AI Article Synopsis
- Spinocerebellar Ataxia 38 (SCA38) is linked to a mutation in the ELOVL5 gene, leading to low levels of serum docosahexaenoic acid (DHA), but DHA supplementation has shown short-term benefits.* -
- A 2-year study with nine SCA38 patients evaluated the long-term effects of daily 600 mg DHA, showing sustained improvement in clinical symptoms and increased cerebellar metabolism without any side effects.* -
- The findings suggest that long-term DHA supplementation is a viable treatment option for individuals with SCA38.*
Article Abstract
Introduction: Spinocerebellar Ataxia 38 (SCA38) is caused by ELOVL5 gene mutation, with significant reduction of serum docosahexaenoic acid (DHA) levels. DHA supplementation has been proven effective at short-term follow-up. In the present paper, we evaluated long-term safety and efficacy of 600 mg/day oral DHA in SCA38 by a 2-year open label extension study.
Methods: Nine SCA38 patients underwent standardised clinical assessment at 62 (T1), 82 (T2) and 104 (T3) weeks, and compared to pre-treatment scores (T0). Brain 18-Fluorodeoxyglucose Positron Emission Tomography and electroneurography were performed at T0 and T3.
Results: We found a significant maintenance of clinical symptom improvement at each follow-up time-point (p < 0.001) as compared to T0, a sustained increase of cerebellar metabolism at T3 as compared to T0 (p = 0.013), and no worsening of neurophysiological parameters. No side effect was recorded.
Conclusions: Long-term DHA supplementation is an eligible treatment for SCA38.
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| http://dx.doi.org/10.1016/j.parkreldis.2019.02.040 | DOI Listing |
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