AKAGAWA HIROYUKI
Department Research Institutes and Facilities, Research Institutes and Facilities Position Associate Professor |
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Article types | Case report |
Language | English |
Peer review | Peer reviewed |
Title | Radiofrequency ablation for DYT-28 dystonia: short term follow-up of three adult cases. |
Journal | Formal name:Annals of clinical and translational neurology Abbreviation:Ann Clin Transl Neurol ISSN code:23289503/23289503 |
Domestic / Foregin | Foregin |
Volume, Issue, Page | 7(10),pp.2047-2051 |
Author and coauthor | Horisawa Shiro†, Azuma Kenkou, Akagawa Hiroyuki, Nonaka Taku, Kawamata Takakazu, Taira Takaomi |
Publication date | 2020/10 |
Summary | Mutations in the lysine methyltransferase 2B (KMT2B) gene have recently been reported to be associated with childhood-onset generalized dystonia. There have been no studies investigating ablative treatments for the management of this disorder. Three patients underwent either a staged unilateral pallidotomy and contralateral pallidothalamic tractotomy (19-year-old man, 2-year follow-up), a unilateral pallidothalamic tractotomy (34-year-old man, 6-month follow-up) or a simultaneous unilateral pallidothalamic tractotomy and ventro-oral thalamotomy (29-year-old man, 6-month follow-up). The average total patient score on the Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale improved from 39.5 to 13.2 (66.6%) after the procedures. No significant complications were identified. Ablative treatments appear to be a promising alternative surgical option for generalized dystonia with KMT2B mutation. |
DOI | 10.1002/acn3.51170 |
PMID | 32886413 |