JMD Scientific Statements and Guidelines in the Early View area have been typeset, copyedited, and reviewed by the writing group or author, but they have not appeared in a print or online issue of the journal. In some cases, the statement or guideline may be revised before it is eventually published in print and online.
Each manuscript published online ahead of print is citable, with the official publication date being the date of the manuscript's first online posting. JMD papers are assigned Digital Object Identifiers (DOIs), providing a persistent, permanent way to identify manuscripts published in the online environment.
Original Articles
- Retinal Thinning as a Marker of Disease Severity in Progressive Supranuclear Palsy
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Yueting Chen, Haotian Wang, Bo Wang, Wenbo Li, Panpan Ye, Wen Xu, Peng Liu, Xinhui Chen, Zhidong Cen, Zhiyuan Ouyang, Sheng Wu, Xiaofeng Dou, Yi Liao, Hong Zhang, Mei Tian, Wei Luo
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Received May 21, 2023 Accepted September 25, 2023 Published online September 26, 2023
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DOI: https://doi.org/10.14802/jmd.23102
[Epub ahead of print]
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Abstract
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- Objective
Progressive supranuclear palsy (PSP) involves a variety of visual symptoms that are thought to be partially caused by structural abnormalities of the retina. However, the relationship between retinal structural changes, disease severity, and intracranial alterations remains unknown. We investigated distinct retinal thinning patterns and their relationship with clinical severity and intracranial alterations in a PSP cohort.
Methods
We enrolled 19 patients with PSP (38 eyes) and 20 age-matched healthy controls (40 eyes). All of the participants underwent peripapillary and macular optical coherence tomography. Brain 11C-2β-carbomethoxy-3β-(4-fluorophenyl) tropane (11C-CFT) and 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography imaging were also performed in patients with PSP. We investigated the association between retinal thickness changes and clinical features, striatal dopamine transporter availability, and cerebral glucose metabolism.
Results
The peripapillary retinal nerve fiber layer (pRNFL) and macula were significantly thinner in patients with PSP than in controls. The thickness of the superior sector of the pRNFL demonstrated a significant negative relationship with the Movement Disorder Society-Unified Parkinson’s Disease Rating Scale part III and Hoehn and Yahr staging scale scores. A significant negative correlation was found between outer inferior macular thickness and disease duration. Outer temporal macular thickness was positively correlated with Montreal Cognitive Assessment scores. In PSP, lower outer temporal macular thickness was also positively correlated with decreased dopamine transporter binding in the caudate.
Conclusion
The pRNFL and macular thinning may be candidate markers for monitoring disease severity. Additionally, macular thinning may be an in vivo indicator of nigrostriatal dopaminergic cell degeneration in PSP patients.
- Emotion Recognition in Multiple System Atrophy: An Exploratory Eye-Tracking Study
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Victoria Sidoroff, Federico Carbone, Philipp Ellmerer, Stefanie Bair, Alexandra Hoffmann, Thomas Maran, Florian Krismer, Philipp Mahlknecht, Katherina Mair, Cecilia Raccagni, Jean-Pierre Ndayisaba, Klaus Seppi, Gregor K. Wenning, Atbin Djamshidian
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Received May 1, 2023 Accepted September 24, 2023 Published online September 26, 2023
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DOI: https://doi.org/10.14802/jmd.23090
[Epub ahead of print]
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Abstract
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- Objective
Emotional processing is a core feature of social interactions and has been well studied in patients with idiopathic Parkinson’s disease (PD), albeit with contradictory results. However, these studies excluded patients with atypical parkinsonism, such as multiple system atrophy (MSA). The objective of this exploratory study was to provide better insights into emotion processing in patients with MSA using eye tracking data.
Methods
We included 21 MSA patients, 15 PD patients and 19 matched controls in this study. Participants performed a dynamic and a static emotion recognition task, and gaze fixations were analyzed in different areas of interest. Participants underwent neuropsychological testing and assessment of depression and alexithymia.
Results
MSA patients were less accurate in recognizing anger than controls (p = 0.02) and had overall fewer fixations than controls (p = 0.001). In the static task, MSA patients had fewer fixations (p < 0.001) and a longer time to first fixation (p = 0.026) on the eye region. Furthermore, MSA patients had a longer fixation duration overall than PD patients (p = 0.004) and longer fixations on the nose than controls (p = 0.005). Alexithymia scores were higher in MSA patients compared to controls (p = 0.038).
Conclusion
This study demonstrated impaired recognition of anger in MSA patients compared to HCs. Fewer and later fixations on the eyes along with a center bias suggest avoidance of eye contact, which may be a characteristic gaze behavior in MSA patients.
- Hair Loss: A Well-Known Yet Understudied Symptom in Parkinson’s Disease Patients During Dopaminergic Therapy
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Jungyeun Lee, Hwa Jung Ryu, Soon Young Hwang, Seong-Beom Koh
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Received May 1, 2023 Accepted September 24, 2023 Published online September 26, 2023
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DOI: https://doi.org/10.14802/jmd.23088
[Epub ahead of print]
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Abstract
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- Objective
Hair loss has been reported to occur during dopaminergic therapy in patients with Parkinson’s disease. The mechanism by which dopaminergic therapy induces hair loss is not well understood. Dopamine receptors are present in the hair follicle, where they regulate melanin production. However, the role of dopamine receptors in hair growth is still not well understood. This study aimed to evaluate the prevalence of hair loss and identify factors associated with complaints of hair loss in patients with Parkinson’s disease.
Methods
A cross-sectional design involving 495 Parkinson’s disease patients was applied to evaluate hair loss status. Patients completed a questionnaire, and scalp/hair examinations were performed. Patients with underlying conditions that could affect hair loss and those prescribed medications known to increase the risk of hair loss were excluded. Finally, 291 patients (58.8%) were included for analysis.
Results
Among the 495 patients, 138 (27.9%) reported hair loss. Interestingly, more than half of the patients who complained of hair loss (79 out of 138) did not utilize treatments such as hair products, massage, dietary modifications, or alopecia medications. Hair inspection by a single investigator revealed objective hair loss in 263 patients (53.1%). An analysis of factors associated with hair loss complaints showed that the intake of dopaminergic medications with a levodopa-equivalent daily dose > 448 mg was associated with complaints of hair loss.
Conclusion
Dopaminergic medication is associated with hair loss complaints in Parkinson’s disease patients.
- Caregiver Burden of Patients With Huntington’s Disease in South Korea
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Chan Young Lee, Chaewon Shin, Yun Su Hwang, Eungseok Oh, Manho Kim, Hyun Sook Kim, Sun Ju Chung, Young Hee Sung, Won Tae Yoon, Jin Whan Cho, Jae-Hyeok Lee, Han-Joon Kim, Hee Jin Chang, Beomseok Jeon, Kyung Ah Woo, Seong-Beom Koh, Kyum-Yil Kwon, Jangsup Moon, Young Eun Kim, Jee-Young Lee
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Received July 19, 2023 Accepted September 8, 2023 Published online September 11, 2023
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DOI: https://doi.org/10.14802/jmd.23134
[Epub ahead of print]
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Abstract
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Supplementary Material
- Objective
This is the first prospective cohort study of Huntington’s disease (HD) in Korea. This study aimed to investigate the caregiver burden in relation to the characteristics of patients and caregivers.
Methods
From August 2020 to February 2022, we enrolled patients with HD from 13 university hospitals in Korea. We used the 12-item Zarit Burden Interview (ZBI-12) to evaluate the caregiver burden. We evaluated the clinical associations of the ZBI-12 scores by linear regression analysis and investigated the differences between the low- and high-burden groups.
Results
Sixty-five patients with HD and 45 caregivers were enrolled in this cohort study. The average age at onset of motor symptoms was 49.3 ± 12.3 years, with an average cytosine-adenine-guanine (CAG)n of 42.9 ± 4.0 (38–65). The median ZBI-12 score among our caregivers was 17.6 ± 14.2. A higher caregiver burden was associated with a more severe Shoulson–Fahn stage (p = 0.038) of the patients. A higher ZBI-12 score was also associated with lower independence scale (B = -0.154, p = 0.006) and functional capacity (B = -1.082, p = 0.002) scores of patients. The caregiving duration was longer in the high- than in the low-burden group. Caregivers’ demographics, blood relation, and marital and social status did not affect the burden significantly.
Conclusion
HD patients’ neurological status exerts an enormous impact on the caregiver burden regardless of the demographic or social status of the caregiver. This study emphasizes the need to establish an optimal support system for families dealing with HD in Korea. A future longitudinal analysis could help us understand how disease progression aggravates the caregiver burden throughout the entire disease course.
Letters to the editor
- Factors associated with anticholinergic-induced oral-buccal-lingual dyskinesia in Parkinson’s disease
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Joonyoung Ha, Suk Yun Kang, Kyoungwon Baik, Young H. Sohn, Phil Hyu Lee, Min Seok Baek, Jin Yong Hong
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Received April 8, 2023 Accepted September 22, 2023 Published online September 22, 2023
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DOI: https://doi.org/10.14802/jmd.23069
[Epub ahead of print]
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Supplementary Material
- Jaw Opening Myoclonus in Subacute Sclerosing Panencephalitis: A New Phenotypic Observation
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Divyani Garg, Vanshika Kakkar, Suvasini Sharma
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Received August 7, 2023 Accepted September 14, 2023 Published online September 14, 2023
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DOI: https://doi.org/10.14802/jmd.23158
[Epub ahead of print]
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- A Novel Pathogenic PSEN1 Variant in a Patient With Dystonia-Parkinsonism Without Dementia
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Maria Chiara Malaguti, Alessio Di Fonzo, Chiara Longo, Raffaella Di Giacopo, Costanza Papagno, Davide Donner, Umberto Rozzanigo, Edoardo Monfrini
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Received June 30, 2023 Accepted September 13, 2023 Published online September 14, 2023
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DOI: https://doi.org/10.14802/jmd.23125
[Epub ahead of print]
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- “Face of the Giant Panda” Sign and Bilateral Thalamic Hyperintensity in Isoniazid-Induced Ataxia
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Vikram V. Holla, Manjunath Netravathi, Nitish Kamble, Jitender Saini, Pramod Kumar Pal
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Received June 4, 2023 Accepted August 2, 2023 Published online August 2, 2023
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DOI: https://doi.org/10.14802/jmd.23112
[Epub ahead of print]
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Supplementary Material
Brief communication
- A Survey of Perspectives on Telemedicine for Patients With Parkinson’s Disease
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Jae Young Joo, Ji Young Yun, Young Eun Kim, Yu Jin Jung, Ryul Kim, Hui-Jun Yang, Woong-Woo Lee, Aryun Kim, Han-Joon Kim
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Received July 12, 2023 Accepted August 21, 2023 Published online August 22, 2023
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DOI: https://doi.org/10.14802/jmd.23130
[Epub ahead of print]
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Abstract
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Supplementary Material
- Objective
Parkinson’s disease (PD) patients often find it difficult to visit hospitals because of motor symptoms, distance to the hospital, or the absence of caregivers. Telemedicine is one way to solve this problem.
Methods
We surveyed 554 PD patients from eight university hospitals in Korea. The questionnaire consisted of the clinical characteristics of the participants, possible teleconferencing methods, and preferences for telemedicine.
Results
A total of 385 patients (70%) expressed interest in receiving telemedicine. Among them, 174 preferred telemedicine whereas 211 preferred in-person visits. The longer the duration of disease, and the longer the time required to visit the hospital, the more patients were interested in receiving telemedicine.
Conclusion
This is the first study on PD patients’ preferences regarding telemedicine in Korea. Although the majority of patients with PD have a positive view of telemedicine, their interest in receiving telemedicine depends on their different circumstances.