A Postural Tremor Highly Responsive to Transcranial Cerebello-Cerebral DCS in ARCA3

Abstract

Background and objectivesCerebellar ataxias are disabling disorders that impact the quality of life of patients. In many cases, an effective treatment is missing. Despite the increasing knowledge on the pathogenesis of cerebellar disorders including genetic aspects, there is currently a gap in the therapeutical management of cerebellar deficits. Cerebellar ataxia associated with ANO10 mutation (ARCA3) presents a disabling cerebellar syndrome. The aim of this study is to report a patient with a marked postural tremor responding to transcranial cerebello-cerebral direct current stimulation (tCCDCS).MethodsWe applied tCCDCS using anodal stimulation over the cerebellum with a return electrode on the contralateral motor cortex. We performed a clinical rating, accelerometry studies, and recordings of voluntary movements at baseline, after sham, and after active tCCDCS.ResultsA dramatic response of postural tremor was observed after tCCDCS, with a major drop of the power spectral density to 26.12% of basal values.DiscussionThe postural tremor of cerebellar ataxia associated with ANO10 mutation was highly responsive to tCCDCS in our patient. This case illustrates that tCCDCS is a novel therapeutic option in the treatment of cerebellar deficits and might represent a promising tool to reduce tremor in ARCA3.

Consensus Paper: Cerebellum and Emotion

“…Figure 2c shows the different TMS protocols commonly applied [10,25,26]. Cerebellar tDCS might be beneficial for patients with cerebellar neurodegenerative diseases such as ataxia (e.g., spinocerebellar ataxias (SCAs)) [27][28][29] and tremor (e.g., essential tremor (ET)) [30][31][32], but only a few studies have assessed the role of cerebellar stimulation during rehabilitation after cerebellar stroke [e.g., [31][32][33][34]. Despite the substantial differences in the stimulation protocol (four studies; Calzolari et al [33]: excitatory anodal tDCS over the lesioned hemisphere; Torriero et al [34]: inhibitory 1 Hz rTMS over the unlesioned hemisphere; Kim et al [35]: inhibitory 1 Hz rTMS over the lesioned hemisphere; and Bonnì et al [36]: excitatory iTBS over the lesioned hemisphere), all studies found have marked improvements after cerebellar stimulation.…”

Non-invasive Transcranial Electrical Stimulation in Movement Disorders

“…The results of a similar trial with 2 weeks of anodal ctDCS in patients with spinocerebellar ataxia type 3 are awaited (Maas et al, 2019). An improvement of marked postural tremor in cerebellar ataxia associated with ANO10 mutation was noted with cerebello-cerebral stimulation (anode over cerebellum and cathode over M1, 1.5 Ma, 20 min) (Bodranghien et al, 2017). No significant beneficial effect was noted with anodal tDCS to the cerebellum or the motor cortex in grip force control (2 mA, 25 min) (John et al, 2017) or forcefield reaching adaptation (2 mA, 22 min) (Hulst et al, 2017) in patients with cerebellar ataxia.…”

“…Cervical dystonia (McCambridge and Bradnam, 2018;Summers et al, 2018) 8. Cerebellar ataxia (Grimaldi and Manto, 2013;Grimaldi et al, 2014b;Benussi et al, 2015Benussi et al, , 2017bBenussi et al, , 2018Bodranghien et al, 2017;Hulst et al, 2017;John et al, 2017;Maas et al, 2019;Pilloni et al, 2019;Vavla et al, 2019) 9. Essential tremor (Gironell et al, 2014;Helvaci et al, 2016) 10.…”

Noninvasive Cerebellar Stimulation as a Complement Tool to Pharmacotherapy

“…Bodranghien and colleagues (2017) [24] conducted a single case study in a patient with ANO10 mutation (ARCA3). They delivered anodal tCCDCS over the cerebellum with a return electrode on the contralateral motor cortex.…”