Table 4. [Treatment of Manifestations in Individuals with Spinocerebellar Ataxia Type 8]. - GeneReviews®

Manifestation/ Concern	Treatment	Considerations/Other
Cerebellar ataxia	PT/OT	PT (balance exercises, gait training, muscle strengthening) to maintain mobility & function ¹ OT to optimize ADL Consider adaptive devices to maintain/improve independence in mobility (e.g., canes, walkers, ramps to accommodate motorized chairs). Inpatient rehab w/OT/PT may improve ataxia & functional abilities in those w/degenerative ataxias. ^2, 3 Weight control to avoid obesity Home adaptations to prevent falls (e.g., grab bars, raised toilet seats)
Pharmacologic treatment	Pharmacotherapy for ataxia is generally disappointing & no approved drugs exist. Anecdotal studies report benefit from drugs incl (e.g.) buspirone, riluzole, & 4-aminopyridine.
Transcranial magnetic stimulation (TMS)	No data for SCA8, but TMS has shown some promise for other ataxias & could be considered.
UMN involvement (spasticity)	Pharmacologic treatment	Drugs incl baclofen & tizanidine may be considered for severe spasticity.
Eyes	Abnormal eye movements may respond to 4-aminopyridine, baclofen, or memantine. Prisms may be used to obviate diplopia.	Expert neuro-ophthalmology consult is useful.
Dysarthria	Speech & language therapy	Consider alternative communication methods as needed (e.g., writing pads & digital devices).
Dysphagia	Feeding therapy programs to improve nutrition & dysphagia & ↓ risk of aspiration	Video esophagram may help define best food consistency. Sensory stimulation (e.g., putting an item such as a straw in the mouth) may ↓ involuntary movements & improve articulation, chewing &/or swallowing. ⁴
Poor weight gain	Nutrition assessment	Consider nutritional & vitamin supplementation to meet dietary needs.
Cognitive/ Psychiatric	Pharmacologic treatment	Standard treatment for psychiatric manifestations (e.g., depression, anxiety, & psychosis)
Psychotherapy / neuropsychological rehab	Consider cognitive & behavioral therapy, incl Goal Management Training^®. ⁵
Dystonia	Consider Botox^® for focal dystonia.
Social support	Social work referral	To assist in identifying sources for in-home &/or local community support

Manifestation/
Concern

Treatment

Considerations/Other

Cerebellar
ataxia

PT/OT

PT (balance exercises, gait training, muscle strengthening) to maintain mobility & function ¹
OT to optimize ADL
Consider adaptive devices to maintain/improve independence in mobility (e.g., canes, walkers, ramps to accommodate motorized chairs).
Inpatient rehab w/OT/PT may improve ataxia & functional abilities in those w/degenerative ataxias. ^2, 3
Weight control to avoid obesity
Home adaptations to prevent falls (e.g., grab bars, raised toilet seats)

Pharmacologic treatment

Pharmacotherapy for ataxia is generally disappointing & no approved drugs exist. Anecdotal studies report benefit from drugs incl (e.g.) buspirone, riluzole, & 4-aminopyridine.

Transcranial magnetic stimulation (TMS)

No data for SCA8, but TMS has shown some promise for other ataxias & could be considered.

UMN involvement (spasticity)

Pharmacologic treatment

Drugs incl baclofen & tizanidine may be considered for severe spasticity.

Eyes

Abnormal eye movements may respond to 4-aminopyridine, baclofen, or memantine.
Prisms may be used to obviate diplopia.

Expert neuro-ophthalmology consult is useful.

Dysarthria

Speech & language therapy

Consider alternative communication methods as needed (e.g., writing pads & digital devices).

Dysphagia

Feeding therapy programs to improve nutrition & dysphagia & ↓ risk of aspiration

Video esophagram may help define best food consistency.
Sensory stimulation (e.g., putting an item such as a straw in the mouth) may ↓ involuntary movements & improve articulation, chewing &/or swallowing. ⁴

Poor weight gain

Nutrition assessment

Consider nutritional & vitamin supplementation to meet dietary needs.

Cognitive/
Psychiatric

Pharmacologic treatment

Standard treatment for psychiatric manifestations (e.g., depression, anxiety, & psychosis)

Psychotherapy / neuropsychological rehab

Consider cognitive & behavioral therapy, incl Goal Management Training^®. ⁵

Dystonia

Consider Botox^® for focal dystonia.

Social support

Social work referral

To assist in identifying sources for in-home &/or local community support

From: Spinocerebellar Ataxia Type 8

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

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