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Functional Neuroanatomy for Posture and Gait Control
Kaoru Takakusaki
J Mov Disord. 2017;10(1):1-17.   Published online January 18, 2017
DOI: https://doi.org/10.14802/jmd.16062
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  • 345 Citations
AbstractAbstract PDF
Here we argue functional neuroanatomy for posture- gait control. Multi-sensory information such as somatosensory, visual and vestibular sensation act on various areas of the brain so that adaptable posture- gait control can be achieved. Automatic process of gait, which is steady-state stepping movements associating with postural reflexes including headeye coordination accompanied by appropriate alignment of body segments and optimal level of postural muscle tone, is mediated by the descending pathways from the brainstem to the spinal cord. Particularly, reticulospinal pathways arising from the lateral part of the mesopontine tegmentum and spinal locomotor network contribute to this process. On the other hand, walking in unfamiliar circumstance requires cognitive process of postural control, which depends on knowledges of self-body, such as body schema and body motion in space. The cognitive information is produced at the temporoparietal association cortex, and is fundamental to sustention of vertical posture and construction of motor programs. The programs in the motor cortical areas run to execute anticipatory postural adjustment that is optimal for achievement of goal-directed movements. The basal ganglia and cerebellum may affect both the automatic and cognitive processes of posturegait control through reciprocal connections with the brainstem and cerebral cortex, respectively. Consequently, impairments in cognitive function by damages in the cerebral cortex, basal ganglia and cerebellum may disturb posture-gait control, resulting in falling.

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Neurodegeneration with Brain Iron Accumulation: Diagnosis and Management
Penelope Hogarth
J Mov Disord. 2015;8(1):1-13.   Published online January 31, 2015
DOI: https://doi.org/10.14802/jmd.14034
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AbstractAbstract PDF
Neurodegeneration with brain iron accumulation (NBIA) encompasses a group of inherited disorders that share the clinical features of an extrapyramidal movement disorder accompanied by varying degrees of intellectual disability and abnormal iron deposition in the basal ganglia. The genetic basis of ten forms of NBIA is now known. The clinical features of NBIA range from rapid global neurodevelopmental regression in infancy to mild parkinsonism with minimal cognitive impairment in adulthood, with wide variation seen between and within the specific NBIA sub-type. This review describes the clinical presentations, imaging findings, pathologic features, and treatment considerations for this heterogeneous group of disorders.

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Many Faces of Parkinson’s Disease: Non-Motor Symptoms of Parkinson’s Disease
Hye Mi Lee, Seong-Beom Koh
J Mov Disord. 2015;8(2):92-97.   Published online May 31, 2015
DOI: https://doi.org/10.14802/jmd.15003
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AbstractAbstract PDF
Parkinson’s disease (PD) is a multi-systemic disorder that is characterized by a combination of motor and non-motor symptoms (NMS). The dopaminergic neurodegeneration of PD is involved in the genesis of NMS, but other conditions and side effects of levodopa are also associated with NMS. NMS can develop at all stage of PD and rapid eyeball movement sleep behavior disorder (RBD), constipation, depression, and olfactory dysfunction are considered prodromal signs of PD. Many NMS related with motor deficits and cognitive dysfunction. Some NMS including olfactory dysfunction, RBD and abnormal stereopsis are associated with presence of other NMS of PD. In addition, several NMS can be helpful to differentiate between idiopathic PD and other parkinsonian disorders. Early recognition and management of NMS in PD patients is important for preserving quality of life.

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Original Article
Exosome-Based Delivery of miR-124 in a Huntington’s Disease Model
Soon-Tae Lee, Wooseok Im, Jae-Jun Ban, Mijung Lee, Keun-Hwa Jung, Sang Kun Lee, Kon Chu, Manho Kim
J Mov Disord. 2017;10(1):45-52.   Published online January 18, 2017
DOI: https://doi.org/10.14802/jmd.16054
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AbstractAbstract PDF
Objective
Huntington’s disease (HD) is a genetic neurodegenerative disease that is caused by abnormal CAG expansion. Altered microRNA (miRNA) expression also causes abnormal gene regulation in this neurodegenerative disease. The delivery of abnormally downregulated miRNAs might restore normal gene regulation and have a therapeutic effect.
Methods
We developed an exosome-based delivery method to treat this neurodegenerative disease. miR-124, one of the key miRNAs that is repressed in HD, was stably overexpressed in a stable cell line. Exosomes were then harvested from these cells using an optimized protocol. The exosomes (Exo-124) exhibited a high level of miR-124 expression and were taken up by recipient cells.
Results
When Exo-124 was injected into the striatum of R6/2 transgenic HD mice, expression of the target gene, RE1-Silencing Transcription Factor, was reduced. However, Exo-124 treatment did not produce significant behavioral improvement.
Conclusion
This study serves as a proof of concept for exosome-based delivery of miRNA in neurodegenerative diseases.

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Review Articles
What Is Wrong with Balance in Parkinson’s Disease?
Jeong-Ho Park, Yeo-Jeong Kang, Fay Bahling Horak
J Mov Disord. 2015;8(3):109-114.   Published online September 10, 2015
DOI: https://doi.org/10.14802/jmd.15018
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AbstractAbstract PDF
Postural instability and resulting falls are major factors determining quality of life, morbidity, and mortality in individuals with Parkinson’s disease (PD). A better understanding of balance impairments would improve management of balance dysfunction and prevent falls in patients with PD. The effects of bradykinesia, rigidity, impaired proprioception, freezing of gait and attention on postural stability in patients with idiopathic PD have been well characterized in laboratory studies. The purpose of this review is to systematically summarize the types of balance impairments contributing to postural instability in people with PD.

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Altered Gut Microbiome and Intestinal Pathology in Parkinson’s Disease
Han-Lin Chiang, Chin-Hsien Lin
J Mov Disord. 2019;12(2):67-83.   Published online May 30, 2019
DOI: https://doi.org/10.14802/jmd.18067
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AbstractAbstract PDF
Parkinson’s disease (PD) is a common neurodegenerative disorder arising from an interplay between genetic and environmental risk factors. Studies have suggested that the pathological hallmarks of intraneuronal α-synuclein aggregations may start from the olfactory bulb and the enteric nervous system of the gut and later propagate to the brain via the olfactory tract and the vagus nerve. This hypothesis correlates well with clinical symptoms, such as constipation, that may develop up to 20 years before the onset of PD motor symptoms. Recent interest in the gut–brain axis has led to vigorous research into the gastrointestinal pathology and gut microbiota changes in patients with PD. In this review, we provide current clinical and pathological evidence of gut involvement in PD by summarizing the changes in gut microbiota composition and gut inflammation associated with its pathogenesis.

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Episodic Ataxias: Clinical and Genetic Features
Kwang-Dong Choi, Jae-Hwan Choi
J Mov Disord. 2016;9(3):129-135.   Published online September 21, 2016
DOI: https://doi.org/10.14802/jmd.16028
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AbstractAbstract PDF
Episodic ataxia (EA) is a clinically heterogeneous group of disorders that are characterized by recurrent spells of truncal ataxia and incoordination lasting minutes to hours. Most have an autosomal dominant inheritance pattern. To date, 8 subtypes have been defined according to clinical and genetic characteristics, and five genes are known to be linked to EAs. Both EA1 and EA2, which are caused by mutations in KCNA1 and CACNA1A, account for the majority of EA, but many patients with no identified mutations still exhibit EA-like clinical features. Furthermore, genetically confirmed EAs have mostly been identified in Caucasian families. In this article, we review the current knowledge on the clinical and genetic characteristics of EAs. Additionally, we summarize the phenotypic features of the genetically confirmed EA2 families in Korea.

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COVID-19: An Early Review of Its Global Impact and Considerations for Parkinson’s Disease Patient Care
Roongroj Bhidayasiri, Sasivimol Virameteekul, Jong-Min Kim, Pramod Kr. Pal, Sun-Ju Chung
J Mov Disord. 2020;13(2):105-114.   Published online April 30, 2020
DOI: https://doi.org/10.14802/jmd.20042
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AbstractAbstract PDF
While many infectious disorders are unknown to most neurologists, COVID-19 is very different. It has impacted neurologists and other health care workers, not only in our professional lives but also through the fear and panic within our own families, colleagues, patients and their families, and even in the wider public. COVID-19 affects all sorts of individuals, but the elderly with underlying chronic conditions are particularly at risk of severe disease, or even death. Parkinson’s disease (PD) shares a common profile as an age-dependent degenerative disorder, frequently associated with comorbidities, particularly cardiovascular diseases, so PD patients will almost certainly fall into the high-risk group. Therefore, the aim of this review is to explore the risk of COVID-19 in PD based on the susceptibility to severe disease, its impact on PD disease severity, potential long-term sequelae, and difficulties of PD management during this outbreak, where neurologists face various challenges on how we can maintain effective care for PD patients without exposing them, or ourselves, to the risk of infection. It is less than six months since the identification of the original COVID-19 case on New Year’s Eve 2019, so it is still too early to fully understand the natural history of COVID-19 and the evidence on COVID-19-related PD is scant. Though the possibilities presented are speculative, they are theory-based, and supported by prior evidence from other neurotrophic viruses closely related to SARS-CoV-2. Neurologists should be on high alert and vigilant for potential acute and chronic complications when encountering PD patients who are suspected of having COVID-19.

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Original Articles
The Frequency and Severity of Gastrointestinal Symptoms in Patients with Early Parkinson’s Disease
Hye-Young Sung, Jeong-Wook Park, Joong-Seok Kim
J Mov Disord. 2014;7(1):7-12.   Published online April 30, 2014
DOI: https://doi.org/10.14802/jmd.14002
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AbstractAbstract PDF
Objective: Although gastrointestinal dysfunctions occur in the majority of patients with Parkinson’s disease (PD), they are often unrecognized because many patients remain relatively asymptomatic in the early stage. We investigated the frequency of gastrointestinal symptoms in patients with PD using newly developed gastrointestinal symptom questionnaires.
Methods: Early PD patients with a symptom duration not exceeding 3 years were included in this study. All PD patients were evaluated using a questionnaire, which consisted of three relevant domains: oropharyngoesophageal (10 items); gastric (3 items); and intestinal-anorectal (7 items). The frequency of symptoms was calculated as a proportion with an item score ≥ 2.
Results: Of the 54 patients enrolled, 48 patients (88.9%) responded that bowel symptoms developed before the onset of Parkinsonian motor symptoms, and four patients reported that the onset of two types of symptoms (i.e., bowel and neurological) occurred approximately simultaneously, with only months between them. The frequencies of gastrointestinal symptoms are as follows: speech disturbance (40.7%), drooling (24.1%), sense of getting stuck (31.5%), choking (27.8%), globus pharyngis (16.7%), repetitive deglutition (29.6%), pain during swallowing (5.6%), food regurgitation (3.7%), acid reflux (7.4%), nausea/ vomiting (11.1%), early satiety (16.7%), postprandial fullness (14.8%), epigastric soreness (9.3%), abdominal pain (3.7%), constipation (46.3%), excessive strain during defecation (33.3%), fecal incontinence (7.4%), tenesmus (20.4%), loose stool or diarrhea (3.7%), and difficulty in relaxing anal sphincter (11.1%). Two patients were scored at zero.
Conclusions: Our findings confirm that gastrointestinal dysfunction occurs in early PD in relatively high frequency.

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Orthostatic and Supine Blood Pressures Are Associated with White Matter Hyperintensities in Parkinson Disease
Yoon-Sang Oh, Joong-Seok Kim, Kwang-Soo Lee
J Mov Disord. 2013;6(2):23-27.   Published online October 30, 2013
DOI: https://doi.org/10.14802/jmd.13006
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AbstractAbstract PDF
Background and Purpose:

Several reports on the elderly population have suggested that orthostatic hypotension is associated with white matter hyperintensities (WMH); however, little information is available on patients with Parkinson’s disease (PD).

Methods:

We analyzed the association blood pressure profiles during tilt table testing with WMH scores in 117 patients with PD. WMH were rated using the semiquantitative visual rating system proposed by Scheltens et al.

Results:

The presence of orthostatic hypotension was associated with increasing tendency of WMH score and the blood pressure changes during tilting and supine blood pressure were positively correlated with increasing WMH score.

Conclusions:

This finding indicates that hemodynamic changes associated with orthostatic hypotension may be associated with white matter changes in patients with PD.

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Review Articles
Clinical Approach to Progressive Supranuclear Palsy
Helen Ling
J Mov Disord. 2016;9(1):3-13.   Published online January 25, 2016
DOI: https://doi.org/10.14802/jmd.15060
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AbstractAbstract PDF
Sixty years ago, Steele, Richardson and Olszewski designated progressive supranuclear palsy (PSP) as a new clinicopathological entity in their seminal paper. Since then, in addition to the classic Richardson’s syndrome (RS), different clinical phenotypic presentations have been linked with this four-repeat tauopathy. The clinical heterogeneity is associated with variability of regional distribution and severity of abnormal tau accumulation and neuronal loss. In PSP subtypes, the presence of certain clinical pointers may be useful for antemortem prediction of the underlying PSP-tau pathology. Midbrain atrophy on conventional MRI correlates with the clinical phenotype of RS but is not predictive of PSP pathology. Cerebrospinal fluid biomarkers and tau ligand positron emission tomography are promising biomarkers of PSP. A multidisciplinary approach to meet the patients’ complex needs is the current core treatment strategy for this devastating disorder.

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123I-Metaiodobenzylguanidine Myocardial Scintigraphy in Lewy Body-Related Disorders: A Literature Review
Eun Joo Chung, Sang Jin Kim
J Mov Disord. 2015;8(2):55-66.   Published online May 31, 2015
DOI: https://doi.org/10.14802/jmd.15015
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AbstractAbstract PDF
Lewy body-related disorders are characterized by the presence of Lewy bodies and Lewy neurites, which have abnormal aggregations of α-synuclein in the nigral and extranigral areas, including in the heart. 123I-metaiodobenzylguanidine (MIBG) scintigraphy is a well-known tool to evaluate cardiac sympathetic denervation in the Lewy body-related disorders. MIBG scintigraphy showed low uptake of MIBG in the Lewy body-related disorders, including Parkinson’s disease, dementia with Lewy bodies, pure autonomic failure and rapid eye movement sleep behavior disorder. This review summarizes previous results on the diagnostic applications of MIBG scintigraphy in Lewy body-related disorders.

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Nonmotor Symptoms and Subthalamic Deep Brain Stimulation in Parkinson’s Disease
Han-Joon Kim, Beom S. Jeon, Sun Ha Paek
J Mov Disord. 2015;8(2):83-91.   Published online May 31, 2015
DOI: https://doi.org/10.14802/jmd.15010
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AbstractAbstract PDF
Subthalamic deep brain stimulation (STN DBS) is an established treatment for the motor symptoms in patients with advanced Parkinson’s disease (PD). In addition to improvements in motor symptoms, many studies have reported changes in various nonmotor symptoms (NMSs) after STN DBS in patients with PD. Psychiatric symptoms, including depression, apathy, anxiety, and impulsivity, can worsen or improve depending on the electrical stimulation parameters, the locations of the stimulating contacts within the STN, and changes in medications after surgery. Global cognitive function is not affected by STN DBS, and there is no increase in the incidence of dementia after STN DBS compared to that after medical treatment, although clinically insignificant declines in verbal fluency have been consistently reported. Pain, especially PD-related pain, improves with STN DBS. Evidence regarding the effects of STN DBS on autonomic symptoms and sleep-related problems is limited and remains conflicting. Many symptoms of nonmotor fluctuations, which are occasionally more troublesome than motor fluctuations, improve with STN DBS. Although it is clear that NMSs are not target symptoms for STN DBS, NMSs have a strong influence on the quality of life of patients with PD, and clinicians should thus be aware of these NMSs when deciding whether to perform surgery and should pay attention to changes in these symptoms after STN DBS to ensure the optimal care for patients.

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Gastrointestinal Autonomic Dysfunction in Patients with Parkinson’s Disease
Joong-Seok Kim, Hye-Young Sung
J Mov Disord. 2015;8(2):76-82.   Published online May 31, 2015
DOI: https://doi.org/10.14802/jmd.15008
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AbstractAbstract PDF
Currently, gastrointestinal dysfunctions in Parkinson’s disease (PD) are well-recognized problems and are known to be an initial symptom in the pathological process that eventually results in PD. Gastrointestinal symptoms may result from the involvement of either the central or enteric nervous systems, or these symptoms may be side effects of antiparkinsonian medications. Weight loss, excessive salivation, dysphagia, nausea/gastroparesis, constipation, and defecation dysfunction all may occur. Increased identification and early detection of these symptoms can result in a significant improvement in the quality of life for PD patients.

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Update on Current Technologies for Deep Brain Stimulation in Parkinson’s Disease
Michelle Paff, Aaron Loh, Can Sarica, Andres M. Lozano, Alfonso Fasano
J Mov Disord. 2020;13(3):185-198.   Published online August 31, 2020
DOI: https://doi.org/10.14802/jmd.20052
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AbstractAbstract PDF
Deep brain stimulation (DBS) is becoming increasingly central in the treatment of patients with Parkinson’s disease and other movement disorders. Recent developments in DBS lead and implantable pulse generator design provide increased flexibility for programming, potentially improving the therapeutic benefit of stimulation. Directional DBS leads may increase the therapeutic window of stimulation by providing a means of avoiding current spread to structures that might give rise to stimulation-related side effects. Similarly, control of current to individual contacts on a DBS lead allows for shaping of the electric field produced between multiple active contacts. The following review aims to describe the recent developments in DBS system technology and the features of each commercially available DBS system. The advantages of each system are reviewed, and general considerations for choosing the most appropriate system are discussed.

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JMD : Journal of Movement Disorders