Pallidal stimulation improves pantothenate kinase-associated neurodegeneration
Pallidal stimulation improves pantothenate kinase-associated neurodegeneration
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DOI：
10.1002/ana.20457
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发表时间：
2005-05-01
影响因子：
11.2
通讯作者：
Coubes, P
中科院分区：
文献类型：
--
作者：
Castelnau, P;Cif, L;Coubes, P
泛酸激酶相关神经变性（PKAN）引起进行性全身性肌张力障碍，这在药理学上仍然难以治疗。我们对6例遗传上确诊的PKAN患者进行了双侧内苍白球刺激，这些患者的疼痛痉挛、肌张力障碍和肢体自主得到了重大而持久的改善。这项研究显示了pallidaldbs治疗PKAN患者肌张力障碍的益处。
Pantothenate kinase-associated neurodegeneration (PKAN) causes a progressive generalized dystonia which remains pharmacologically intractable. We performed bilateral internal globus pallidus stimulation in six patients with genetically confirmed PKAN who obtained a major and long-lasting improvement of their painful spasms, dystonia, and finictional autonomy. This study shows the benefits of pallidal DBS for the dystonia of PKAN patients.