Journal
PARKINSONISM & RELATED DISORDERS
Volume 63, Issue -, Pages 191-194Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.parkreldis.2019.02.040
Keywords
Spinocerebellar ataxia 38 (SCA38); Cerebellum; Ataxia; Docosahexaenoic acid (DHA); Clinical trial
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Funding
- Fondazione Telethon [GGP14225]
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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 TO (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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