Radiofrequency ablation for DYT‐28 dystonia: short term follow‐up of three adult cases

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 pall...

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Bibliographic Details
Published inAnnals of clinical and translational neurology Vol. 7; no. 10; pp. 2047 - 2051
Main Authors Horisawa, Shiro, Azuma, Kenkou, Akagawa, Hiroyuki, Nonaka, Taku, Kawamata, Takakazu, Taira, Takaomi
Format Journal Article
LanguageEnglish
Published Bognor Regis John Wiley & Sons, Inc 01.10.2020
John Wiley and Sons Inc
Wiley
Subjects
Ablation
Age
Botulinum toxin
Case Study
Dystonia
Hospitals
Larynx
Microcephaly
Mutation
Oral administration
Patients
Surgical outcomes
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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.
Bibliography:This study was supported by the Japan Brain Foundation.
Funding Information
ISSN:2328-9503
2328-9503
DOI:10.1002/acn3.51170