This protocol addresses the specific situation in which tremor — a significant and intrusive complaint in hereditary spinocerebellar ataxia — has not responded adequately to an optimised course of pharmacological management, and a structured next-line approach is indicated.
Tremor is a recognised and often intrusive complaint in patients with hereditary spinocerebellar ataxia. It is classically of the intention type, with the amplitude of shaking increasing as the target is approached, and can substantially impair daily function.
A sequential pharmacological trial was undertaken, working through agents including propranolol, primidone, topiramate, clonazepam, and gabapentin (individually and in combination where appropriate), alongside physiotherapy. The primary goal — relief of tremor symptoms — was not achieved. This failure of pharmacological management is the condition that escalates care to the protocol below.
Tremor can be an intrusive complaint in some patients with ataxia, and is classically described as being of "intention" type (with the amplitude of shaking increasing as the target is approached).
In patients where tremor is extremely debilitating and not responsive to medication, a referral to a centre specialising in functional neurosurgery should be considered.
This is because there may be a useful role for functional neurosurgery, including deep brain stimulation (DBS), in the management of tremor, although here too studies specifically for the management of cerebellar tremor are lacking.
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