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JMD : Journal of Movement Disorders

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Original Article
Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia
Kyung Ah Woo, Han-Joon Kim, Seung-Ho Jeon, Hye Ran Park, Kye Won Park, Seung Hyun Lee, Sun Ju Chung, Jong-Hee Chae, Sun Ha Paek, Beomseok Jeon
J Mov Disord. 2022;15(3):241-248.   Published online July 26, 2022
DOI: https://doi.org/10.14802/jmd.22002
  • 733 View
  • 62 Download
AbstractAbstract PDFSupplementary Material
Objective
To investigate the long-term clinical outcomes of pallidal deep brain stimulation (GPi-DBS) in patients with pantothenate kinase-associated neurodegeneration (PKAN).
Methods
We reviewed the records of patients with genetically confirmed PKAN who received bilateral GPi-DBS for refractory dystonia and were clinically followed up for at least 2 years postoperatively at two centers in Korea. Pre- and postoperative Burke– Fahn–Marsden Dystonia Rating Scale motor subscale (BFMDRS-M) scores, disability subscale (BFMDRS-D) scores, and qualitative clinical information were prospectively collected. Descriptive analysis was performed for BFMDRS-M scores, BFMDRSD scores, and the orofacial, axial, and limb subscores of the BFMDRS-M at 6–12, 24–36, and 60–72 months postoperatively.
Results
Five classic-type, four atypical-type, and one unknown-type PKAN cases were identified. The mean preoperative BFMDRS-M score was 92.1 for the classic type and 38.5 for the atypical or unknown type, with a mean BFMDRS follow-up of 50.7 months and a clinical follow-up of 69.0 months. The mean improvements in BFMDRS-M score were 11.3%, 41.3%, and 30.5% at 6–12, 24–36, and 60–72 months, respectively. In four patients with full regular evaluations until 60–72 months, improvements in the orofacial, axial, and limb subscores persisted, but the disability scores worsened from 24–36 months post-operation compared to the baseline, mainly owing to the aggravation of eating and feeding disabilities.
Conclusion
The benefits of GPi-DBS on dystonia may persist for more than 5 years in PKAN. The effects on patients’ subjective disability may have a shorter duration despite improvements in dystonia owing to the complex manifestations of PKAN.
Letters to the editor
Pseudodystonia and Neuropathic Tremor in a Patient With Monomelic Amyotrophy
Seung Hyun Lee, Yun Su Hwang, Sungyang Jo, Sun Ju Chung
J Mov Disord. 2022;15(2):181-183.   Published online March 22, 2022
DOI: https://doi.org/10.14802/jmd.21138
  • 882 View
  • 120 Download
PDFSupplementary Material
Syringomyelia Manifesting With Pseudodystonia: A Case Report
Yun Su Hwang, Seung Hyun Lee, Sungyang Jo, Sun Ju Chung
J Mov Disord. 2022;15(2):175-177.   Published online November 3, 2021
DOI: https://doi.org/10.14802/jmd.21121
  • 3,490 View
  • 255 Download
PDFSupplementary Material
Original Article
Therapeutic Effect of Levodopa/Carbidopa/Entacapone on Sleep Disturbance in Patients with Parkinson’s Disease
Kye Won Park, Sungyang Jo, Seung Hyun Lee, Yun Su Hwang, Dagyo Lee, Ho-Sung Ryu, Sun Ju Chung
J Mov Disord. 2020;13(3):205-212.   Published online September 9, 2020
DOI: https://doi.org/10.14802/jmd.20055
  • 5,281 View
  • 245 Download
  • 2 Citations
AbstractAbstract PDF
Objective
To investigate the efficacy of levodopa/carbidopa/entacapone (LCE) at bedtime for treating sleep disturbance in patients with Parkinson’s disease (PD) with motor fluctuations.
Methods
Participants included 128 PD patients with motor fluctuations. All patients were assessed for motor, nonmotor, and sleep-specific symptoms using the United Parkinson’s Disease Rating Scale (UPDRS), the Korean version of the Nonmotor Symptom Scale, the Parkinson’s Disease Sleep Scale (PDSS), the Epworth Sleepiness Scale, and the Rapid Eye Movement Sleep Behavior Disorder Screening Questionnaire (RBDSQ). We compared the baseline characteristics of patients with sleep disturbance (PDSS score < 120) and those without sleep disturbance (PDSS score ≥ 120). Thirty-nine patients with sleep disturbance who agreed to take LCE at bedtime completed 3-month follow-ups. We analyzed changes in the scores of motor, nonmotor, and sleep symptom scales over the 3 months.
Results
PD patients with sleep disturbance were at more advanced disease stages and had more severe motor, nonmotor, and sleep symptoms than those without sleep disturbance. Patients who took LCE at night showed improvements in motor (UPDRS part III, p = 0.007) and sleep symptoms (total PDSS, p < 0.001). Sleep features that benefitted from LCE included not only nocturnal motor components but also insomnia (PDSS items 2 and 3, p = 0.005 and p < 0.001) and rapid eye movement behavior disorder (PDSS item 6, p = 0.002; and RBDSQ, p < 0.001).
Conclusion
The use of LCE at bedtime may be a useful treatment for sleep disturbance in advanced PD patients with motor fluctuations.

Citations

Citations to this article as recorded by  
  • The Home-Based Sleep Laboratory
    Yael Hanein, Anat Mirelman, Anat Mirelman, E. Ray Dorsey, Patrik Brundin, Bastiaan R. Bloem
    Journal of Parkinson's Disease.2021; 11(s1): S71.     CrossRef
  • Shudi Pingchan Decoction combined with repetitive transcranial magnetic stimulation in the treatment of Parkinson’s disease with sleep disorders
    Qing Ye, Xiqun Chen, Yuqing Hu, Jie Zhou, Chen Gao, Zhenguo Liu
    Traditional Medicine and Modern Medicine.2020; 03(02): 85.     CrossRef

JMD : Journal of Movement Disorders