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 in:Annals of clinical and translational neurology 2020-10, Vol.7 (10), p.2047-2051
Main Authors: Horisawa, Shiro, Azuma, Kenkou, Akagawa, Hiroyuki, Nonaka, Taku, Kawamata, Takakazu, Taira, Takaomi
Format: Article
Language:English
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.
ISSN:2328-9503
2328-9503
DOI:10.1002/acn3.51170