Deep brain stimulation for patients with dystonia in Machado–Joseph disease: three case reports

Case report: Short-term efficacy and changes in 18F-FDG-PET with acute multi-target stimulation in spinocerebellar ataxia type 3 (SCA3/MJD)

“…Daily living was barely improved; therefore, because the patient had high expectations for the procedure, she was slightly anxious about the efficacy of the treatment. Only a few SCA3/MJD cases with DBS treatment have been reported, and all of them were single-target DBS (3)(4)(5)(6)(7)(8). In 2015, Teixeira et al reported a 50-year-old right-handed woman who underwent resection of a right acoustic neuroma, which was complicated by an ischemic injury of the right cerebellar hemisphere.…”

“…It is situated adjacent to the fourth ventricle, buried in white matter, and measures ~9–20 mm in width, 13–23 mm in length, and 7–20 mm in height. It has a unique shape and is oriented in a craniocaudal direction and from lateral to medial ( 8 , 9 , 11 – 13 ). With reference to previous studies ( 14 , 15 ), we targeted the tip of each stimulation lead to the origin of the superior cerebellar peduncle such that the electrode contacted the anterior and upper third of the DN, a region related to motor function.…”

“…Though ataxia syndromes have not been formally addressed with DBS, patients with ataxia, tremor, or dystonia have been administered DBS in different targets, resulting in partial relief of symptoms. Studies reporting treatment of SCA3/MJD with DBS have targeted the thalamus and cerebellum (3)(4)(5)(6)(7)(8). These treatments were singletarget stimulations, and the results were not very satisfactory.…”