Harries Anwen M, Sandhu Mandeep, Spacey Sian D, Aly Mohamed M, Honey Christopher R
Division of Neurosurgery, University of British Columbia, Vancouver, B.C., Canada.
Stereotact Funct Neurosurg. 2013;91(4):233-5. doi: 10.1159/000345265. Epub 2013 Mar 26.
BACKGROUND/AIMS: This paper describes the use of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in the treatment of secondary dystonia caused by expisodic ataxia type 2 (EA2).
We present the case of a patient with EA2, an autosomal dominant condition, who developed late-onset cervical and right upper limb segmental dystonia. The patient underwent left GPi DBS.
Within 4 months of commencing stimulation of the left GPi, the patient had resolution of his neck pain and was able to keep the head straighter for longer time intervals. There was also improvement in right arm segmental dystonia. There was an improvement in the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS 21.5) of 55% at 4 months and of 51% at 22 months.
The treatment of secondary dystonia is difficult and the results with GPi DBS are less favourable compared with primary dystonia. This case illustrates the successful treatment of secondary dystonia caused by EA2.
背景/目的：本文描述了苍白球内侧部（GPi）的深部脑刺激（DBS）在治疗由发作性共济失调2型（EA2）引起的继发性肌张力障碍中的应用。
我们报告了一例患有常染色体显性疾病EA2的患者，该患者出现迟发性颈部和右上肢节段性肌张力障碍。该患者接受了左侧GPi DBS治疗。
在开始刺激左侧GPi后的4个月内，患者的颈部疼痛消失，并且能够更长时间地保持头部挺直。右臂节段性肌张力障碍也有所改善。在4个月时，多伦多西部痉挛性斜颈评定量表（TWSTRS 21.5）改善了55%，在22个月时改善了51%。
继发性肌张力障碍的治疗较为困难，与原发性肌张力障碍相比，GPi DBS的治疗效果较差。本病例说明了EA2引起的继发性肌张力障碍的成功治疗。
