal dominant and recessive ataxia subtypes, including multiple SCAs.

Managing spasticity in this population requires a structured, stepwise approach. For cases where conservative measures have not achieved adequate control, the protocol outlines a surgical pathway — the full decision algorithm and patient selection criteria are available in the complete regimen.

Treatment direction

When spasticity has not responded sufficiently to non-surgical management, the protocol considers surgical intervention. This may involve procedures targeting either the musculoskeletal system or — depending on the clinical picture — the nervous system. The complete structured regimen specifies which approach applies and under what circumstances.

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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.
Surgery should be considered in cases where physiotherapy and pharmacological treatments have not worked.
Surgical treatments include orthopaedic procedures such as tendon lengthening, tenotomy or tendon transfer; and neurosurgical procedures such as peripheral neurotomies, dorsal rhizotomies and microsurgical ablation of the dorsal root entry zone ('DREZotomy').

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