Table 5. [Treatment of Manifestations of SCA 7: Adolescent or Adult Onset]. - 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 ADLs (incl use of adaptive devices, e.g., weighted eating utensils & dressing hooks) Consider adaptive devices to maintain/improve independence in mobility (e.g., canes, walkers, motorized chairs). Inpatient rehab w/OT/PT may improve ataxia & functional abilities. ^2, 3 Weight control to avoid obesity Home adaptations to prevent falls (e.g., grab bars, raised toilet seats) & improve mobility (e.g., ramps to accommodate motorized chairs)
Pharmacologic treatment	Therapies intended to ↓ symptoms work variably well in different individuals. Most commonly used drugs: amantadine, buspirone, riluzole
TMS	This treatment modality is still being evaluated, w/most promising initial results obtained w/cerebellar repetitive TMS.
UMN involvement (spasticity)	Pharmacologic treatment	Consider pharmacologic treatment of generalized spasticity w/oral medications (usually in this order due to the profile of side effects & better tolerance): baclofen, tizanidine, gabapentin, clonazepam, dantrolene sodium, diazepam
LMN involvement (weakness)	Mainly supportive	Braces, orthotics, PT
Ophthalmologic involvement	Use of low vision aids	Consultation w/agencies for visually impaired
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.
Weight	Nutrition assessment	Consider nutritional & vitamin supplementation to meet dietary needs. Avoid obesity (which can exacerbate difficulties with ambulation & mobility).
Bladder dysfunction	Pharmacologic treatment	If physical rehab or biofeedback do not remedy problem, consider anticholinergic drugs for overactive bladder. Anticholinergic agents are also indicated for neurogenic bladder. Botulinum toxin injections should be reserved for severe or unresponsive bladder dysfunction.
Restless legs syndrome	Pharmacologic treatment	Levodopa or dopamine agonist
Chronic pain	Requires specialist eval & management	Refer to pain clinic or pain specialist.
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/
Concern

Treatment

Considerations/Other

Cerebellar ataxia

PT/OT

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

Pharmacologic treatment

Therapies intended to ↓ symptoms work variably well in different individuals.
Most commonly used drugs: amantadine, buspirone, riluzole

TMS

This treatment modality is still being evaluated, w/most promising initial results obtained w/cerebellar repetitive TMS.

UMN involvement
(spasticity)

Pharmacologic treatment

Consider pharmacologic treatment of generalized spasticity w/oral medications (usually in this order due to the profile of side effects & better tolerance): baclofen, tizanidine, gabapentin, clonazepam, dantrolene sodium, diazepam

LMN involvement
(weakness)

Mainly supportive

Braces, orthotics, PT

Ophthalmologic
involvement

Use of low vision aids

Consultation w/agencies for visually impaired

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.

Weight

Nutrition assessment

Consider nutritional & vitamin supplementation to meet dietary needs.
Avoid obesity (which can exacerbate difficulties with ambulation & mobility).

Bladder
dysfunction

Pharmacologic treatment

If physical rehab or biofeedback do not remedy problem, consider anticholinergic drugs for overactive bladder.
Anticholinergic agents are also indicated for neurogenic bladder.
Botulinum toxin injections should be reserved for severe or unresponsive bladder dysfunction.

Restless legs
syndrome

Pharmacologic treatment

Levodopa or dopamine agonist

Chronic pain

Requires specialist eval & management

Refer to pain clinic or pain specialist.

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: Spinocerebellar Ataxia Type 7

GeneReviews^® [Internet].

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

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