Open AccessRadiofrequency ablation for DYT‐28 dystonia: short term follow‐up of three adult cases
Author(s) -
Horisawa Shiro,
Azuma Kenkou,
Akagawa Hiroyuki,
aka Taku,
Kawamata Takakazu,
Taira Takaomi
Publication year - 2020
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.51170
Subject(s) - medicine , dystonia , thalamotomy , pallidotomy , surgery , movement disorders , rating scale , ablation , deep brain stimulation , disease , psychiatry , parkinson's disease , psychology , developmental psychology
Abstract 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.