Abstract
Introduction: In recent years, a growing body of literature has investigated the use of non-invasive brain stimulation (NIBS) techniques as a putative treatment in Huntington's Disease (HD). Our aim was to evaluate the effects of cerebellar transcranial Direct Current Simulation (ctDCS) on the motor outcome in patients affected by HD, encompassing at the same time the current knowledge about the effects of NIBS both on motor and non-motor dysfunctions in HD.Materials and Methods: Four patients (two females) were enrolled and underwent ctDCS (both anodal or sham, elapsed by at least 3 months: 2.0 mA, 20 min per day, 5 days a week). Clinical scores were assessed by using the Unified Huntington's Disease Rating Scale – part I (UHDRS-I), immediately before ctDCS (T0), at the end of the 5-days treatment (T1) and 4 weeks later (T2).Results: Anodal ctDCS improved motor scores compared to baseline (p = 0.0046), whereas sham stimulation left them unchanged (p = 0.33, Friedman test). In particular, following anodal ctDCS, UHDRS-I score significantly improved, especially regarding the subitem “dystonia,” both at T1 and T2 compared to sham condition (p < 0.05; Wilcoxon matched-pairs signed test).Conclusions: ctDCS improved motor scores in HD, with effects lasting for about 4 weeks after tDCS completion. This is the first study discussing the putative role of cerebellar non-invasive simulation for the treatment of HD.
Highlights
In recent years, a growing body of literature has investigated the use of non-invasive brain stimulation (NIBS) techniques as a putative treatment in Huntington’s Disease (HD)
Four HD patients were enrolled in a timeline ranging from April 2016 to November 2018
Cerebellar direct current polarization improved motor scores in HD patients, especially dystonia, with the effects lasting for 1 month after the end of stimulation
Summary
A growing body of literature has investigated the use of non-invasive brain stimulation (NIBS) techniques as a putative treatment in Huntington’s Disease (HD). The disease is caused by an expanded CAG trinucleotide repeat (of variable length) in HTT, the gene encoding the protein huntingtin; NIBS in Huntington’s Disease it is inherited in an autosomal dominant manner, with agedependent penetrance, where longer CAG repeats predicting earlier onset [3]. Non-invasive brain stimulation techniques (NIBS), as repetitive Transcranial Magnetic Stimulation (rTMS) and transcranial Direct Current Stimulation (tDCS), have reported conflicting results in the treatment of severe choreic and dystonic movements, failing to provide a significant, possibly long-lasting, clinical improvement in HD patients [4,5,6,7,8]
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