Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease
Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease
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DOI：
10.1002/mds.21723
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发表时间：
2007-10-31
影响因子：
8.6
通讯作者：
Coubes, Philippe
中科院分区：
文献类型：
--
作者：
Cif, Laura;Biolsi, Brigitte;Coubes, Philippe
Lesch-Nyhan综合征是一种X连锁隐性疾病，由嘌呤补救酶次黄嘌呤-鸟嘌呤磷酸核糖基转移酶缺乏引起。患者表现出典型的神经行为障碍，运动技能获得延迟，肌张力障碍，严重的自残和攻击性行为。深部脑刺激此前曾被提议用于控制孤立的不自主运动和精神障碍。我们对1例患者的边缘和运动内苍白球同时进行双侧同时刺激，以分别控制行为和运动障碍。术后28个月伤害性强迫症状消失，肌张力障碍和运动障碍减轻。(C)2007年运动无序协会。
The Lesch-Nyhan syndrome is an X-linked recessive disorder caused by a deficiency in hypoxanthine-guanine phosphoribosyl transferase, a purine salvage enzyme.Affected individuals exhibit a characteristic neurobehavioral disorder with delayed acquisition of motor skills, dystonia, severe self-mutilations, and aggressive behavior. Deep brain stimulation has been previously proposed for controlling isolated involuntary movements and psychiatric disorders. We applied a double bilateral simultaneous stimulation to limbic and motor internal pallidum in one patient for controlling both behavioral and movement disorders, respectively. The injurious compulsions disappeared; dystonia and dyskinesia were decreased at 28 months follow-up. (C) 2007 Movement Disorder Society.