Table 6. [Treatment of Manifestations in Individuals with a Hereditary Ataxia]. - GeneReviews®

Manifestation	Treatment	Considerations/Other
Ataxia	Care by physiatrist, OT/PT	Consider adaptive devices to maintain/improve independence in mobility (e.g., canes, walkers, ramps to accommodate motorized chairs), feeding (e.g., weighted eating utensils), & dressing (e.g., dressing hooks). PT (balance exercises, gait training, muscle strengthening) to maintain mobility & function ¹ OT to optimize ADL Inpatient rehab w/OT/PT may improve ataxia & functional abilities in persons w/degenerative ataxias. ^2, 3 Weight control to avoid obesity Home adaptations to prevent falls (e.g., grab bars, raised toilet seats)
Pharmacologic treatment	Consider riluzole (100 mg/d ³), the only drug shown to improve ataxia symptoms in persons w/ataxia of mixed etiologies; use requires monitoring of liver enzymes.
Transcranial magnetic stimulation	Consider transcranial magnetic stimulation over cerebellum, ² which may improve cerebellar motor signs after 21 daily treatments (tested in persons w/various causes of spinocerebellar degeneration).
UMN involvement (spasticity)	Pharmacologic treatment	Baclofen, tizanidine, or dantrolene may relieve muscle spasms & spasticity.
Dysarthria	Speech-language therapy	Consider alternative communication methods as needed (e.g., writing pads & digital devices).
Dysphagia	Modify food consistency to ↓ aspiration risk.	Video esophagram may help define best consistency.
Poor weight gain	Nutrition assessment	Consider nutritional & vitamin supplementation to meet dietary needs.
Scoliosis / Skeletal involvement	Surgical treatment	Refer to orthopedic surgeon when required.
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^®. ⁴

Manifestation

Treatment

Considerations/Other

Ataxia

Care by physiatrist, OT/PT

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

Pharmacologic treatment

Consider riluzole (100 mg/d ³), the only drug shown to improve ataxia symptoms in persons w/ataxia of mixed etiologies; use requires monitoring of liver enzymes.

Transcranial magnetic stimulation

Consider transcranial magnetic stimulation over cerebellum, ² which may improve cerebellar motor signs after 21 daily treatments (tested in persons w/various causes of spinocerebellar degeneration).

UMN involvement (spasticity)

Pharmacologic treatment

Baclofen, tizanidine, or dantrolene may relieve muscle spasms & spasticity.

Dysarthria

Speech-language therapy

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

Dysphagia

Modify food consistency to ↓ aspiration risk.

Video esophagram may help define best consistency.

Poor weight gain

Nutrition assessment

Consider nutritional & vitamin supplementation to meet dietary needs.

Scoliosis / Skeletal involvement

Surgical treatment

Refer to orthopedic surgeon when required.

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^®. ⁴

From: Hereditary Ataxia Overview

GeneReviews^® [Internet].

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

Seattle (WA): University of Washington, Seattle; 1993-2024.

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