From the Research
The treatment approach for Spinocerebellar Ataxia type 3 (SCA3) focuses on symptom management as there is currently no cure or disease-modifying therapy available. Treatment typically involves a multidisciplinary approach with physical therapy to maintain mobility and balance, occupational therapy to assist with daily activities, and speech therapy for dysarthria and dysphagia. Pharmacological management includes medications targeted at specific symptoms: baclofen (10-25 mg three times daily) or tizanidine (2-4 mg three times daily) for spasticity; levodopa (starting at 100 mg/day, increasing gradually) for parkinsonian features; amantadine (100 mg twice daily) for dystonia; and medications like meclizine (25 mg as needed) for vertigo. Depression and anxiety may be treated with SSRIs such as sertraline (50-200 mg daily), however, it is essential to consider the potential exacerbation of spasticity by certain antidepressants 1. Regular follow-up with a neurologist is essential to adjust medications as the disease progresses. Genetic counseling should be offered to patients and families. Supportive care becomes increasingly important in advanced stages, including assistive devices for mobility, swallowing evaluations to prevent aspiration, and management of urinary symptoms. This symptomatic approach aims to maintain quality of life and functional independence as long as possible, while research continues to develop potential disease-modifying treatments targeting the underlying CAG repeat expansion in the ATXN3 gene, as highlighted in recent reviews 2.
Some key points to consider in the management of SCA3 include:
- The importance of a multidisciplinary approach to symptom management
- The use of medications such as baclofen and tizanidine for spasticity, with consideration of their potential side effects and interactions 3, 4
- The need for regular follow-up and adjustment of medications as the disease progresses
- The importance of genetic counseling and supportive care in advanced stages of the disease
- The ongoing research into potential disease-modifying treatments for SCA3, which may offer hope for improved outcomes in the future 2.