Managing Upper Limb Dysfunction in Friedreich Ataxia
Impaired upper limb function is a recognised manifestation of Friedreich ataxia that warrants a structured, evidence-informed clinical approach. Guideline recommendations address both the rehabilitation framework and specific physical interventions suited to this presentation.
Clinical scenario
This protocol applies to individuals with Friedreich ataxia who present with upper limb dysfunction — including impaired reach, grip, coordination, or limb control — that affects daily function. The recommendations are specifically tailored to those experiencing these upper limb deficits, including those with spasticity, spasm, or contracture.
Approach overview
Management is centred on intensive rehabilitation delivered in a clinical setting. Physical and assistive interventions may be considered for individuals with specific presentations such as spasticity or contracture. Current guidelines also provide clear direction on which intervention modalities are appropriate and which are not recommended for this population.
References
DOI: 10.1186/s13023-022-02568-3
- We conditionally recommend intensive upper limb rehabilitation for individuals with Friedreich ataxia in a clinical setting
- We conditionally recommend considering upper limb splinting/orthoses for individuals with Friedreich ataxia who experience spasticity, spasm or contracture
- We conditionally recommend considering electrical stimulation (but not tDCS or TMS) for management of upper limb function in individuals with Friedreich ataxia in a clinical setting, with appropriate evaluation prior to treatment
- We recommend against the use of pharmacological agents (baclofen and botulinum toxin) for specific management of upper limb function in individuals with Friedreich ataxia experiencing spasticity and spasm