The striking effects of deep cerebellar stimulation on generalized fixed dystonia: case report

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Cerebellar neuromodulation could influence the pathological abnormalities of movement disorders through several connections between the cerebellum and the basal ganglia or other cortices. In the present report, the authors demonstrate the effects of cerebellar deep brain stimulation (DBS) on a patient with severe generalized fixed dystonia (FD) that was refractory to bilateral pallidotomy and intrathecal baclofen therapy. A previously healthy 16-year-old girl presented with generalized FD. Bilateral pallidotomy and intrathecal baclofen therapy had failed to resolve her condition, following which she received DBS through the bilateral superior cerebellar peduncle (SCP) and dentate nucleus (DN). Ipsilateral stimulation of the SCP or DN improved the FD, and the ability of DBS administered via the SCP to relax muscles was better than that of DN DBS. A considerable improvement of generalized FD, from a bedridden state to a wheelchair-bound state, was observed in the patient following 6 months of chronic bilateral DBS via the SCP; moreover, the patient was able to move her arms and legs. The findings in the present case suggest that neuromodulation of deep cerebellar structures is a promising treatment for FD that is refractory to conventional treatments.

ABBREVIATIONS AS = Ashworth Scale; BADS = Barry-Albright Dystonia Rating Scale; DBS = deep brain stimulation; DN = dentate nucleus; FD = fixed dystonia; MCS = motor cortex stimulation; SCP = superior cerebellar peduncle; VAS = visual analog scale.
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Contributor Notes

Correspondence Shiro Horisawa: Neurological Institute, Tokyo Women’s Medical University, Tokyo, Japan. neurosurgery21@yahoo.co.jp.INCLUDE WHEN CITING Published online March 1, 2019; DOI: 10.3171/2018.11.JNS182180.Disclosures Dr. Taira received consulting fees and a speaking fee from Daiichi-Sankyo.
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