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Volume 13(1); January 2020
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Review Articles
Diagnostic Criteria for Dementia with Lewy Bodies: Updates and Future Directions
Masahito Yamada, Junji Komatsu, Keiko Nakamura, Kenji Sakai, Miharu Samuraki-Yokohama, Kenichi Nakajima, Mitsuhiro Yoshita
J Mov Disord. 2020;13(1):1-10.   Published online November 8, 2019
DOI: https://doi.org/10.14802/jmd.19052
  • 19,078 View
  • 1,413 Download
  • 15 Citations
AbstractAbstract PDF
The aim of this article is to describe the 2017 revised consensus criteria for the clinical diagnosis of dementia with Lewy bodies (DLB) with future directions for the diagnostic criteria. The criteria for the clinical diagnosis of probable and possible DLB were first published as the first consensus report in 1996 and were revised in the third consensus report in 2005. After discussion at the International DLB Conference in Fort Lauderdale, Florida, USA, in 2015, the International DLB Consortium published the fourth consensus report including the revised consensus criteria in 2017. The 2017 revised criteria clearly distinguish between clinical features and diagnostic biomarkers. Significant new information about previously reported aspects of DLB has been incorporated, with increased diagnostic weighting given to rapid eye movement (REM) sleep behavior disorder (RBD) and iodine-123-metaiodobenzylguanidine (MIBG) myocardial scintigraphy. Future directions include the development of the criteria for early diagnosis (prodromal DLB) and the establishment of new biomarkers that directly indicate Lewy-related pathology, including α-synuclein imaging, biopsies of peripheral tissues (skin, etc.) for the demonstration of α-synuclein deposition, and biochemical markers (cerebrospinal fluid/blood), as well as the pathological evaluation of the sensitivity and specificity of the 2017 revised diagnostic criteria. In conclusion, the revised consensus criteria for the clinical diagnosis of DLB were reported with the incorporation of new information about DLB in 2017. Future directions include the development of the criteria for early diagnosis and the establishment of biomarkers directly indicative of Lewy-related pathology.
Immunotherapy Targeting Neurodegenerative Proteinopathies: α-Synucleinopathies and Tauopathies
Junghwan Shin, Han-Joon Kim, Beomseok Jeon
J Mov Disord. 2020;13(1):11-19.   Published online December 19, 2019
DOI: https://doi.org/10.14802/jmd.19057
  • 5,826 View
  • 372 Download
  • 12 Citations
AbstractAbstract PDF
α-Synuclein and tau deposition in the central nervous system is responsible for various parkinsonian syndromes, including Parkinson’s disease, multiple system atrophy, dementia with Lewy bodies, progressive supranuclear palsy and corticobasal degeneration. Emerging evidence has suggested that pathologic α-synuclein and tau are transmitted from cell to cell and further accelerate the aggregation of pathologic proteins in neighboring cells. Furthermore, extracellular pathologic proteins have also been reported to provoke inflammatory responses that lead to neurodegeneration. Therefore, immunotherapies targeting extracellular α-synuclein and tau have been proposed as potential disease-modifying strategies. In this review, we summarize completed phase I trials and ongoing phase II trials of immunotherapies against α-synuclein and tau and further discuss concerns and hurdles to overcome in the future.
Progressive Supranuclear Palsy with Predominant Cerebellar Ataxia
Shoichiro Ando, Masato Kanazawa, Osamu Onodera
J Mov Disord. 2020;13(1):20-26.   Published online December 19, 2019
DOI: https://doi.org/10.14802/jmd.19061
  • 6,026 View
  • 332 Download
  • 10 Citations
AbstractAbstract PDF
Progressive supranuclear palsy (PSP) is characterized by supranuclear gaze palsy, dystonic rigidity of the neck and upper trunk, frequent falls and mild cognitive impairment. Cerebellar ataxia is one of the exclusion criteria given by the National Institute of Neurological Disorders and Stroke and the Society for Progressive Supranuclear Palsy. As a result, pathologically proven PSP patients exhibiting cerebellar ataxia have often been misdiagnosed with spinocerebellar degeneration, specifically multiple system atrophy with predominant cerebellar ataxia (MSA-C). However, more recently, it has been recognized that patients with PSP can present with truncal and limb ataxia as their initial symptom and/or main manifestation. These patients can be classified as having PSP with predominant cerebellar ataxia (PSP-C), a new subtype of PSP. Since the development of this classification, patients with PSP-C have been identified primarily in Asian countries, and it has been noted that this condition is very rare in Western communities. Furthermore, the clinical features of PSP-C have been identified, enabling it to be distinguished from other subtypes of PSP and MSA-C. In this review, we describe the clinical and neuropathological features of PSP-C. The hypothesized pathophysiology of cerebellar ataxia in PSP-C is also discussed.
Principles of Electrophysiological Assessments for Movement Disorders
Kai-Hsiang Stanley Chen, Robert Chen
J Mov Disord. 2020;13(1):27-38.   Published online January 31, 2020
DOI: https://doi.org/10.14802/jmd.19064
  • 8,736 View
  • 796 Download
  • 21 Citations
AbstractAbstract PDFSupplementary Material
Electrophysiological studies can provide objective and quantifiable assessments of movement disorders. They are useful in the diagnosis of hyperkinetic movement disorders, particularly tremors and myoclonus. The most commonly used measures are surface electromyography (sEMG), electroencephalography (EEG) and accelerometry. Frequency and coherence analyses of sEMG signals may reveal the nature of tremors and the source of the tremors. The effects of voluntary tapping, ballistic movements and weighting of the limbs can help to distinguish between organic and functional tremors. The presence of Bereitschafts-potentials and beta-band desynchronization recorded by EEG before movement onset provide strong evidence for functional movement disorders. EMG burst durations, distributions and muscle recruitment orders may identify and classify myoclonus to cortical, subcortical or spinal origins and help in the diagnosis of functional myoclonus. Organic and functional cervical dystonia can potentially be distinguished by EMG power spectral analysis. Several reflex circuits, such as the long latency reflex, blink reflex and startle reflex, can be elicited with different types of external stimuli and are useful in the assessment of myoclonus, excessive startle and stiff person syndrome. However, limitations of the tests should be recognized, and the results should be interpreted together with clinical observations.
Original Articles
Automated Brainstem Segmentation Detects Differential Involvement in Atypical Parkinsonian Syndromes
Martina Bocchetta, Juan Eugenio Iglesias, Viorica Chelban, Edwin Jabbari, Ruth Lamb, Lucy L. Russell, Caroline V. Greaves, Mollie Neason, David M. Cash, David L. Thomas, Jason D. Warren, John Woodside, Henry Houlden, Huw R. Morris, Jonathan D. Rohrer
J Mov Disord. 2020;13(1):39-46.   Published online September 26, 2019
DOI: https://doi.org/10.14802/jmd.19030
  • 4,319 View
  • 200 Download
  • 8 Citations
AbstractAbstract PDFSupplementary Material
Objective
Brainstem segmentation has been useful in identifying potential imaging biomarkers for diagnosis and progression in atypical parkinsonian syndromes (APS). However, the majority of work has been performed using manual segmentation, which is time consuming for large cohorts.
Methods
We investigated brainstem involvement in APS using an automated method. We measured the volume of the medulla, pons, superior cerebellar peduncle (SCP) and midbrain from T1-weighted MRIs in 67 patients and 42 controls. Diagnoses were corticobasal syndrome (CBS, n = 14), multiple system atrophy (MSA, n = 16: 8 with parkinsonian syndrome, MSA-P; 8 with cerebellar syndrome, MSA-C), progressive supranuclear palsy with a Richardson’s syndrome (PSP-RS, n = 12), variant PSP (n = 18), and APS not otherwise specified (APS-NOS, n = 7).
Results
All brainstem regions were smaller in MSA-C (19–42% volume difference, p < 0.0005) and in both PSP groups (18–33%, p < 0.0005) than in controls. MSA-P showed lower volumes in all regions except the SCP (15–26%, p < 0.0005). The most affected region in MSA-C and MSA-P was the pons (42% and 26%, respectively), while the most affected regions in both the PSP-RS and variant PSP groups were the SCP (33% and 23%, respectively) and midbrain (26% and 24%, respectively). The brainstem was less affected in CBS, but nonetheless, the pons (14%, p < 0.0005), midbrain (14%, p < 0.0005) and medulla (10%, p = 0.001) were significantly smaller in CBS than in controls. The brainstem was unaffected in APS-NOS.
Conclusion
Automated methods can accurately quantify the involvement of brainstem structures in APS. This will be important in future trials with large patient numbers where manual segmentation is unfeasible.
Comparison of Spontaneous Motor Tempo during Finger Tapping, Toe Tapping and Stepping on the Spot in People with and without Parkinson’s Disease
Dawn Rose, Daniel J. Cameron, Peter J. Lovatt, Jessica A. Grahn, Lucy E. Annett
J Mov Disord. 2020;13(1):47-56.   Published online January 31, 2020
DOI: https://doi.org/10.14802/jmd.19043
  • 10,132 View
  • 133 Download
  • 7 Citations
AbstractAbstract PDFSupplementary Material
Objective
Spontaneous motor tempo (SMT), observed in walking, tapping and clapping, tends to occur around 2 Hz. Initiating and controlling movement can be difficult for people with Parkinson’s (PWP), but studies have not identified whether PWP differ from controls in SMT. For community-based interventions, e.g. dancing, it would be helpful to know a baseline SMT to optimize the tempi of cued activities. Therefore, this study compared finger tapping (FT), toe tapping (TT) and stepping ‘on the spot’ (SS) in PWP and two groups of healthy controls [age-matched controls (AMC) and young healthy controls (YHC)], as SMT is known to change with age.
Methods
Participants (PWP; n = 30, AMC; n = 23, YHC; n = 35) were asked to tap or step on the spot at a natural pace for two trials lasting 40 seconds. The central 30 seconds were averaged for analyses using mean inter-onset intervals (IOI) and coefficient of variation (CoV) to measure rate and variability respectively.
Results
PWP had faster SMT than both control groups, depending on the movement modality: FT, F(2, 87) = 7.92, p < 0.01 (PWP faster than YHC); TT, F(2, 87) = 4.89, p = 0.01 (PWP faster than AMC); and SS, F(2, 77) = 3.26, p = 0.04 (PWP faster than AMC). PWP had higher CoV (more variable tapping) than AMC in FT only, F(2, 87) = 4.10, p = 0.02.
Conclusion
This study provides the first direct comparison of SMT between PWP and two control groups for different types of movements. Results suggest SMT is generally faster in PWP than control groups, and more variable when measured with finger tapping compared to stepping on the spot.
Brief communication
Rescue Levodopa/Carbidopa Intestinal Gel for Secondary Deep Brain Stimulation Failure
Juan Miguel Pilar Bautista, Genko Oyama, Maierdanjiang Nuermaimaiti, Satoko Sekimoto, Fuyuko Sasaki, Taku Hatano, Kenya Nishioka, Masanobu Ito, Atsushi Umemura, Yuji Ishibashi, Yasushi Shimo, Nobutaka Hattori
J Mov Disord. 2020;13(1):57-61.   Published online January 31, 2020
DOI: https://doi.org/10.14802/jmd.19051
  • 4,000 View
  • 132 Download
  • 5 Citations
AbstractAbstract PDF
Objective
The long-term efficacy of deep brain stimulation (DBS) for motor fluctuations in advanced Parkinson’s disease (PD) has been well established; however, motor fluctuations may recur over time despite multiple adjustments of DBS settings and medications.
Methods
We conducted a retrospective chart review of three patients for whom levodopa-carbidopa intestinal gel (LCIG) was additionally administered as a rescue therapy for secondary DBS failure due to the recurrence of motor fluctuations.
Results
The three patients had advanced PD with a disease duration of 14–19 years, and had undergone DBS for motor fluctuations refractory to standard medical management. LCIG was administered to the patients because of symptom recurrence years after DBS and provided complementary effects in all patients.
Conclusion
The cases presented here show that rescue LCIG therapy may be a complementary treatment option for patients with post-DBS advanced PD who have a recurrence of troublesome motor complications.
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
  • 3,218 View
  • 111 Download
  • 1 Citations
AbstractAbstract PDFSupplementary 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.
Letters to the editor
A Patient with Neuroferritinopathy Presenting with Juvenile-Onset Voice Tremor
Chan Wook Park, Nan Young Kim, Yun Joong Kim, Sook Keun Song, Chul Hyoung Lyoo
J Mov Disord. 2020;13(1):66-68.   Published online August 9, 2019
DOI: https://doi.org/10.14802/jmd.19038
  • 4,769 View
  • 87 Download
  • 3 Citations
PDF
Unilateral Pseudo-Orthostatic Tremor Provoked by a Remote Limb Movement in Parkinson’s Disease
Sang-Won Yoo, Youngje Heo, Joong-Seok Kim, Kwang-Soo Lee
J Mov Disord. 2020;13(1):69-71.   Published online November 8, 2019
DOI: https://doi.org/10.14802/jmd.19056
  • 3,319 View
  • 88 Download
  • 1 Citations
PDFSupplementary Material
Combined Hemichorea and Seizures in a Patient with Nonketotic Hyperglycemia
Ryul Kim, Hee-Jin Cho, Ho-Won Lee, Jin-Sun Jun
J Mov Disord. 2020;13(1):72-73.   Published online November 8, 2019
DOI: https://doi.org/10.14802/jmd.19058
  • 3,736 View
  • 116 Download
  • 3 Citations
PDFSupplementary Material
Brainstem-Predominant Lewy-Related Pathology in a Patient with Parkinson’s Disease without Dementia
Ji-Hyun Choi, Sung-Hye Park, Sung Sup Park, Beomseok Jeon
J Mov Disord. 2020;13(1):74-76.   Published online December 19, 2019
DOI: https://doi.org/10.14802/jmd.19059
  • 2,972 View
  • 87 Download
  • 1 Citations
PDFSupplementary Material
Erratum
Modeling α-Synuclein Propagation with Preformed Fibril Injections
Hyun Kyung Chung, Hoang-Anh Ho, Dayana Pérez-Acuña, Seung-Jae Lee
J Mov Disord. 2020;13(1):77-79.   Published online October 14, 2019
DOI: https://doi.org/10.14802/jmd.19046.e
Corrects: J Mov Disord 2019;12(3):139
  • 3,353 View
  • 105 Download
  • 3 Citations
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JMD : Journal of Movement Disorders