Transcranial Magnetic Resonance-Guided Focused Ultrasound in X-Linked Dystonia-Parkinsonism

Life

Takaomi Taira¹  RolandDominic G. Jamora²  Wei-Chieh Chang³ 

[1] Department of Neurosurgery, Tokyo Women’s Medical University, Tokyo 162-86666, Japan;Division of Adult Neurology, Department of Neurosciences, College of Medicine - Philippine General Hospital, University of the Philippines Manila, Manila 1000, Philippines;MR-Guided Focused Ultrasound Center, Chang Bing Show Chwan Memorial Hospital, Changhua County 50529, Taiwan;

关键词: X-linked dystonia-parkinsonism;    XDP;    pallidothalamic tractotomy;    transcranial magnetic resonance-guided focused ultrasound;    XDP-MDSP Scale;   

DOI  :  10.3390/life11050392

来源: DOAJ

【 摘 要 】

X-linked dystonia-parkinsonism (XDP) is a neurodegenerative condition found among males with maternal ancestry from Panay Island, Philippines. The treatment options are limited. We report on our experience of three XDP patients who underwent transcranial magnetic resonance-guided focused ultrasound (tcMRgFUS) pallidothalamic tractotomy. The three patients were all genetically confirmed XDP, with a mean XDP-Movement Disorder Society of the Philippines (MDSP) Scale score of 68.7/200. All patients were on stable doses of their oral medications and their last botulinum toxin injection was 12 months prior to study. Two patients complained of moderate to severe arm pain 2–7 months after the procedure. There was an overall improvement in the XDP-MDSP Scale score of 36.2% (18.7 vs. 15) at 6 months and 30.1% (68.7 vs. 45.5) at 1 year. Notably, there was worsening of the nonmotor subscale (part IIIB, nonbehavioral aspect) by 350% at 1 year. While these numbers are encouraging, there is a need to do a larger study on the safety and efficacy of tcMRgFUS on XDP.

【 授权许可】

Unknown