Sathe Kiran P, Hegde Anaita U, Doshi Paresh K
Department of Pediatrics, Jaslok Hospital and Research Centre, Mumbai, India.
J Pediatr Neurosci. 2013 Jan;8(1):46-8. doi: 10.4103/1817-1745.111423.
Pantothenate kinase-associated neurodegeneration (PKAN) is an uncommon extrapyramidal movement disorder characterized by the progressive incapacitating dystonia. Medical management is often incapable of reversing the dystonic symptoms. In recent years, stereotactic procedure like deep brain stimulation has been found effective in resolving the disabling dystonia and improving the quality of life. There are few cases in the world literature highlighting the usefulness of this technique. We report a case of 10-year-old girl who underwent bilateral Globus pallidus internus stimulation for PKAN.
泛酸激酶相关神经变性(PKAN)是一种罕见的锥体外系运动障碍,其特征为进行性失能性肌张力障碍。药物治疗往往无法逆转肌张力障碍症状。近年来,已发现立体定向手术如脑深部电刺激在解决致残性肌张力障碍和改善生活质量方面有效。世界文献中鲜有病例突出该技术的效用。我们报告一例10岁女孩因PKAN接受双侧内侧苍白球刺激的病例。