Journal of Movement Disorders

Diagnosing Cerebrotendinous Xanthomatosis in a Middle-Aged Woman with Cervical Dystonia: Wei-Sheng Wang, Yu-Ping Chiu, Meng-Han Tsai, Shey-Lin Wu, Yen-Chung Chen; Received September 28, 2024 Accepted January 17, 2025 Published online January 20, 2025; DOI: https://doi.org/10.14802/jmd.24202 [Accepted]

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Levodopa Pharmacokinetics in Switching from Levodopa/Carbidopa Intestinal Gel to Continuous Subcutaneous Foslevodopa/Foscarbidopa Infusion in a Patient with Parkinson's Disease: a case report: Tomonori Nukariya, Toshiki Tezuka, Shohei Okusa, Ryotaro Okochi, Yuto Sakai, Yoshihiro Nihei, Jin Nakahara, Morinobu Seki; Received November 28, 2024 Accepted January 6, 2025 Published online January 6, 2025; DOI: https://doi.org/10.14802/jmd.24247 [Accepted]

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Pre-DBS modified Tremor/Postural instability and gait difficulty score ratio as indicator of short-term outcome of subthalamic nucleus DBS in Parkinson’s disease: Chakradhar Reddy, Kanchana Pillai, Shejoy Joshua, Anup Nair, Harshad Chavotiya, Manas Chacko, Asha Kishore; Received August 5, 2024 Accepted January 2, 2025 Published online January 2, 2025; DOI: https://doi.org/10.14802/jmd.24175 [Accepted]

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Abstract PDF: Purpose
-The outcomes of motor and non-motor features of Parkinson’s disease (PD) following DBS vary among its subtypes. We tested whether pre-operative motor subtyping using the modified Tremor/PIGD ratio, could indicate the short-term motor, non-motor and quality of life (QOL) outcomes of STN-DBS.
Method
In this prospective study, 39 consecutive STN-DBS cases were assessed in Drug-OFF state before surgery and subtyped using the ratio of tremor and PIGD scores (T/P ratio). 6 months after surgery patients were reassessed in Stimulation ON-Drug OFF state and the percentage change in motor, non-motor and QOL scores (PDQ39) was calculated.
Results
The modified T/P ratio had a moderate, positive correlation with the percentage change in scores of UPDRS III in OFF, sum of cardinal motor signs, non-motor symptoms scale (NMSS) and quality of life (PDQ39).
Conclusion
Preoperative PD motor subtyping can be used as an indicator of the short-term, outcomes of STN-DBS in PD.

Validation of the Korean Version of the Huntington’s Disease Quality of Life Battery for Carers: Hee Jin Chang, Eungseok Oh, Won Tae Yoon, Chan Young Lee, Kyum-Yil Kwon, Yun Su Hwang, Chaewon Shin, Jee-Young Lee; Received October 21, 2024 Accepted December 20, 2024 Published online December 30, 2024; DOI: https://doi.org/10.14802/jmd.24217 [Accepted]

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Abstract PDF: Objectives
The Huntington's Disease Quality of Life Battery for Carers (HDQoL-C) evaluates caregiver quality of life. This study aims to develop and validate the Korean version (K-HDQoL-C) to assess the burden on Korean caregivers of HD patients.
Methods
Nineteen HD caregivers (7 females, mean age 55.4±14.6 years) participated. The K-HDQoL-C, a translation of the English version, consisted of demographic information, caring aspects, life satisfaction, and feelings about life. It was administered twice, two weeks apart. Internal consistency was evaluated using Cronbach’s α, and test-retest reliability was assessed with intraclass correlation coefficients. The relationship with the Zarit Burden Interview-12 (ZBI-12) was analyzed.
Results
Internal consistencies of K-HDQoL-C were 0.771 (part 2), 0.938 (part 3), and 0.891 (part 4). Test-retest reliability ranged from 0.908 to 0.936. Part 3 negatively correlated with ZBI-12, and part 4 positively correlated (R = -0.780, 0.923; p < 0.001).
Conclusion
The K-HDQoL-C effectively evaluates challenges faced by HD caregivers, particularly in care aspects and life satisfaction.

Gait instability and compensatory mechanisms in Parkinson's disease with camptocormia: An exploratory study: Hideyuki Urakami, Yasutaka Nikaido, Yuta Okuda, Yutaka Kikuchi, Ryuichi Saura, Yohei Okada; Received November 8, 2024 Accepted December 27, 2024 Published online December 27, 2024; DOI: https://doi.org/10.14802/jmd.24226 [Accepted]

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Abstract PDF: Objective
Camptocormia has been considered to contribute to vertical gait instability and, at times, may also lead to forward instability in experimental settings in Parkinson's disease (PD). However, these aspects, along with compensatory mechanisms, remain largely unexplored. This study comprehensively investigated gait instability and compensatory strategies in PD patients with camptocormia (PD+CC).
Methods
Ten PD+CC, 30 without camptocormia (PD-CC), and 27 healthy controls (HCs) participated. Self-paced gait tasks were analyzed using three-dimensional motion capture systems to assess gait stability, spatiotemporal, and kinematic parameters. Unique cases with pronounced forward gait stability or instability were first identified, followed by group comparisons. Correlation analysis was performed to examine associations between trunk flexion angles (lower/upper) and gait parameters. Significance level was set at 0.05.
Results
Excluding one unique case, the PD+CC group showed a significantly lower vertical center of mass (COM) position (p=0.019), along with increased mediolateral COM velocity (p=0.004) and step width (p=0.013), compared to PD-CC group. Both PD groups showed higher anterior-posterior margin of stability than HCs (p<0.001). Significant correlations were found between lower/upper trunk flexion angles and a lower vertical COM position (r=-0.690/-0.332), as well as increased mediolateral COM velocity (r=0.374/0.446) and step width (r=0.580/0.474).
Conclusions
Most PD+CC patients exhibited vertical gait instability, increasing fall risk, and adopted compensatory strategies involving greater lateral COM shift and wider base of support, with these trends intensifying as trunk flexion angles increased. These findings may guide targeted interventions for gait instability in PD+CC.

Two cases of genetically proven SCARB2-Related Progressive Myoclonic Epilepsy without renal failure: A report from India: Pavankumar Katragadda, Vikram V Holla, Gautham Arunachal, Nitish Kamble, Ravi Yadav, Pramod Kumar Pal; Received October 27, 2024 Accepted December 17, 2024 Published online December 27, 2024; DOI: https://doi.org/10.14802/jmd.24222 [Accepted]

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A practical guide for diagnostic investigations and special considerations in patients with Huntington’s disease in Korea: Jangsup Moon, Eungseok Oh, Minkyeong Kim, Ryul Kim, Dallah Yoo, Chaewon Shin, Jee-Young Lee, Jong-Min Kim, Seong-Beom Koh, Manho Kim, Beomseok Jeon; Received November 15, 2024 Accepted December 24, 2024 Published online December 26, 2024; DOI: https://doi.org/10.14802/jmd.24232 [Accepted]

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Abstract PDF: The Korean Huntington’s Disease Society (KHDS) has recently published a practical guide for clinical approach to patients with Huntington’s disease (HD) in Korea in April issue of Journal of Movement Disorders this year.¹ This article is the second practical guide particularly focused on 1) essential points of genetic counseling for families of HD covering issues of testing minors and prenatal/preimplantation testing; and 2) premanifest HD and useful laboratory investigations for assessing disease severity and progression. The latter part of this article deals with special issues of juvenile and very late-onset HD, and common comorbidities in HD patients. 3) Finally, the management principles of HD patients are briefly explained. The contents were drafted by the guideline task force members of the KHDS. We intended to include published literature containing South Korean data in this paper. We believe this article will be a pocket guide to physicians as well as movement disorders specialists in Korea in planning diagnostic work-up for and management of HD patients and families.

Spatiotemporal Gait Parameters During Turning and Imbalance in Parkinson's Disease: Video-Based Analysis from a Single Camera: HoYoung Jeon, Jung Hwan Shin, Ri Yu, Min Kyung Kang, Seungmin Lee, Seoyeon Kim, Bora Jin, Kyung Ah Woo, Han-Joon Kim, Beomseok Jeon; Received October 13, 2024 Accepted December 23, 2024 Published online December 23, 2024; DOI: https://doi.org/10.14802/jmd.24210 [Accepted]

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Abstract PDF: Objectives
This study aims to objectively evaluate turning gait parameters in Parkinson disease (PD) patients using 2D-RGB video-based analysis and explore their relationships with imbalance.
Methods
We prospectively enrolled PD patients for clinical assessments, balance analysis and gait with 180’ turning. Spatiotemporal gait parameters during turning were derived based on video-based analysis and correlated with modified Hoehn and Yahr (mHY) stages and center of pressure (COP) oscillations.
Results
A total of 64 PD patients were enrolled. The PD patients with higher mHY stages (≥2.5) had significantly longer turning times, higher number of steps, wider step bases and showed less variability in step length during turns. COP oscillations were positively correlated with mean turning time in the both anterio-posterior (AP) and right-left (RL) axis.
Conclusions
Spatiotemporal gait parameters during turning, derived from video-based gait analysis, may be a promising biomarker for monitoring postural instability in PD patients.

Non-motor fluctuations in Parkinson’s disease: underdiagnosed, yet important: Iro Boura, Karolina Poplawska-Domaszewicz, Cleanthe Spanaki, Rosabel Chen, Daniele Urso, Riaan van Coller, Alexander Storch, Kallol Ray Chaudhuri; Received November 13, 2024 Accepted December 20, 2024 Published online December 20, 2024; DOI: https://doi.org/10.14802/jmd.24227 [Accepted]

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Abstract PDF: Non-motor fluctuations (NMF) in Parkinson's disease (PD) significantly affect patients’ well-being. Despite being identified over two decades ago, NMF remain largely under-recognized, under-treated, and poorly understood. While they are often temporally associated with motor fluctuations (MF) and can share common risk factors and pathophysiologic mechanisms, NMF and MF are currently considered distinct entities. The prevalence and severity of NMF, often categorized into neuropsychiatric, sensory, and autonomic subtypes, vary significantly across studies due to the heterogeneous PD populations screened and the diverse evaluation tools applied. The consistent negative impact of NMF on PD patients’ quality of life (QoL) underscores the importance of further investigation via focused and controlled studies, validated assessment instruments and novel digital technologies. High-quality research is essential to illuminate the complex pathophysiology and clinical nuances of NMF, ultimately enhancing clinicians’ diagnostic and treatment options in routine clinical practice.

Muscle Dystonia Manifesting as Unilateral Rectus Abdominis Hypertrophy and Abdominal Pain in Parkinson’s Disease: A Case Report: Chengjian Wu, Yanhui Meng, Ming Yang; Received November 14, 2024 Accepted December 10, 2024 Published online December 10, 2024; DOI: https://doi.org/10.14802/jmd.24230 [Epub ahead of print]

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Complete Relief of Tardive Truncal Dystonia With a Low Dose of Levodopa/Carbidopa: A Case Report: Shinichi Matsumoto, Satoshi Goto; Received November 15, 2024 Accepted December 8, 2024 Published online December 9, 2024; DOI: https://doi.org/10.14802/jmd.24231 [Epub ahead of print]

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Barriers to Accessing Deep Brain Stimulation for Parkinson’s Disease Patients in Morocco: Mohamed DAGHI, Abdelhakim LAKHDAR, Hicham EL OTMANI; Received November 3, 2024 Accepted December 8, 2024 Published online December 9, 2024; DOI: https://doi.org/10.14802/jmd.24225 [Accepted]

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Video-oculography for enhancing the diagnostic accuracy of early oculomotor dysfunction in Progressive Supranuclear Palsy: Harshad Chovatiya, Kanchana Pillai, Chakradhar Reddy, Amiya Thalakkattu, Ayana Avarachan, Manas Chacko, Asha Kishore; Received August 1, 2024 Accepted December 8, 2024 Published online December 9, 2024; DOI: https://doi.org/10.14802/jmd.24171 [Accepted]

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Abstract PDF: Background
Oculomotor impairment is an important diagnostic feature of Progressive Supranuclear Palsy (PSP) and PSP subtypes.
Objectives
We assessed the role of video oculography (VOG) in confirming clinically suspected slow saccades in PSP and differentiating PSP from Parkinson’s disease (PD). We also measured the correlation of both saccadic velocity and latency in PSP with scores in PSP rating scale, Montreal Cognitive Assessment (MoCA) and Frontal assessment battery (FAB). We assessed the frequency of apraxia of eyelid opening (ALO) and reflex blepharospasm in PSP and PD.
Method
112 PSP cases with slow saccades but not gaze palsy, 50 PD and 50 healthy controls (HC) were recruited. MDS task force-PSP and PD criteria were used respectively, for the diagnoses. All subjects underwent VOG.
Result
Horizontal and vertical saccadic velocities and latencies differentiated PSP from PD and HC (p<0.001). Vertical saccadic velocity and latency accurately differentiated PSP-P from PD (p<0.001 and 0.003 respectively). Vertical and horizontal saccadic velocities differentiated PSP- RS and PSP- P (p=0.026 and 0.036 respectively). In vertical gaze, the mean velocity cut-off showed good sensitivity and specificity in differentiating PSP from HC and PD. Prolonged horizontal gaze latency was associated with more severe PSP and worse global cognitive and frontal dysfunction. ALO and reflex blepharospasm were only seen in PSP.
Conclusion
VOG is useful for confirming slow saccades in PSP-RS and PSP-P and in differentiating PSP-P from PD. Prolonged horizontal gaze latency was associated with more severe PSP and worse cognitive dysfunction. ALO and reflex blepharospasm were seen only in PSP.

The eye movement and gait variability analysis in Chinese patients with Huntington's disease: Shu-Xia Qian, Yu-Feng Bao, Xiao-Yan Li, Yi Dong, Zhi-Ying Wu; Received June 30, 2024 Accepted December 6, 2024 Published online December 9, 2024; DOI: https://doi.org/10.14802/jmd.24151 [Accepted]

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Abstract PDF: BACKGROUND
Huntington's disease disease (HD) is characterized by motor, cognitive, and neuropsychiatric symptoms. Oculomotor impairments and gait variability have been independently considered as potential markers in HD. But there lacks an integration analysis of eye movement and gait.
OBJECTIVE
We assessed multiple examinations of eye movement and gait variability in HTT mutation carriers, analyzed the consistency between these parameters and clinical severity, then examined the association between oculomotor impairments and gait deficits.
METHODS
Seven pre-HD, 30 HD patients and 30 age-matched controls were included. We collected demographics and assessed the Unified Huntington's Disease Rating Scale (UHDRS). Examinations including saccade, smooth pursuit and optokinetic (OPK) test were performed to evaluate eye movement function. The parameters of gait include stride length, walking velocity, step deviation, step length and gait phase.
RESULTS
There are significant impairments in HD patients in the latency and velocity of saccades, the gain of smooth pursuit as well as the gain and slow phase velocities (SPV) of OPK tests. Only the speed of saccades has significant difference between pre-HD and controls. There are significant impairments of stride length, walking velocity, step length and gait phase in HD patients. Parameters of eye movement and gait variability in HD patients showed consistency with the scores of UHDRS. There were significant correlations between eye movement and gait parameters.
CONCLUSIONS
Our results show that eye movement and gait are impaired in HD patients, and speed of saccades is early impaired in pre-HD. Eye movement and gait abnormalities in HD are significantly correlated with clinical disease severities.

The Application of Deep Brain Stimulation in the Syndrome of Irreversible Lithium-Effectuated Neurotoxicity: Lina Okar, Peyton Murin, Anagha Prabhune, Richard Bucholz, Pratap Chand; Received October 10, 2024 Accepted November 30, 2024 Published online December 2, 2024; DOI: https://doi.org/10.14802/jmd.24209 [Epub ahead of print]

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