{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T18:28:22Z","timestamp":1776796102832,"version":"3.51.2"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T00:00:00Z","timestamp":1695772800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is a rare neurodegenerative disease for which there is no specific treatment. Very few cases have been treated with single-target deep brain stimulation (DBS), and the results were not satisfactory. We applied multi-target DBS to an SCA3\/MJD patient and performed positron emission computed tomography (PET) before and after DBS to explore the short-term clinical therapeutic effect.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and methods<\/jats:title><jats:p>A 26-year-old right-hand-dominant female with a family history of SCA3\/MJD suffered from cerebellar ataxia and dystonia. Genetic testing indicated an expanded CAG trinucleotide repeat in the <jats:italic>ATXN3<\/jats:italic> gene and a diagnosis of SCA3\/MJD. Conservative treatment had no obvious effect; therefore, leads were implanted in the bilateral dentate nucleus (DN) and the globus pallidus internus (GPi) and connected to an external stimulation device. The treatment effect was evaluated in a double-blind, randomized protocol in five phases (over a total of 15 days): no stimulation, GPi, DN, or sham stimulation, and combined GPi and DN stimulation. <jats:sup>18<\/jats:sup>F-fluoro-2-deoxy-d-glucose and dopamine transporter PET, Scale for the Assessment and Rating of Ataxia, Fahn-Tolosa-Marin Clinical Rating Scale for Tremor (FTM), Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), and SF-36 quality of life scores were compared before and after DBS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The Total Scale for the Assessment and Rating of Ataxia scores improved by ~42% (from 24 to 14). The BFMDRS movement scores improved by ~30% (from 40.5 to 28.5). The BFMDRS disability scores improved by ~12.5% (from 16 to 14). Daily living activities were not noticeably improved. Compared with the findings in pre-DBS imaging, <jats:sup>18<\/jats:sup>F-fluoro-2-deoxy-d-glucose uptake increased in the cerebellum, while according to dopamine transporter imaging, there were no significant differences in the bilateral caudate nucleus and putamen.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Multi-target acute stimulation (DN DBS and GPi DBS) in SCA3\/MJD can mildly improve cerebellar ataxia and dystonia and increase cerebellar metabolism.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fneur.2023.1246430","type":"journal-article","created":{"date-parts":[[2023,9,28]],"date-time":"2023-09-28T04:16:35Z","timestamp":1695874595000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Case report: Short-term efficacy and changes in 18F-FDG-PET with acute multi-target stimulation in spinocerebellar ataxia type 3 (SCA3\/MJD)"],"prefix":"10.3389","volume":"14","author":[{"given":"Zhiqiang","family":"Cui","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yina","family":"Lan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yan","family":"Chang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xinyun","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jian","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xin","family":"Lou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruimin","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,9,27]]},"reference":[{"key":"B1","article-title":"Spinocerebellar ataxia type 3","volume-title":"GeneReviews","author":"Paulson","year":""},{"key":"B2","unstructured":"Machado-Joseph disease\/spinocerebellar ataxia type 343749\n            PaulsonH\n          10.1016\/B978-0-444-51892-7.00027-921827905Handbook Clin Neurol.1032012"},{"key":"B3","doi-asserted-by":"publisher","first-page":"2075","DOI":"10.1212\/WNL.0000000000002204","article-title":"Deep brain stimulation of the dentate nucleus improves cerebellar ataxia after cerebellar stroke","volume":"85","author":"Teixeira","year":"2015","journal-title":"Neurology."},{"key":"B4","doi-asserted-by":"publisher","first-page":"1714","DOI":"10.1007\/s00415-018-8922-8","article-title":"Globus pallidus internus stimulation in spino-cerebellar ataxia type 3","volume":"265","author":"Aupy","year":"2018","journal-title":"J Neurol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1016\/j.parkreldis.2019.10.029","article-title":"Effects of dentate nucleus stimulation in spinocerebellar ataxia type 3","volume":"69","author":"Cury","year":"2019","journal-title":"Parkinsonism Relat Disord."},{"key":"B6","doi-asserted-by":"publisher","first-page":"861","DOI":"10.1007\/s12311-021-01326-8","article-title":"Safety and outcomes of dentate nucleus deep brain stimulation for cerebellar ataxia","volume":"21","author":"Cury","year":"2022","journal-title":"Cerebellum."},{"key":"B7","doi-asserted-by":"publisher","first-page":"2544","DOI":"10.1111\/ene.15339","article-title":"Long-term efficacy of bilateral subthalamic deep brain stimulation in the parkinsonism of SCA 3: a rare case report","volume":"29","author":"Kuo","year":"2022","journal-title":"Eur J Neurol."},{"key":"B8","unstructured":"Deep brain stimulation for patients with dystonia in Machado-Joseph disease: three case reports32615\n            IkezawaJ\n            YokochiF\n            OkiyamaR\n            IsooA\n            AgariT\n            SunamiY\n          10.1007\/s00415-023-11627-z36862147J Neurol.2702023"},{"key":"B9","doi-asserted-by":"publisher","first-page":"1717","DOI":"10.1212\/01.wnl.0000219042.60538.92","article-title":"Scale for the assessment and rating of ataxia: development of a new clinical scale","volume":"66","author":"Schmitz-H\u00fcbsch","year":"2006","journal-title":"Neurology."},{"key":"B10","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1212\/WNL.35.1.73","article-title":"Validity and reliability of a rating scale for the primary torsion dystonias","volume":"35","author":"Burke","year":"1985","journal-title":"Neurology."},{"key":"B11","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1016\/j.neuchi.2020.04.132","article-title":"Dentate nucleus as target for deep brain stimulation in dystono-dyskinetic syndromes","volume":"66","author":"Nicholson","year":"2020","journal-title":"Neurochirurgie."},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1227\/NEU.0000000000000293","article-title":"The dentate nucleus and its projection system in the human cerebellum: the dentate nucleus microsurgical anatomical study","author":"Akakin","year":"2014","journal-title":"Neurosurgery"},{"key":"B13","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1007\/978-3-7091-8355-7_14","article-title":"The position and extent of the human dentate nucleus","volume":"21","author":"Gortvai","year":"1974","journal-title":"Acta Neurochir"},{"key":"B14","doi-asserted-by":"publisher","first-page":"400","DOI":"10.25259\/SNI_338_2021","article-title":"Dentate nucleus deep brain stimulation: technical note of a novel methodology assisted by tractography","volume":"12","author":"Diniz","year":"2021","journal-title":"Surg Neurol Int."},{"key":"B15","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1159\/000100159","article-title":"Computerized tomography-guided stereotactic dentatotomy","volume":"49","author":"Weigel","year":"1986","journal-title":"Appl Neurophysiol."},{"key":"B16","first-page":"271","article-title":"Clinical rating scale for tremor","volume-title":"Parkinson's Disease and Movement Disorders","author":"Fahn","year":"1993"},{"key":"B17","doi-asserted-by":"publisher","first-page":"2371","DOI":"10.1002\/mds.23834","article-title":"Health-related quality-of-life scales in Parkinson's disease: critique and recommendations","volume":"26","author":"Martinez-Martin","year":"2011","journal-title":"Mov Disord."},{"key":"B18","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1093\/geront\/9.3_Part_1.179","article-title":"Assessment of older people: self-maintaining and instrumental activities of daily living","volume":"9","author":"Lawton","year":"1969","journal-title":"Gerontologist."},{"key":"B19","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/0022-3956(75)90026-6","article-title":"\u201cMini-mental state\u201d. A practical method for grading the cognitive state of patients for the clinician","volume":"12","author":"Folstein","year":"1975","journal-title":"J Psychiat Res."},{"key":"B20","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1111\/j.1532-5415.2005.53221.x","article-title":"The montreal cognitive assessment, MoCA: a brief screening tool for mild cognitive impairment","volume":"53","author":"Nasreddine","year":"2005","journal-title":"J Am Geriatr Soc."},{"key":"B21","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1002\/mpr.323","article-title":"Identifying HAM-A cutoffs for mild, moderate, and severe generalized anxiety disorder","volume":"19","author":"Matza","year":"2010","journal-title":"Int J Methods Psychiatr Res."},{"key":"B22","doi-asserted-by":"publisher","first-page":"384","DOI":"10.1016\/j.jad.2013.04.028","article-title":"Severity classification on the Hamilton Depression Rating Scale","volume":"150","author":"Zimmerman","year":"2013","journal-title":"J Affect Disord."},{"key":"B23","unstructured":"Neural correlates of ataxia severity in spinocerebellar ataxia type 3\/Machado-Joseph disease7\n            Hernandez-CastilloCR\n            DiazR\n            Campos-RomoA\n            Fernandez-RuizJ\n          10.1186\/s40673-017-0065-728593048Cerebellum Ataxias.42017"},{"key":"B24","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1016\/j.neulet.2006.09.007","article-title":"Dissociation of grey and white matter reduction in spinocerebellar ataxia type 3 and 6: a voxel-based morphometry study","volume":"408","author":"Lukas","year":"2006","journal-title":"Neurosci Lett."},{"key":"B25","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1093\/brain\/aws369","article-title":"Genotype-specific patterns of atrophy progression are more sensitive than clinical decline in SCA1, SCA3 and SCA6","volume":"136","author":"Reetz","year":"2013","journal-title":"Brain."},{"key":"B26","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1161\/01.RES.44.1.127","article-title":"The [18F]fluorodeoxyglucose method for the measurement of local cerebral glucose utilization in man","volume":"44","author":"Reivich","year":"1979","journal-title":"Circ Res."},{"key":"B27","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1111\/j.1471-4159.1977.tb03919.x","article-title":"Relation between physiological function and energy metabolism in the central nervous system","volume":"29","author":"Sokoloff","year":"1977","journal-title":"J Neurochem."},{"key":"B28","doi-asserted-by":"publisher","first-page":"1267","DOI":"10.2174\/1567205015666180913102615","article-title":"Crossed cerebellar diaschisis in Alzheimer's disease","volume":"15","author":"Reesink","year":"2018","journal-title":"Curr Alzheimer Res."},{"key":"B29","doi-asserted-by":"publisher","first-page":"1601","DOI":"10.1093\/brain\/124.8.1601","article-title":"Networks mediating the clinical effects of pallidal brain stimulation for Parkinson's disease: a PET study of resting-state glucose metabolism","volume":"124","author":"Fukuda","year":"2001","journal-title":"Brain"},{"key":"B30","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1159\/000455930","article-title":"Modified fluorodeoxyglucose metabolism in motor circuitry by subthalamic deep brain stimulation","volume":"95","author":"Cao","year":"2017","journal-title":"Stereotact Funct Neurosurg."},{"key":"B31","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1097\/01.WCB.0000092831.44769.09","article-title":"Subthalamic nucleus stimulation restores glucose metabolism in associative and limbic cortices and in cerebellum: evidence from a FDG-PET study in advanced Parkinson's disease","volume":"24","author":"Hilker","year":"2004","journal-title":"J Cereb Blood Flow Metab."},{"key":"B32","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1111\/j.1525-1403.2007.00110.x","article-title":"Changes in glucose metabolism in cerebral cortex and cerebellum correlate with tremor and rigidity control by subthalamic nucleus stimulation in Parkinson's disease: a positron emission tomography study","volume":"10","author":"Nagaoka","year":"2007","journal-title":"Neuromodulation."}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2023.1246430\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,28]],"date-time":"2023-09-28T04:16:42Z","timestamp":1695874602000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2023.1246430\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9,27]]},"references-count":32,"alternative-id":["10.3389\/fneur.2023.1246430"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2023.1246430","relation":{},"ISSN":["1664-2295"],"issn-type":[{"value":"1664-2295","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,9,27]]},"article-number":"1246430"}}