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

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.

Unilateral pallidothalamic tractotomy at Forel's field H1 for cervical dystonia

“…considered transiently decreased consciousness after pallidothalamic tractotomy (author called campotomy) resulting from lesion encroachment on the ascending reticular activating system. Other previous and current studies of pallidothalamic tractotomy for dystonia, Huntington's disease, and epilepsy did not report transient somnolence 11,32–35 . Here, only two out of 35 patients had transient somnolence (5.7%).…”

“…Other previous and current studies of pallidothalamic tractotomy for dystonia, Huntington's disease, and epilepsy did not report transient somnolence. 11,[32][33][34][35] Here, only two out of 35 patients had transient somnolence (5.7%).…”

Neurosurgical and pharmacological management of dystonia

“…MRgFUS may be the preferred technique for surgical ablation as more transient and less severe adverse effects have been reported compared with radiofrequency ablation[ 86 , 101 , 102 ]. For radiofrequency ablation, electrode placement requires craniotomy or a small burr hole[ 103 , 104 ]. However, recent trials have demonstrated safe bilateral lesioning with this technique[ 104 ].…”

“…For radiofrequency ablation, electrode placement requires craniotomy or a small burr hole[ 103 , 104 ]. However, recent trials have demonstrated safe bilateral lesioning with this technique[ 104 ]. Additionally, the capacity for region mapping prior to the procedure gives radiofrequency ablation an upper hand over stereotactic radiosurgery in preoperative surgical preparation.…”