Journal of Movement Disorders

Emerging Concepts of Motor Reserve in Parkinson’s Disease: Seok Jong Chung, Jae Jung Lee, Phil Hyu Lee, Young H. Sohn; J Mov Disord. 2020;13(3):171-184. Published online August 31, 2020; DOI: https://doi.org/10.14802/jmd.20029

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Abstract PDF: The concept of cognitive reserve (CR) in Alzheimer’s disease (AD) explains the differences between individuals in their susceptibility to AD-related pathologies. An enhanced CR may lead to less cognitive deficits despite severe pathological lesions. Parkinson’s disease (PD) is also a common neurodegenerative disease and is mainly characterized by motor dysfunction related to striatal dopaminergic depletion. The degree of motor deficits in PD is closely correlated to the degree of dopamine depletion; however, significant individual variations still exist. Therefore, we hypothesized that the presence of motor reserve (MR) in PD explains the individual differences in motor deficits despite similar levels of striatal dopamine depletion. Since 2015, we have performed a series of studies investigating MR in de novo patients with PD using the data of initial clinical presentation and dopamine transporter PET scan. In this review, we summarized the results of these published studies. In particular, some premorbid experiences (i.e., physical activity and education) and modifiable factors (i.e., body mass index and white matter hyperintensity on brain image studies) could modulate an individual’s capacity to tolerate PD pathology, which can be maintained throughout disease progression.

Update on Current Technologies for Deep Brain Stimulation in Parkinson’s Disease: Michelle Paff, Aaron Loh, Can Sarica, Andres M. Lozano, Alfonso Fasano; J Mov Disord. 2020;13(3):185-198. Published online August 31, 2020; DOI: https://doi.org/10.14802/jmd.20052

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Abstract PDF: Deep brain stimulation (DBS) is becoming increasingly central in the treatment of patients with Parkinson’s disease and other movement disorders. Recent developments in DBS lead and implantable pulse generator design provide increased flexibility for programming, potentially improving the therapeutic benefit of stimulation. Directional DBS leads may increase the therapeutic window of stimulation by providing a means of avoiding current spread to structures that might give rise to stimulation-related side effects. Similarly, control of current to individual contacts on a DBS lead allows for shaping of the electric field produced between multiple active contacts. The following review aims to describe the recent developments in DBS system technology and the features of each commercially available DBS system. The advantages of each system are reviewed, and general considerations for choosing the most appropriate system are discussed.

Parkinson Anxiety Scale: A Validation Study for the Brazilian Population: Renilson Moraes-Ferreira, Wilson Mateus Gomes da Costa Alves, Maysa Alves Rodrigues Brandao-Rangel, Odilon Abrahin, Clebson Pantoja Pimentel, Evitom Correa-Sousa, Rodolfo Paula Vieira, Erik Artur Cortinhas-Alves; J Mov Disord. 2020;13(3):199-204. Published online July 28, 2020; DOI: https://doi.org/10.14802/jmd.20031

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Abstract PDF: Objective
The Parkinson Anxiety Scale (PAS) was developed to measure the severity of anxiety symptoms in patients with Parkinson’s disease (PD), and it has not yet been adapted and validated in Portuguese. Thus, this study evaluated the reliability and validity of a translated and adapted version of the PAS for the Brazilian population of PD patients.
Methods
The Parkinson Anxiety Scale – Brazilian Version (PAS-BV) was completed by 55 patients with PD. The reliability (test-retest reliability, interrater reliability and internal consistency) and construct validity of the PAS-BV were assessed by comparing it with the Beck Anxiety Inventory (BAI), the Parkinson’s Disease Fatigue Scale (PFS) and the Unified Parkinson Disease Rating Scale (UPDRS) part III.
Results
Patients with PD had an average age of 64.51 ± 9.20 years and had PD for an average of 6.98 ± 5.02 years. The reliability of the PAS-BV was 0.83, and the intraclass correlation coefficient (ICC) (retest-test) was 0.88. The scale presented good convergent validity with the BAI (r_s = 0.82, p < 0.05). It also presented good divergent validity with the PFS (r_s = 0.24, p > 0.05) and the UPDRS part II (r_s = -0.10, p > 0.05), part III (r_s = -0.21, p > 0.05), and part IV (r_s = 0.03, p > 0.05), as indicated by the absence of significant correlations. However, there was a significant correlation between the PAS-BV and part I of the UPDRS (r_s = 0.67, p < 0.05).
Conclusion
The PAS-BV presents substantial reliability and validity for patients with PD without dementia.

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

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Abstract 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.

Effectiveness of Exercise on the Sequence Effect in Parkinson’s Disease: Suk Yun Kang, Young Ho Sohn; J Mov Disord. 2020;13(3):213-217. Published online August 31, 2020; DOI: https://doi.org/10.14802/jmd.20045

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Abstract PDF Supplementary Material: Objective
To determine the benefits of motor training on the sequence effect (SE), an essential component of bradykinesia in Parkinson’s disease (PD).
Methods
Seven patients with de novo PD participated in this study. The patients performed regular pentagon drawing tests and exercises during four visits. The first two visits occurred before the start of medication, and the last two visits occurred at least six months after the start of medication. We assessed the severity of bradykinesia and SE at each visit and compared the results before and after exercise in both the de novo and treatment conditions.
Results
In the de novo condition, the severity of bradykinesia significantly improved after motor training (p = 0.018), but it did not resolve and only showed a trend of improvement after treatment (p = 0.068). The severity of the SE decreased significantly in the drug-naïve condition (p = 0.028) but not after medication (p = 0.273).
Conclusion
Our study suggests that regular motor training may be beneficial for the SE in PD.

Deep Brain Stimulation Battery Exhaustion during the COVID-19 Pandemic: Crisis within a Crisis: Vikram Venkappayya Holla, Koti Neeraja, Bharath Kumar Surisetti, Shweta Prasad, Nitish Kamble, Dwarakanath Srinivas, Ravi Yadav, Pramod Kumar Pal; J Mov Disord. 2020;13(3):218-222. Published online August 31, 2020; DOI: https://doi.org/10.14802/jmd.20073

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Abstract PDF: Objective
The novel coronavirus disease (COVID-19) pandemic and public health measures to control it have resulted in unique challenges in the management of patients with deep brain stimulation (DBS). We report our experience with the management of acute worsening of symptoms due to battery exhaustion in 3 patients with DBS.
Methods
Patients with DBS for movement disorders who visited the emergency room due to battery exhaustion during the nationwide lockdown from April to May 2020 were included.
Results
Two patients with subthalamic nucleus-DBS for Parkinson’s disease (PD) and one with globus pallidus interna-DBS for generalized dystonia presented with acute worsening of symptoms due to battery exhaustion. Urgent battery replacement was performed in both patients with PD. The patient with generalized dystonia was managed with medication adjustment as he chose to defer battery replacement.
Conclusion
DBS battery replacement can be an emergency. Decisions regarding DBS battery replacement should be individualized during this COVID-19 pandemic.

Focal Unilateral Palatal Myoclonus Causing Objective Clicking Tinnitus without Uvula Elevation Diagnosed by Concurrent Auscultation: Chindhuri Selvadurai, Sara Maguire Schaefer; J Mov Disord. 2020;13(3):223-224. Published online July 14, 2020; DOI: https://doi.org/10.14802/jmd.20010

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Abstract PDF Supplementary Material: Palatal myoclonus generally entails a visible elevation of the palate and uvula and may be accompanied by myoclonus of other oropharyngeal muscles. A 55-year-old man presented with left ear clicking and hyperacusis. Examination showed arrhythmic left lateral soft palate contraction in the tensor veli palatini region without elevation of the uvula, which correlated with an audible click by auscultation with a stethoscope over the left ear. This is a rare case of focal, unilateral palatal myoclonus without visual uvula elevation with concurrent auscultation, demonstrating the importance of careful examination to detect focal myoclonic contractions.

Young-Onset Parkinson’s Disease with Impulse Control Disorder Due to Novel Variants of F-Box Only Protein 7: Dallah Yoo, Ji-Hyun Choi, Jin-Hee Im, Man Jin Kim, Han-Joon Kim, Sung Sup Park, Beomseok Jeon; J Mov Disord. 2020;13(3):225-228. Published online September 9, 2020; DOI: https://doi.org/10.14802/jmd.20026

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Abstract PDF: F-box only protein 7 (FBXO7) is a rare monogenic cause of hereditary Parkinson’s disease (PD) with an autosomal recessive mode of inheritance and a broad spectrum of clinical manifestations. Here, we report a de novo PD patient with onset at the age of 28 with novel compound heterozygous variants in the FBXO7 gene (c.1162C>T, p.Gln388X; c.80G>A, p.Arg27His). The clinical features of the patient were problematic impulse control disorder behaviors and pyromania, and pyramidal signs were negative. We describe the novel pathogenic variants of the FBXO7 gene with detailed clinical pictures to report the expanding genotypes and phenotypes of FBXO7-associated parkinsonism.

A New Allelic Variant in the PANK2 Gene in a Patient with Incomplete HARP Syndrome: Myriam Ley Martos, María Jesús Salado Reyes, Rosario Marín Iglesias, Carmen Gutiérrez Moro, Manuel Lubián Gutiérrez, Lorena Estepa Pedregosa; J Mov Disord. 2020;13(3):229-231. Published online July 14, 2020; DOI: https://doi.org/10.14802/jmd.19071

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Asymmetric Periodic Leg Movements during Sleep after Unilateral Supratentorial Infarction: Two Legs with One Lesion: Sang-Won Yoo, Ko Eun Choi, Joong-Seok Kim; J Mov Disord. 2020;13(3):232-234. Published online June 3, 2020; DOI: https://doi.org/10.14802/jmd.20004

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Anti-GABA_B Receptor Encephalitis Presenting with Atypical Corticobasal Syndrome in a Patient with Parkinson’s Disease: Noor Sharizat Abdullah, Tan Hui Jan, Rabani Remli, Shahizon Azura Mohamad Mukari, Norlinah Mohamed Ibrahim; J Mov Disord. 2020;13(3):235-237. Published online September 9, 2020; DOI: https://doi.org/10.14802/jmd.20011

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Recurrent ADCY5 Mutation in Mosaic Form with Nocturnal Paroxysmal Dyskinesias and Video Electroencephalography Documentation of Dramatic Response to Caffeine Treatment: Kuldeep Shetty, Asodu Sandeep Sarma, Meera Devan, Ashwin Dalal, Gopal Krishna Dash, Apuroopa Jannabhatla, Siddaramappa Jagadish Patil; J Mov Disord. 2020;13(3):238-240. Published online July 28, 2020; DOI: https://doi.org/10.14802/jmd.20014

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Bupropion-Induced Dystonia in a Patient with Parkinson’s Disease: Monalisa Vegda, Samhita Panda; J Mov Disord. 2020;13(3):241-243. Published online August 31, 2020; DOI: https://doi.org/10.14802/jmd.20046

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Spinocerebellar Ataxia 13 Presenting with Pure Cerebellar Syndrome in a Korean Family: Minkyeong Kim, Seung Hwan Oh, Jae Wook Cho, Jae-Hyeok Lee; J Mov Disord. 2020;13(3):244-246. Published online September 9, 2020; DOI: https://doi.org/10.14802/jmd.20064

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