Journal of Movement Disorders

Original Article

Impact of Additional Occipital Involvement in Parkinson’s Disease With Posterior Cortical Hypoperfusion: Chan Wook Park, Su Hong Kim, Phil Hyu Lee, Yun Joong Kim, Young H. Sohn, Yong Jeong, Seok Jong Chung; J Mov Disord. 2026;19(1):58-66. Published online November 7, 2025; DOI: https://doi.org/10.14802/jmd.25231

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Abstract PDF Supplementary Material: Objective
This study aims to investigate the clinical relevance of occipital hypoperfusion in patients with Parkinson’s disease (PD) with respect to clinical phenotype and the risk of dementia conversion.
Methods
We enrolled 349 patients with newly diagnosed PD and 48 healthy controls who underwent dual-phase ¹⁸F-N-(3-fluoropropyl)-2β-carboxymethoxy-3β-(4-iodophenyl) nortropane (¹⁸F-FP-CIT) positron emission tomography (PET). Patients with PD were classified into three groups based on posterior cortical perfusion patterns on early-phase ¹⁸F-FP-CIT PET images: PD with preserved posterior cortical perfusion (n=186), PD with parieto-temporal hypoperfusion (n=84), and PD with parieto-temporo-occipital hypoperfusion (n=79). Baseline clinical features and dementia conversion risk were compared across PD groups.
Results
Patients with preserved posterior cortical perfusion were younger than those in the other PD groups. Compared with the other groups, the parieto-temporo-occipital hypoperfusion group tended to have lower Cross-Cultural Smell Identification Test scores, a higher prevalence of rapid eye movement sleep behavior disorder, higher Unified PD Rating Scale motor scores, and more severe reductions in striatal dopamine transporter availability. The risk of dementia conversion was lower in patients with preserved posterior cortical perfusion than in those with posterior cortical hypoperfusion. However, the risk of dementia conversion did not differ between the parieto-temporal and parieto-temporo-occipital hypoperfusion groups.
Conclusion
Additional occipital hypoperfusion was not associated with an imminent risk of dementia conversion in patients with PD with posterior cortical hypoperfusion. Nonetheless, occipital involvement may serve as an indicator of the diffuse malignant subtype of PD.

Review Article

Drug Repositioning and Repurposing for Disease-Modifying Effects in Parkinson’s Disease: Seong Ho Jeong, Phil Hyu Lee; J Mov Disord. 2025;18(2):113-126. Published online February 7, 2025; DOI: https://doi.org/10.14802/jmd.25008

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Parkinson’s disease (PD) is the second most prevalent neurodegenerative disorder and is characterized by progressive dopaminergic and nondopaminergic neuronal loss and the presence of Lewy bodies, which are primarily composed of aggregated α-synuclein. Despite advancements in symptomatic therapies, such as dopamine replacement and deep brain stimulation, no disease-modifying therapies (DMTs) have been identified to slow or arrest neurodegeneration in patients with PD. Challenges in DMT development include disease heterogeneity, the absence of reliable biomarkers, and the multifaceted pathophysiology of PD, encompassing neuroinflammation, mitochondrial dysfunction, lysosomal impairment, and oxidative stress. Drug repositioning and repurposing strategies using existing drugs for new therapeutic applications offer promising approaches to accelerate the development of DMTs for PD. These strategies minimize time, cost, and risk by using compounds with established safety profiles. Prominent candidates include glucagon-like peptide-1 receptor agonists, dipeptidyl peptidase-4 inhibitors, ambroxol, calcium channel blockers, statins, iron-chelating agents, c-Abl inhibitors, and memantine. Although preclinical and early clinical studies have demonstrated encouraging results, numerous phase III trials have yielded unfavorable outcomes, elucidating the complexity of PD pathophysiology and the need for innovative trial designs. This review evaluates the potential of prioritized repurposed drugs for PD, focusing on their mechanisms, preclinical evidence, and clinical trial outcomes, and highlights the ongoing challenges and opportunities in this field.

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Teneligliptin, a DPP4 inhibitor protects dopaminergic neurons in PD models via inhibiting of oxidative stress and ferroptosis
Linting Huang, Jiakai Pi, Liqin Gu, Zirou Liao, Wenya Wang
European Journal of Pharmacology.2025; 1002: 177782. CrossRef
Deciphering the Janus‐Faced Nature of the Apolipoprotein Superfamily in Parkinsonian Neurodegeneration: Molecular Crosstalk Between the Astroglial Secretome and Neuronal Homeostasis
Yingying Dai, Mingxia Bi, Qian Jiao, Xixun Du, Xi Chen, Chunling Yan, Hong Jiang
Movement Disorders.2025; 40(11): 2299. CrossRef
Glucagon-Like Peptide-1 Receptor Agonists Are Promising for the Treatment of Brain Diseases: an Outlook from the Perspective of Integrative Physiology
N. V. Gulyaeva
Journal of Evolutionary Biochemistry and Physiology.2025; 61(5): 1326. CrossRef
Therapeutic innovation through drug repurposing: A multidimensional approach toward treating Parkinson's disease
Saswati Swagatika Sahoo, Sudhir Kumar Paidesetty, Pratap Kumar Sahu, Swagata Pattanaik, Rambabu Dandela
Bioorganic Chemistry.2025; 166: 109129. CrossRef

Brief communication

Evaluating the Validity and Reliability of the Korean Version of the Scales for Outcomes in Parkinson’s Disease–Cognition: Jinse Park, Eungseok Oh, Seong-Beom Koh, In-Uk Song, Tae-Beom Ahn, Sang Jin Kim, Sang-Myung Cheon, Yoon-Joong Kim, Jin Whan Cho, Hyeo-Il Ma, Mee Young Park, Jong Sam Baik, Phil Hyu Lee, Sun Ju Chung, Jong-Min Kim, Han-Joon Kim, Young-Hee Sung, Do Young Kwon, Jae-Hyeok Lee, Jee-Young Lee, Ji Seon Kim, Ji Young Yun, Hee Jin Kim, Jin Yong Hong, Mi-Jung Kim, Jinyoung Youn, Hui-Jun Yang, Won Tae Yoon, Sooyeoun You, Kyum-Yil Kwon, Su-Yun Lee, Younsoo Kim, Hee-Tae Kim, Joong-Seok Kim, Ji-Young Kim; J Mov Disord. 2024;17(3):328-332. Published online April 3, 2024; DOI: https://doi.org/10.14802/jmd.24061

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Abstract PDF Supplementary Material

Objective
The Scales for Outcomes in Parkinson’s Disease–Cognition (SCOPA-Cog) was developed to assess cognition in patients with Parkinson’s disease (PD). In this study, we aimed to evaluate the validity and reliability of the Korean version of the SCOPACog (K-SCOPA-Cog).
Methods
We enrolled 129 PD patients with movement disorders from 31 clinics in South Korea. The original version of the SCOPA-Cog was translated into Korean using the translation-retranslation method. The test–retest method with an intraclass correlation coefficient (ICC) and Cronbach’s alpha coefficient were used to assess reliability. Spearman’s rank correlation analysis with the Montreal Cognitive Assessment-Korean version (MOCA-K) and the Korean Mini-Mental State Examination (K-MMSE) were used to assess concurrent validity.
Results
The Cronbach’s alpha coefficient was 0.797, and the ICC was 0.887. Spearman’s rank correlation analysis revealed a significant correlation with the K-MMSE and MOCA-K scores (r = 0.546 and r = 0.683, respectively).
Conclusion
Our results demonstrate that the K-SCOPA-Cog has good reliability and validity.

Citations

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Validation of the Korean Moss Attention Rating Scale in Traumatic Brain Injury: Reliability and Validity
Sung Hoon Jeong, Jun Hee Won, Byung-Mo Oh, Junghoon J. Kim, Ja-Ho Leigh, Gain Shin, Sodam Lee, Hoo Young Lee
Korean Journal of Neurotrauma.2026; 22(1): 24. CrossRef
Clinical Outcome Assessments in Parkinson's Disease: A Scoping Review of Current Rating Scales and Future Needs
Evita Papathoma, Panagiota Tsitsi, Nirosen Vijiaratnam, Camila Aquino, Stephen R. Duma, Norbert Kovacs, Kigocha Lameck Okeng'o, Aparna Wagle Shukla, Roongroj Bhidayasiri, Tiago A. Mestre, Alvaro Sanchez Ferro, Alberto J. Espay, Michelle H.S. Tosin, Matej
Movement Disorders Clinical Practice.2025;[Epub] CrossRef

Original Article

Comparing Montreal Cognitive Assessment Performance in Parkinson’s Disease Patients: Age- and Education-Adjusted Cutoffs vs. Machine Learning: Kyeongmin Baek, Young Min Kim, Han Kyu Na, Junki Lee, Dong Ho Shin, Seok-Jae Heo, Seok Jong Chung, Kiyong Kim, Phil Hyu Lee, Young H. Sohn, Jeehee Yoon, Yun Joong Kim; J Mov Disord. 2024;17(2):171-180. Published online February 13, 2024; DOI: https://doi.org/10.14802/jmd.23271

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Abstract PDF Supplementary Material

Objective
The Montreal Cognitive Assessment (MoCA) is recommended for general cognitive evaluation in Parkinson’s disease (PD) patients. However, age- and education-adjusted cutoffs specifically for PD have not been developed or systematically validated across PD cohorts with diverse education levels.
Methods
In this retrospective analysis, we utilized data from 1,293 Korean patients with PD whose cognitive diagnoses were determined through comprehensive neuropsychological assessments. Age- and education-adjusted cutoffs were formulated based on 1,202 patients with PD. To identify the optimal machine learning model, clinical parameters and MoCA domain scores from 416 patients with PD were used. Comparative analyses between machine learning methods and different cutoff criteria were conducted on an additional 91 consecutive patients with PD.
Results
The cutoffs for cognitive impairment decrease with increasing age within the same education level. Similarly, lower education levels within the same age group correspond to lower cutoffs. For individuals aged 60–80 years, cutoffs were set as follows: 25 or 24 years for those with more than 12 years of education, 23 or 22 years for 10–12 years, and 21 or 20 years for 7–9 years. Comparisons between age- and education-adjusted cutoffs and the machine learning method showed comparable accuracies. The cutoff method resulted in a higher sensitivity (0.8627), whereas machine learning yielded higher specificity (0.8250).
Conclusion
Both the age- and education-adjusted cutoff methods and machine learning methods demonstrated high effectiveness in detecting cognitive impairment in PD patients. This study highlights the necessity of tailored cutoffs and suggests the potential of machine learning to improve cognitive assessment in PD patients.

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Does cognition affect supervised and unsupervised mobility differently in people with Parkinson’s disease? A cross-sectional study
Edoardo Bianchini, Francesco Garramone, Domiziana Rinaldi, Marika Alborghetti, Lanfranco De Carolis, Silvia Galli, Antonio Suppa, Marco Salvetti, Clint Hansen, Nicolas Vuillerme
Journal of NeuroEngineering and Rehabilitation.2026;[Epub] CrossRef
A spatio-temporal fusion-based approach for multi-dimensional classification of Parkinson’s disease progression using multi-modal dataset
Vinay Kukreja, Vandana Ahuja, Modafar Ati, Hariharan Shanmugasundaram, Murugaperumal Krishnamoorthy, Rishabh Sharma, Abhishek Bhattacherjee
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Letter to the editor

Factors associated with anticholinergic-induced oral-buccal-lingual dyskinesia in Parkinson’s disease: Joonyoung Ha, Suk Yun Kang, Kyoungwon Baik, Young H. Sohn, Phil Hyu Lee, Min Seok Baek, Jin Yong Hong; J Mov Disord. 2024;17(1):109-111. Published online September 22, 2023; DOI: https://doi.org/10.14802/jmd.23069

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Review Article

Subjective Cognitive Complaints in Cognitively Normal Patients With Parkinson’s Disease: A Systematic Review: Jin Yong Hong, Phil Hyu Lee; J Mov Disord. 2023;16(1):1-12. Published online November 10, 2022; DOI: https://doi.org/10.14802/jmd.22059

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Abstract PDF

Subjective cognitive complaints (SCCs) refer to self-perceived cognitive decline and are related to objective cognitive decline. SCCs in cognitively normal individuals are considered a preclinical sign of subsequent cognitive impairment due to Alzheimer’s disease, and SCCs in cognitively normal patients with Parkinson’s disease (PD) are also gaining attention. The aim of this review was to provide an overview of the current research on SCCs in cognitively normal patients with PD. A systematic search found a lack of consistency in the methodologies used to define and measure SCCs. Although the association between SCCs and objective cognitive performance in cognitively normal patients with PD is controversial, SCCs appear to be predictive of subsequent cognitive decline. These findings support the clinical value of SCCs in cognitively normal status in PD; however, further convincing evidence from biomarker studies is needed to provide a pathophysiological basis for these findings. Additionally, a consensus on the definition and assessment of SCCs is needed for further investigations.

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Original Article

Potential Link Between Cognition and Motor Reserve in Patients With Parkinson’s Disease: Seok Jong Chung, Yae Ji Kim, Yun Joong Kim, Hye Sun Lee, Mijin Yun, Phil Hyu Lee, Yong Jeong, Young H. Sohn; J Mov Disord. 2022;15(3):249-257. Published online September 7, 2022; DOI: https://doi.org/10.14802/jmd.22063

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Abstract PDF Supplementary Material

Objective
To investigate whether there is a link between cognitive function and motor reserve (i.e., individual capacity to cope with nigrostriatal dopamine depletion) in patients with newly diagnosed Parkinson’s disease (PD).
Methods
A total of 163 patients with drug-naïve PD who underwent ¹⁸F-FP-CIT PET, brain MRI, and a detailed neuropsychological test were enrolled. We estimated individual motor reserve based on initial motor deficits and striatal dopamine depletion using a residual model. We performed correlation analyses between motor reserve estimates and cognitive composite scores. Diffusion connectometry analysis was performed to map the white matter fiber tracts, of which fractional anisotropy (FA) values were well correlated with motor reserve estimates. Additionally, Cox regression analysis was used to assess the effect of initial motor reserve on the risk of dementia conversion.
Results
The motor reserve estimate was positively correlated with the composite score of the verbal memory function domain (γ = 0.246) and with the years of education (γ = 0.251). Connectometry analysis showed that FA values in the left fornix were positively correlated with the motor reserve estimate, while no fiber tracts were negatively correlated with the motor reserve estimate. Cox regression analysis demonstrated that higher motor reserve estimates tended to be associated with a lower risk of dementia conversion (hazard ratio, 0.781; 95% confidence interval, 0.576–1.058).
Conclusion
The present study demonstrated that the motor reserve estimate was well correlated with verbal memory function and with white matter integrity in the left fornix, suggesting a possible link between cognition and motor reserve in patients with PD.

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Is motor reserve associated with a rapid progression of Parkinson disease?
Myung Jun Lee, Kyoungjune Pak, Jae-Hyeok Lee, Seok Jong Chung, Min Seok Baek
Journal of Parkinson’s Disease.2025; 15(3): 552. CrossRef
Dopamine Transporter Imaging as Objective Monitoring Biomarker in Parkinson's Disease
Verena Dzialas, Gérard N. Bischof, Kathrin Möllenhoff, Alexander Drzezga, Thilo van Eimeren
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Tal Kozlovski, Inbal Maidan, Eran Gazit, Amgad Droby, Avner Thaler, Jeffrey M Hausdorff, Nir Giladi, Yoav Benjamini, Anat Mirelman
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Hippocampal Perfusion Affects Motor and Cognitive Functions in Parkinson Disease: An Early Phase 18F‐FP‐CIT Positron Emission Tomography Study
Min Young Chun, Seok Jong Chung, Su Hong Kim, Chan Wook Park, Seong Ho Jeong, Hye Sun Lee, Phil Hyu Lee, Young H. Sohn, Yong Jeong, Yun Joong Kim
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Occipital hypoperfusion and motor reserve in Parkinson’s disease: an early-phase 18F-FP-CIT PET study
Yeo Jun Yoon, Su Hong Kim, Seong Ho Jeong, Chan Wook Park, Hye Sun Lee, Phil Hyu Lee, Yun Joong Kim, Young H. Sohn, Yong Jeong, Seok Jong Chung
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Extra-Basal Ganglia Brain Structures Are Related to Motor Reserve in Parkinson’s Disease
Jinyoung Youn, Ji Hye Won, Mansu Kim, Junmo Kwon, Seung Hwan Moon, Minkyeong Kim, Jong Hyun Ahn, Jun Kyu Mun, Hyunjin Park, Jin Whan Cho
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Review Article

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.

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Original Article

Association between Olfactory Deficit and Motor and Cognitive Function in Parkinson’s Disease: Han Soo Yoo, Seok Jong Chung, Yang Hyun Lee, Byoung Seok Ye, Young H. Sohn, Phil Hyu Lee; J Mov Disord. 2020;13(2):133-141. Published online April 6, 2020; DOI: https://doi.org/10.14802/jmd.19082

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302 Download
32 Web of Science
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Abstract PDF Supplementary Material

Objective
To investigate whether baseline olfactory dysfunction in Parkinson’s disease (PD) patients is associated with baseline and longitudinal motor and cognitive function.
Methods
We recruited 228 drug-naïve PD patients who were followed for a mean of 6 years. Patients underwent the Cross-Cultural Smell Identification Test (CCSIT), a neuropsychological test, and N-(3-[18F]fluoropropyl)-2β-carbomethoxy-3β-(4-iodophenyl) nortropane positron emission tomography within 6 months of the baseline evaluation. Olfactory dysfunction was categorized as normosmia (CCSIT score ≥ 9), hyposmia (CCSIT score 5–8), and anosmia (CCSIT score ≤ 4). During the follow-up period, we investigated changes in the levodopa-equivalent dose (LED) and the occurrence of wearing-off, levodopa-induced dyskinesia, and dementia.
Results
Among the PD patients, 80.7% were hyposmic at the time of diagnosis, and 26.1% were anosmic. Baseline olfactory dysfunction was not associated with either initial parkinsonian motor symptoms or with the longitudinal LED increment and motor complications. Meanwhile, the anosmic group had lower baseline scores on the Korea version of the Boston Naming Test and Stroop color reading test than the normosmic and hyposmic groups. The anosmic group exhibited a higher rate of conversion to dementia than the normosmic [adjusted hazard ratio (HR) 3.99, 95% confidence interval (CI) 1.08–14.72] and hyposmic (adjusted HR 2.48, 95% CI 1.15–5.32) PD groups, regardless of baseline motor deficits and cognitive status.
Conclusion
Baseline olfactory dysfunction was not associated with motor deficits and complications, but it was associated with cognitive dysfunction and prognosis, suggesting that severe olfactory impairment may reflect early cortical involvement, probably in the frontotemporal region, and rapid spreading of Lewy body pathology.

Citations

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Case Report

A Case of Abnormal Postures in the Left Extremities after Pontine Hemorrhage: Dystonia or Pseudodystonia?: Chan Wook Park, Seok Jong Chung, Young H. Sohn, Phil Hyu Lee; J Mov Disord. 2020;13(1):62-65. Published online January 31, 2020; DOI: https://doi.org/10.14802/jmd.19074

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136 Download
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Abstract PDF Supplementary Material

It is difficult to determine the pathoanatomical correlates of dystonia because of its complex pathophysiology, and most cases with secondary dystonia are associated with basal ganglia lesions. Moreover, it is a challenging issue that patients with abnormal postures accompanied by other neurological findings in the affected body part (e.g., sensory loss) can be diagnosed with true dystonia or pseudodystonia. Here, we report a case of abnormal postures with loss of proprioception in the left extremities after right dorsal pontine hemorrhage.

Citations

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Melanie Aing, Craig DiTommaso
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Original Articles

Heterogeneous Patterns of Striatal Dopamine Loss in Patients with Young- versus Old-Onset Parkinson’s Disease: Impact on Clinical Features: Seok Jong Chung, Han Soo Yoo, Yang Hyun Lee, Phil Hyu Lee, Young H. Sohn; J Mov Disord. 2019;12(2):113-119. Published online May 30, 2019; DOI: https://doi.org/10.14802/jmd.18064

10,859 View
176 Download
29 Web of Science
30 Crossref

Abstract PDF Supplementary Material

Objective
Ample evidence has suggested that age at onset of Parkinson’s disease (PD) is associated with heterogeneous clinical features in individuals. We hypothesized that this may be attributed to different patterns of nigrostriatal dopamine loss.
Methods
A total of 205 consecutive patients with de novo PD who underwent 18F-FP-CIT PET scans (mean follow-up duration, 6.31 years) were divided into three tertile groups according to their age at onset of parkinsonian motor symptoms. Striatal dopamine transporter (DAT) availability was compared between the old- (n = 73) and young-onset (n = 66) groups. In addition, the risk of developing freezing of gait (FOG) and longitudinal requirements for dopaminergic medications were examined.
Results
The old-onset PD group (mean age at onset, 72.66 years) exhibited more severe parkinsonian motor signs than the young-onset group (52.58 years), despite comparable DAT availability in the posterior putamen; moreover, the old-onset group exhibited more severely decreased DAT availability in the caudate than the young-onset group. A Cox regression model revealed that the old-onset PD group had a higher risk for developing FOG than the young-onset group [hazard ratio 2.523, 95% confidence interval (1.239–5.140)]. The old-onset group required higher doses of dopaminergic medications for symptom control than the young-onset group over time.
Conclusion
The present study demonstrated that the old-onset PD group exhibited more severe dopamine loss in the caudate and were more likely to develop gait freezing, suggesting that age at onset may be one of the major determinants of the pattern of striatal dopamine depletion and progression of gait disturbance in PD.

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Patients and Their Caregivers’ Burdens for Parkinson’s Disease in Korea: Jong Sam Baik, Joong-Seok Kim, Seong-Beom Koh, Jin Whan Cho, Phil Hyu Lee, Hyeo-Il Ma, Yun Joong Kim, Tae-Beom Ahn, Sang Jin Kim, Yong Duk Kim, Seong-min Choi, Ho-Won Lee, Hee Tae Kim; J Mov Disord. 2017;10(3):109-115. Published online September 22, 2017; DOI: https://doi.org/10.14802/jmd.17053

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Abstract PDF

Objective
Many patients with Parkinson’s disease (PD) suffer from motor and non-motor symptoms. According to these variable symptoms of PD, patients or caregivers have a poorer quality of life than patients with other neurodegenerative diseases. Since the difficulties are varied for all patients, prioritizing their difficulties differs among all cases. The goal of this study was to investigate the burdens of PD among the caregivers as well as patients and to identify areas requiring aid from the government.
Methods
We surveyed the awareness and perceptions of PD in patients and caregivers of PD by a face-to-face questionnaire. The questionnaire was divided into three sections: symptoms of PD (part A), desire for policies (part B), and difficulties faced by their caregivers (part C). Part A comprised 8 questions, Part B had 2 questions, and Part C had 3 questions.
Results
In total, 853 subjects (702 patients and 151 caregivers) were enrolled in this study. The major difficulties experienced by PD patients were physical (67%), psychiatric (60%) and socio-economic (52%). Assessing the physical difficulties, more than half the patients experienced severe difficulties (29% very severe, 39% severe). Psychiatric difficulties were assessed as severe (35%) and very severe (21%) among the patients. Severe difficulties were also experienced socio-economically, at 52% in patients and 49% in caregivers, especially among patients in their fifties (58%) and those with their spouse (65%) as caregivers. The topmost need was the introduction of new technology for treatment of PD (62%), followed by relief of costs for treatment (38%) and a family support system (31%). The majority (91%) of the patients were diagnosed with PD within two years after onset of symptoms.
Conclusion
We know that the difficulties of PD and the needs for government assistance are different between patients and caregivers. These results emphasize that perceiving the difficulties and needs of patients and caregivers early can help to prevent and ameliorate the burden of disease.

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Review Article

MicroRNA Biomarkers in Neurodegenerative Diseases and Emerging NanoSensors Technology: Pratik Shah, Seok Keun Cho, Peter Waaben Thulstrup, Morten Jannik Bjerrum, Phil Hyu Lee, Ju-Hee Kang, Yong-Joo Bhang, Seong Wook Yang; J Mov Disord. 2017;10(1):18-28. Published online January 18, 2017; DOI: https://doi.org/10.14802/jmd.16037

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Abstract PDF

MicroRNAs (miRNAs) are essential small RNA molecules (20–24 nt) that negatively regulate the expression of target genes at the post-transcriptional level. Due to their roles in a variety of biological processes, the aberrant expression profiles of miRNAs have been identified as biomarkers for many diseases, such as cancer, diabetes, cardiovascular disease and neurodegenerative diseases. In order to precisely, rapidly and economically monitor the expression of miRNAs, many cutting-edge nanotechnologies have been developed. One of the nanotechnologies, based on DNA encapsulated silver nanoclusters (DNA/AgNCs), has increasingly been adopted to create nanoscale bio-sensing systems due to its attractive optical properties, such as brightness, tuneable emission wavelengths and photostability. Using the DNA/AgNCs sensor methods, the presence of miRNAs can be detected simply by monitoring the fluorescence alteration of DNA/AgNCs sensors. We introduce these DNA/ AgNCs sensor methods and discuss their possible applications for detecting miRNA biomarkers in neurodegenerative diseases.

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Original Article

Validation of the Korean Version of the Scale for Outcomes in Parkinson’s Disease-Autonomic: Ji-Young Kim, In-Uk Song, Seong-Beom Koh, Tae-Beom Ahn, Sang Jin Kim, Sang-Myung Cheon, Jin Whan Cho, Yun Joong Kim, Hyeo-Il Ma, Mee-Young Park, Jong Sam Baik, Phil Hyu Lee, Sun Ju Chung, Jong-Min Kim, Han-Joon Kim, Young-Hee Sung, Do Young Kwon, Jae-Hyeok Lee, Jee-Young Lee, Ji Sun Kim, Ji Young Yun, Hee Jin Kim, Jin Young Hong, Mi-Jung Kim, Jinyoung Youn, Ji Seon Kim, Eung Seok Oh, Hui-Jun Yang, Won Tae Yoon, Sooyeoun You, Kyum-Yil Kwon, Hyung-Eun Park, Su-Yun Lee, Younsoo Kim, Hee-Tae Kim, Joong-Seok Kim; J Mov Disord. 2017;10(1):29-34. Published online January 18, 2017; DOI: https://doi.org/10.14802/jmd.16057

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Abstract PDF Supplementary Material

Objective
Autonomic symptoms are commonly observed in patients with Parkinson’s disease (PD) and often limit the activities of daily living. The Scale for Outcomes in Parkinson’s disease-Autonomic (SCOPA-AUT) was developed to evaluate and quantify autonomic symptoms in PD. The goal of this study was to translate the original SCOPA-AUT, which was written in English, into Korean and to evaluate its reliability and validity for Korean PD patients.
Methods
For the translation, the following processes were performed: forward translation, backward translation, expert review, pretest of the pre-final version and development of the final Korean version of SCOPA-AUT (K-SCOPA-AUT). In total, 127 patients with PD from 31 movement disorder clinics of university-affiliated hospitals in Korea were enrolled in this study. All patients were assessed using the K-SCOPA-AUT and other motor, non-motor, and quality of life scores. Test-retest reliability for the K-SCOPA-AUT was assessed over a time interval of 10−14 days.
Results
The internal consistency and reliability of the K-SCOPA-AUT was 0.727 as measured by the mean Cronbach’s α-coefficient. The test-retest correlation reliability was 0.859 by the Guttman split-half coefficient. The total K-SCOPA-AUT score showed a positive correlation with other non-motor symptoms [the Korean version of non-motor symptom scale (K-NMSS)], activities of daily living (Unified Parkinson’s Disease Rating Scale part II) and quality of life [the Korean version of Parkinson’s Disease Quality of Life 39 (K-PDQ39)].
Conclusion
The K-SCOPA-AUT had good reliability and validity for the assessment of autonomic dysfunction in Korean PD patients. Autonomic symptom severities were associated with many other motor and non-motor impairments and influenced quality of life.

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Case Report

Familial Hyperekplexia, a Potential Cause of Cautious Gait: A New Korean Case and a Systematic Review of Phenotypes: Yoonju Lee, Nan Young Kim, Sangkyoon Hong, Su Jin Chung, Seong Ho Jeong, Phil Hyu Lee, Young H. Sohn; J Mov Disord. 2017;10(1):53-58. Published online December 27, 2016; DOI: https://doi.org/10.14802/jmd.16044

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Abstract PDF Supplementary Material

Familial hyperekplexia, also called startle disease, is a rare neurological disorder characterized by excessive startle responses to noise or touch. It can be associated with serious injury from frequent falls, apnea spells, and aspiration pneumonia. Familial hyperekplexia has a heterogeneous genetic background with several identified causative genes; it demonstrates both dominant and recessive inheritance in the α1 subunit of the glycine receptor (GLRA1), the β subunit of the glycine receptor and the presynaptic sodium and chloride-dependent glycine transporter 2 genes. Clonazepam is an effective medical treatment for hyperekplexia. Here, we report genetically confirmed familial hyperekplexia patients presenting early adult cautious gait. Additionally, we review clinical features, mode of inheritance, ethnicity and the types and locations of mutations of previously reported hyperekplexia cases with a GLRA1 gene mutation.

Citations

Citations to this article as recorded by

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Four Turkish families with hyperekplexia: A missense mutation and the exon 1–7 deletion in the GLRA1 gene
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Original Articles

The MMSE and MoCA for Screening Cognitive Impairment in Less Educated Patients with Parkinson’s Disease: Ji In Kim, Mun Kyung Sunwoo, Young H. Sohn, Phil Hyu Lee, Jin Y. Hong; J Mov Disord. 2016;9(3):152-159. Published online September 21, 2016; DOI: https://doi.org/10.14802/jmd.16020

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Abstract PDF

Objective
To explore whether the Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) can be used to screen for dementia or mild cognitive impairment (MCI) in less educated patients with Parkinson’s disease (PD).
Methods
We reviewed the medical records of PD patients who had taken the Korean MMSE (K-MMSE), Korean MoCA (K-MoCA), and comprehensive neuropsychological tests. Predictive values of the K-MMSE and K-MoCA for dementia or MCI were analyzed in groups divided by educational level.
Results
The discriminative powers of the K-MMSE and K-MoCA were excellent [area under the curve (AUC) 0.86–0.97] for detecting dementia but not for detecting MCI (AUC 0.64–0.85). The optimal screening cutoff values of both tests increased with educational level for dementia (K-MMSE < 15 for illiterate, < 20 for 0.5–3 years of education, < 23 for 4–6 years, < 25 for 7–9 years, and < 26 for 10 years or more; K-MoCA < 7 for illiterate, < 13 for 0.5–3 years, < 16 for 4–6 years, < 19 for 7–9 years, < 20 for 10 years or more) and MCI (K-MMSE < 19 for illiterate, < 26 for 0.5–3 years, < 27 for 4–6 years, < 28 for 7–9 years, and < 29 for 10 years or more; K-MoCA < 13 for illiterate, < 21 for 0.5–3 years, < 23 for 4–6 years, < 25 for 7–9 years, < 26 for 10 years or more).
Conclusion
Both MMSE and MoCA can be used to screen for dementia in patients with PD, regardless of educational level; however, neither test is sufficient to discriminate MCI from normal cognition without additional information.

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Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans: Jae-Hyeok Lee, Jongkyu Park, Ho-Sung Ryu, Hyeyoung Park, Young Eun Kim, Jin Yong Hong, Sang Ook Nam, Young-Hee Sung, Seung-Hwan Lee, Jee-Young Lee, Myung Jun Lee, Tae-Hyoung Kim, Chul Hyoung Lyoo, Sun Ju Chung, Seong Beom Koh, Phil Hyu Lee, Jin Whan Cho, Mee Young Park, Yun Joong Kim, Young H. Sohn, Beom Seok Jeon, Myung Sik Lee; J Mov Disord. 2016;9(1):20-27. Published online January 25, 2016; DOI: https://doi.org/10.14802/jmd.15058

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Objective
Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea.
Methods
We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN).
Results
Four subtypes of NBIA including PKAN (n = 30), PLA2G6-related neurodegeneration (n = 2), beta-propeller protein-associated neurodegeneration (n = 1), and aceruloplasminemia (n = 1) have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG). Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN.
Conclusions
We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.

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Gender Differences in Age-Related Striatal Dopamine Depletion in Parkinson’s Disease: Jae Jung Lee, Jee Hyun Ham, Phil Hyu Lee, Young H. Sohn; J Mov Disord. 2015;8(3):130-135. Published online September 10, 2015; DOI: https://doi.org/10.14802/jmd.15031

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Objective Gender differences are a well-known clinical characteristic of Parkinson’s disease (PD). In-vivo imaging studies demonstrated that women have greater striatal dopamine transporter (DAT) activity than do men, both in the normal population and in PD patients. We hypothesize that women exhibit more rapid aging-related striatal DAT reduction than do men, as the potential neuroprotective effect of estrogen wanes with age.
Methods This study included 307 de novo PD patients (152 men and 155 women) who underwent DAT scans for an initial diagnostic work-up. Gender differences in age-related DAT decline were assessed in striatal sub-regions using linear regression analysis.
Results Female patients exhibited greater DAT activity compared with male patients in all striatal sub-regions. The linear regression analysis revealed that age-related DAT decline was greater in the anterior and posterior caudate, and the anterior putamen in women compared with men; we did not observe this difference in other sub-regions.
Conclusions This study demonstrated the presence of gender differences in age-related DAT decline in striatal sub-regions, particularly in the antero-dorsal striatum, in patients with PD, presumably due to aging-related decrease in estrogen. Because this difference was not observed in the sensorimotor striatum, this finding also suggests that women may not have a greater capacity to tolerate PD pathogenesis than do men.

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Effect of Rivastigmine on Behavioral and Psychiatric Symptoms of Parkinson’s Disease Dementia: Yoon-Sang Oh, Joong-Seok Kim, Phil Hyu Lee; J Mov Disord. 2015;8(2):98-102. Published online May 31, 2015; DOI: https://doi.org/10.14802/jmd.15041

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Objective A recent study showed that rivastigmine and memantin improved behavioral and psychiatric symptoms of dementia (BPSD) in Alzheimer’s dementia. Furthermore, according to recent guidelines presented by the Movement Disorder Society, rivastigmine is efficacious for the treatment of dementia in Parkinson’s disease (PD). We investigated the efficacy of rivastigmine for BPSD in patients with Parkinson’s disease dementia (PDD).
Methods Twenty-three patients in whom cognitive impairment occurred at least one year after a diagnosis of PD participated in this open-label trial. Cognitive, psychiatric, and motor symptoms were assessed before and after 24 weeks of treatment with rivastigmine using unstructured clinical assessments and rating scales including the Unified Parkinson’s Disease Rating Scale, Mini-Mental State Examination (MMSE), and the Neuropsychiatric Inventory.
Results Age (± standard deviation) was 74.7 ± 5.9 years, average duration of PD was 3.5 ± 3.7 years, Hoehn and Yahr scores were 2.2 ± 0.8, and baseline MMSE scores were 19.1 ± 4.2. Improvements in global mental symptoms and neuropsychiatric symptoms were significant; among them, hallucination, depression and appetite changes improved. Caregiver distress significantly decreased, including distress resulting from hallucinations, depression, apathy, and appetite changes.
Conclusions Although controlled trials are required, the findings suggest that rivastigmine is useful for control of several neuropsychiatric symptoms and beneficial for caregiver distress in patients with PDD.

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Current Status of Huntington’s Disease in Korea: A Nationwide Survey and National Registry Analysis: Hyun Sook Kim, Chul Hyoung Lyoo, Phil Hyu Lee, Sang Jin Kim, Mee Young Park, Hyeo-Il Ma, Jae Hyeok Lee, Sook Kun Song, Jong Sam Baik, Jin Ho Kim, Myung Sik Lee; J Mov Disord. 2015;8(1):14-20. Published online January 31, 2015; DOI: https://doi.org/10.14802/jmd.14038

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Abstract PDF

Objective Huntington’s disease (HD) is a rare neurological disorder, and its current status in Korea is not well investigated. This study aims to determine the prevalence and incidence of HD and to investigate the clinical features of HD patients in Korea.
Methods We estimated the crude prevalence and annual incidence of HD based on the databases of the Rare Diseases Registry (RDR) and the National Health Insurance (NHI). The clinical data of genetically confirmed HD patients was collected from 10 referral hospitals and analyzed.
Results The mean calculated annual incidence was 0.06 cases per 100,000 persons, and the mean calculated prevalence was 0.38 based on the NHI database. The estimated crude prevalence based on the RDR was 0.41. Of the sixty-eight HD patients recruited, the mean age of onset was 44.16 ± 14.08 years and chorea was most frequently reported as the initial symptom and chief complaint. The mean CAG repeat number of the expanded allele was 44.7 ± 4.8 and correlated inversely with the age of onset (p < 0.001). About two-thirds of the patients have a positive family history, and HD patients without positive family history showed a delay in onset of initial symptoms, a prolonged interval between initial symptom onset and genetic diagnosis and a delay in the age of genetic diagnosis.
Conclusions To the best of our knowledge, this is the first study to estimate the prevalence and incidence of HD in Korea and the largest HD series in the Asian population. Our analyses might be useful for further studies and large-scale investigations in HD patients.

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Apathy and Olfactory Dysfunction in Early Parkinson’s Disease: Jin Yong Hong, Mun Kyung Sunwoo, Jee Hyun Ham, Jae Jung Lee, Phil Hyu Lee, Young H. Sohn; J Mov Disord. 2015;8(1):21-25. Published online January 31, 2015; DOI: https://doi.org/10.14802/jmd.14029

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Abstract PDF

Objective Olfactory and emotional dysfunctions are very common in patients with Parkinson’s disease (PD). Olfaction and emotions share common neuroanatomical substrates. Therefore, in this study, we evaluated the association between olfactory and emotional dysfunctions in patients with PD.
Methods Parkinson’s disease patients who had been assessed for their olfactory function and neuropsychiatric symptoms including emotional dysfunction were included. A logistic regression analysis was performed to evaluate the association between low olfaction and different neuropsychiatric symptoms.
Results The patients with low olfaction (cross cultural smell identification test score ≤ 6) showed a higher prevalence of apathy when compared with those with high olfaction, whereas the frequencies of other neuropsychiatric symptoms were comparable between the two groups. A multivariate logistic regression analysis revealed that the presence of apathy/indifference [odds ratio (OR) = 2.859, p = 0.007], age 70 years or more (OR = 2.281, p = 0.009), and the male gender (OR = 1.916, p = 0.030) were significantly associated with low olfaction.
Conclusions Our results demonstrate that apathy/indifference is a unique emotional dysfunction associated with olfactory dysfunction in PD. The findings also suggest that PD patients with low olfaction have a high prevalence of apathy.

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Neuropsychiatric Symptoms in Parkinson’s Disease Dementia Are Associated with Increased Caregiver Burden: Yoon-Sang Oh, Ji E. Lee, Phil Hyu Lee, Joong-Seok Kim; J Mov Disord. 2015;8(1):26-32. Published online January 31, 2015; DOI: https://doi.org/10.14802/jmd.14019

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Abstract PDF

Objective Neuropsychiatric symptoms are common in Parkinson’s disease dementia (PDD). Frequent and severe neuropsychiatric symptoms create high levels of distress for patients and caregivers, decreasing their quality of life. The aim of this study was to investigate neuropsychiatric symptoms that may contribute to increased caregiver burden in PDD patients.
Methods Forty-eight PDD patients were assessed using the 12-item Neuropsychiatric Inventory (NPI) to determine the frequency and severity of mental and behavioral problems. The Burden Interview and Caregiver Burden Inventory were used to evaluate caregiver burden.
Results All but one patient showed one or more neuropsychiatric symptoms. The three most frequent neuropsychiatric symptoms were apathy (70.8%) and anxiety (70.8%), followed by depression (68.7%). More severe neuropsychiatric symptoms were significantly correlated with increased caregiver burden. The domains of delusion, hallucination, agitation and aggression, anxiety, irritability and lability, and aberrant motor behavior were associated with caregiver stress. After controlling for age and other potential confounding variables, total NPI score was significantly associated with caregiver burden.
Conclusions The results of this study confirm that neuropsychiatric symptoms are frequent and severe in patients with PDD and are associated with increased caregiver distress. A detailed evaluation and management of neuropsychiatric symptoms in PDD patients appears necessary to improve patient quality of life and reduce caregiver burden.

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Analysis of the Substantia Innominata Volume in Patients with Parkinson’s Disease with Dementia, Dementia with Lewy Bodies, and Alzheimer’s Disease: Hee Jin Kim, Ji Eun Lee, Soo Jeong Shin, Young Ho Sohn, Phil Hyu Lee; J Mov Disord. 2011;4(2):68-72.; DOI: https://doi.org/10.14802/jmd.11014

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Abstract PDF

Background and Purpose

The substantia innominata (SI) contains the nucleus basalis of Meynert, which is the major source of cholinergic input to the cerebral cortex. We hypothesized that degeneration of the SI and its relationship to general cognitive performance differs in amyloidopathy and synucleinopathy.

Methods

We used magnetic resonance imaging (MRI)-based volumetric analysis to evaluate the SI volume in patients with amnestic mild cognitive impairment (aMCI), Alzheimer’s disease (AD), Parkinson’s disease-mild cognitive impairment (PD-MCI), PD with dementia (PDD), dementia with Lewy bodies (DLB), and healthy elderly controls. The correlation between SI volume and general cognitive performance, measured using the Korean version of the Mini-Mental State Examination (K-MMSE), was examined.

Results

Compared to control subjects, the mean normalized SI volume was significantly decreased in all of the other groups. The normalized SI volume did not differ between the subjects with PDD and DLB, whereas it was significantly smaller in subjects with PDD (p = 0.029) and DLB (p = 0.011) compared with AD. In subjects with PD-related cognitive impairment (PD-MCI, PDD, or DLB), there was a significant positive correlation between the SI volume and K-MMSE score (r = 0.366, p < 0.001), whereas no correlation was seen in subjects with AD-related cognitive impairment (aMCI or AD).

Conclusions

Our data suggest that the SI loss is greater in synucleinopathy-related dementia (PDD or DLB) than in AD and that the contribution of the SI to cognitive performance is greater in synucleinopathy than in amyloidopathy.

Citations

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Immunohistochemical relationships of huntingtin-associated protein 1 with choline acetyltransferase in the forebrain cholinergic nuclei of adult mice
Mirza Mienur Meher, Md Nabiul Islam, Akie Yanai, Marya Afrin, Mir Rubayet Jahan, Kanako Nozaki, Koh-hei Masumoto, Koh Shinoda
Cell and Tissue Research.2025; 401(3): 237. CrossRef
Regional associations between cerebrovascular disease and cholinergic white matter pathways in the Lewy body continuum
Anna Rennie, Milan Nemy, Cene Jerele, Iñigo Rodríguez-Baz, Victor Montal, Alexandre Bejanin, Milica G Kramberger, Dag Aarsland, Juan Fortea, Alberto Lleó, Eric Westman, Daniel Alcolea, Daniel Ferreira
npj Parkinson's Disease.2025;[Epub] CrossRef
Long‐term effects of cholinesterase inhibitors and memantine on cognitive decline, cardiovascular events, and mortality in dementia with Lewy bodies: An up to 10‐year follow‐up study
Hong Xu, Annegret Habich, Daniel Ferreira, Londos Elisabet, Eric Westman, Maria Eriksdotter
Alzheimer's & Dementia.2024; 20(10): 6740. CrossRef
Cortical Thickness and Complexity in aMCI Patients: Altered Pattern Analysis and Early Diagnosis
Mengling Tao, Zhongfeng Xie, Peiying Chen, Xiaowen Xu, Peijun Wang
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Separating dementia with Lewy bodies from Alzheimer’s disease dementia using a volumetric MRI classifier
Aniek M. van Gils, Antti J. Tolonen, Hanneke F. M. Rhodius-Meester, Patrizia Mecocci, Ritva Vanninen, Kristian Steen Frederiksen, Frederik Barkhof, Bas Jasperse, Jyrki Lötjönen, Wiesje M. van der Flier, Afina W. Lemstra
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Deep Learning Segmentation of the Nucleus Basalis of Meynert on 3T MRI
D.J. Doss, G.W. Johnson, S. Narasimhan, J.S. Shless, J.W. Jiang, H.F.J. González, D.L. Paulo, A. Lucas, K.A. Davis, C. Chang, V.L. Morgan, C. Constantinidis, B.M. Dawant, D.J. Englot
American Journal of Neuroradiology.2023; 44(9): 1020. CrossRef
Manual and automated analysis of atrophy patterns in dementia with Lewy bodies on MRI
Eya Khadhraoui, Sebastian Johannes Müller, Niels Hansen, Christian Heiner Riedel, Philip Langer, Charles Timäeus, Jens Wiltfang, Caroline Bouter, Claudia Lange, Marielle Ernst
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Cholinergic white matter pathways in dementia with Lewy bodies and Alzheimer’s disease
Julia Schumacher, Nicola J Ray, Calum A Hamilton, Paul C Donaghy, Michael Firbank, Gemma Roberts, Louise Allan, Rory Durcan, Nicola Barnett, John T O’Brien, John-Paul Taylor, Alan J Thomas
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Metric magnetic resonance imaging analysis reveals pronounced substantia-innominata atrophy in dementia with Lewy bodies with a psychiatric onset
Niels Hansen, Sebastian Johannes Müller, Eya Khadhraoui, Christian Heiner Riedel, Philip Langer, Jens Wiltfang, Charles-Arnold Timäus, Caroline Bouter, Marielle Ernst, Claudia Lange
Frontiers in Aging Neuroscience.2022;[Epub] CrossRef
In vivo nucleus basalis of Meynert degeneration in mild cognitive impairment with Lewy bodies
Julia Schumacher, John-Paul Taylor, Calum A. Hamilton, Michael Firbank, Ruth A. Cromarty, Paul C. Donaghy, Gemma Roberts, Louise Allan, Jim Lloyd, Rory Durcan, Nicola Barnett, John T. O'Brien, Alan J. Thomas
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Julia Schumacher, Alan J. Thomas, Luis R. Peraza, Michael Firbank, Ruth Cromarty, Calum A. Hamilton, Paul C. Donaghy, John T. O’Brien, John-Paul Taylor
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Transcriptional network analysis in frontal cortex in Lewy body diseases with focus on dementia with Lewy bodies
Gabriel Santpere, Paula Garcia‐Esparcia, Pol Andres‐Benito, Belen Lorente‐Galdos, Arcadi Navarro, Isidro Ferrer
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Deepak Kumbhare, Viktoras Palys, Jamie Toms, Chathurika S. Wickramasinghe, Kasun Amarasinghe, Milos Manic, Evan Hughes, Kathryn L. Holloway
Frontiers in Neuroscience.2018;[Epub] CrossRef
Dementia with Lewy Bodies: Molecular Pathology in the Frontal Cortex in Typical and Rapidly Progressive Forms
Paula Garcia-Esparcia, Irene López-González, Oriol Grau-Rivera, María Francisca García-Garrido, Anusha Konetti, Franc Llorens, Saima Zafar, Margarita Carmona, José Antonio del Rio, Inga Zerr, Ellen Gelpi, Isidro Ferrer
Frontiers in Neurology.2017;[Epub] CrossRef
Meynert’s Nucleus Complex White Matter Abnormalities in Autism Spectrum Disorders: An MRI Study
Matteo Pardini, Francesco G. Garaci, Laszlo Zaborszky, Filadelfo Coniglione, Gianluca Serafini, Martina Siracusano, Francesca Benassi, Leonardo Emberti Gialloreti
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Biomarkers for dementia and mild cognitive impairment in Parkinson's disease
Manuel Delgado‐Alvarado, Belén Gago, Irene Navalpotro‐Gomez, Haritz Jiménez‐Urbieta, María C. Rodriguez‐Oroz
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Nucleus basalis of Meynert revisited: anatomy, history and differential involvement in Alzheimer’s and Parkinson’s disease
Alan King Lun Liu, Raymond Chuen-Chung Chang, Ronald K. B. Pearce, Steve M. Gentleman
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Cognitive Impairments in Multiple System Atrophy of the Cerebellar Type: Hyun J. Hong, Sook Keun. Song, Phil Hyu Lee, Young Ho Sohn, Ji E. Lee; J Mov Disord. 2011;4(1):41-45.; DOI: https://doi.org/10.14802/jmd.11007

14,898 View
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Abstract PDF

Background and Purpose

We investigated the cognitive profiles in a large sample of patients with multiple system atrophy-cerebellar ataxia (MSA-C) and compared directly them in patients with clinical diagnosis of probable MSA-C without dementia and control subjects with intact cognition.

Methods

We prospectively enrolled 26 patients with clinical diagnosis of probable MSA-C. All patients underwent a standardized neuropsychological test of the Seoul Neuropsychological Screening Battery.

Results

The score of Korean version of the Mini- Mental State Examination was significantly lower in patients with MSA-C (27.2 ± 2.5) than in control subjects (28.9 ± 1.0, p = 0.003). Patients with MSA-C showed a significantly worse performance in visuospatial function, 3 words recall, verbal immediate, delayed and recognition memory, visual delayed memory, phonemic and sementic Controlled Oral Word Association Test, and ideomotor praxis (p < 0.05).

Conclusions

Patients with MSA-C show more severe and more widespread cognitive dysfunctions than controls. Our results also indicate that cognitive dysfunction in patients with MCA-C is suggestive of disruption of the cerebellocortical circuits.

Citations

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Stacha F. I. Reumers, Fleur L. P. Bongaerts, Frank-Erik de Leeuw, Bart P. C. van de Warrenburg, Dennis J. L. G. Schutter, Roy P. C. Kessels
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Morphological differences between the two major subtypes of multiple system atrophy with cognitive impairment
Kurt A. Jellinger
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Mild cognitive impairment in multiple system atrophy: a brain network disorder
Kurt A. Jellinger
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Cognition in Patients With Multiple System Atrophy (MSA) and Its Neuroimaging Correlation: A Prospective Case-Control Study
Santosh Dash, Rohan Mahale, M. Netravathi, Nitish L Kamble, Vikram Holla, Ravi Yadav, Pramod K Pal
Cureus.2022;[Epub] CrossRef
Cognition in multiple system atrophy: a single‐center cohort study
Sabine Eschlböck, Margarete Delazer, Florian Krismer, Thomas Bodner, Alessandra Fanciulli, Beatrice Heim, Antonio Heras Garvin, Christine Kaindlstorfer, Elfriede Karner, Katherina Mair, Christoph Rabensteiner, Cecilia Raccagni, Klaus Seppi, Werner Poewe,
Annals of Clinical and Translational Neurology.2020; 7(2): 219. CrossRef
Neuropathological findings in multiple system atrophy with cognitive impairment
Kurt A. Jellinger
Journal of Neural Transmission.2020; 127(7): 1031. CrossRef
An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention
Viorica Chelban, Daniela Catereniuc, Daniela Aftene, Alexandru Gasnas, Ekawat Vichayanrat, Valeria Iodice, Stanislav Groppa, Henry Houlden
Journal of Neurology.2020; 267(9): 2754. CrossRef
A case of multiple system atrophy
Jing Guo, Fuying Liu, Tingting Liu, Xin Zhang, Yong Luo
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Cognitive impairment before changes appear on [18F]‐fluoro‐D‐glucose positron emission tomography images in a patient with possible early‐stage cerebellar‐predominant multiple system atrophy
Masahiko Takaya, Masahiko Atsumi, Tomoyuki Hirose, Kazunari Ishii, Osamu Shirakawa
Psychogeriatrics.2016; 16(3): 216. CrossRef
Fragile X-associated tremor/ataxia syndrome: phenotypic comparisons with other movement disorders
Erin E. Robertson, Deborah A. Hall, Andrew R. McAsey, Joan A. O’Keefe
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A brain-targeted, modified neurosin (kallikrein-6) reduces α-synuclein accumulation in a mouse model of multiple system atrophy
Brian Spencer, Elvira Valera, Edward Rockenstein, Margarita Trejo-Morales, Anthony Adame, Eliezer Masliah
Molecular Neurodegeneration.2015;[Epub] CrossRef
Cognitive Impairment and Its Structural Correlates in the Parkinsonian Subtype of Multiple System Atrophy
Ji Sun Kim, Jin-ju Yang, Dong-Kyun Lee, Jong-min Lee, Jinyoung Youn, Jin Whan Cho
Neurodegenerative Diseases.2015; 15(5): 294. CrossRef

Case Reports

A Cerebellar Tremor in a Patient with Human Immunodeficiency Virus-1 Associated with Progressive Multifocal Leukoencephalopathy: Hee-Jin Kim, Jae-Jung Lee, Phil Hyu Lee; J Mov Disord. 2009;2(2):88-90.; DOI: https://doi.org/10.14802/jmd.09024

65,535 View
48 Download
2 Crossref

Abstract PDF

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system (CNS) caused by JC virus infection in oligodendrocytes, especially in patients with acquired immunodeficiency syndrome (AIDS). Movement disorders associated with PML are very rare. Here, we report a case of PML in an AIDS patient who presented with a cerebellar tremor, caused by lesions in the cerebellar outflow tract. A cerebellar tremor can be a rare clinical manifestation in patients with PML.

Citations

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Holmes tremor in progressive multifocal leukoencephalopathy: A video case report
Takako Matsushima, Ryotaro Ikeguchi, Mutsumi Iijima, Ayato Shimomura, Shuntaro Takahashi, Kazuo Nakamichi, Yuko Shimizu, Kazuo Kitagawa
Clinical and Experimental Neuroimmunology.2024; 15(2): 101. CrossRef
Holmes tremor caused by a natalizumab-related progressive multifocal leukoencephalopathy: a case report and brief review of the literature
Luca Magistrelli, Domizia Vecchio, Paola Naldi, Cristoforo Comi, Roberto Cantello
Neurological Sciences.2019; 40(9): 1943. CrossRef

Parkinsonsim due to a Chronic Subdural Hematoma: Bosuk Park, Sook Keun Song, Jin Yong Hong, Phil Hyu Lee; J Mov Disord. 2009;2(1):43-44.; DOI: https://doi.org/10.14802/jmd.09011

14,386 View
86 Download
4 Crossref

Abstract PDF

Subdural hematoma is a rare cause of parkinsonism. We present the case of a 78-year-old man with right-side dominant parkinsonism about 3 months after a minor head injury. MRI reveals a chronic subdural hematoma on the left side with mildly displaced midline structures. The parkinsonian features were almost completely disappeared after neurosurgical evacuation of the hematoma without any anti-parkinson drug.

Citations

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Systematic Review of Post-Traumatic Parkinsonism, an Emerging Parkinsonian Disorder Among Survivors of Traumatic Brain Injury
Catherine Rojvirat, Gabriel R. Arismendi, Erin Feinstein, Maynard Guzman, Bruce A. Citron, Vedad Delic
Neurotrauma Reports.2024; 5(1): 37. CrossRef
Parkinsonism-like features following reconstructive cranioplasty
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Chronic subdural hematoma-induced parkinsonism: A systematic review
Achmad Fahmi, Heru Kustono, Komang Sena Adhistira, Heri Subianto, Budi Utomo, Agus Turchan
Clinical Neurology and Neurosurgery.2021; 208: 106826. CrossRef
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Involuntary Scapular Movements as a Possible Manifestation of Radicular Myoclonus: Bosuk Park, Sook Keun Song, Phil Hyu Lee; J Mov Disord. 2008;1(2):104-106.; DOI: https://doi.org/10.14802/jmd.08021

9,899 View
46 Download

Abstract PDF: Radicular myoclonus (RM) is a kind of peripheral myoclonus exclusively related with traumatic spinal root lesion. Here we describe a case with involuntary scapular movements as a possible manifestation of RM. A 37-year-old woman without any underlying disease developedinvoluntary movements of left shoulder two days after cervical trauma. On needle electromyographic recordings, the myoclonic jerky movements were found in left serratus anterior and rhomboid major muscles, and the duration of bursts ranged from 100 to 300 ms with the irregular frequency of 1–2 Hz. Electromyography studies showed accompanying left C5 radiculopathy. Treatment with clonazepm markedly improved involuntary scapular movements.

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