Subthalamic Nuclei Stimulation in Patients With Pantothenate Kinase-Associated Neurodegeneration (PKAN)

Subthalamic Nuclei Stimulation in Patients With Pantothenate Kinase-Associated Neurodegeneration (PKAN)

Liu Z; Liu Y; Yang Y; Wang L; Dou W; Guo J; Wang Y; Guo Y; Wan X; Ma W; Wang R

Neuromodulation

2017

adolescent; subthalamic nucleus; follow up; scoring system; human; article; female; male; adult; middle aged; dystonia; case report; treatment outcome; brain depth stimulation; Deep brain stimulation; neurologic disease assessment; Burke Fahn Marsden Dystonia Rating Scale movement rating scale; fluency disorder; globus pallidus internus; neurodegeneration with brain iron accumulation/th [Therapy]; neurologic examination; pantothenate kinase-associated neurodegeneration; subthalamic nuclei; tone and motor problems; IND; surgical intervention; subthalamic nuclei stimulation

Introduction: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as dystonia, ataxia, dysarthria, and involuntary movements. Treatment of PKAN with deep brain stimulation (DBS) has been reported, but mainly focuses on targeting the globus pallidus internus (GPi). Subthalamic nuclei (STN) may also be a potential target for treatment of PKAN. Methods: In this study, we reviewed three patients with PKAN (two with typical PKAN and one with atypical PKAN) treated by bilateral STN stimulation and present a review of the literature. All patients received neurological evaluation using the Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFMDRS) scoring system before and after surgery. Patients were then subject to regular clinical follow-ups (ranging from 22 to 44 months). Results: The mean stimulation amplitude, pulse width and frequency was 2.65 +/- 0.45 V, 91.7 +/- 21.9 mus, and 146.7 +/- 12.5 Hz, respectively. BFMDRS scores were improved in all patients after surgery, ranging from 41.6 to 73.1%. Improvements of appendicular symptoms ranged from 46.2 to 94.1%, and improvements of axial symptoms ranged from 27.3 to 33.3%. No side effects were reported in patients 1 and 2; whereas patient 3 exhibited a mild decline in verbal fluency one year after surgery. Conclusion: STN stimulation could serve as a candidate DBS target in the treatment of PKAN, especially for patients with prominent appendicular symptoms. Copyright © 2017 International Neuromodulation Society

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