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Volume 11(2); May 2018
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Review Article
Parkinson’s Disease in Sub-Saharan Africa: A Review of Epidemiology, Genetics and Access to Care
Uduak Williams, Oliver Bandmann, Richard Walker
J Mov Disord. 2018;11(2):53-64.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.17028
  • 9,864 View
  • 185 Download
  • 25 Citations
AbstractAbstract PDF
A low prevalence of Parkinson’s disease (PD) has been reported in the Sub-Saharan Africa (SSA) region. The genetic causes and clinical features of PD in this region have been poorly described. Very few reports have examined the availability and access to evidence-based quality care for people living with PD in this region. We reviewed all publications focusing on idiopathic PD from SSA published up to May 2016 and observed a prevalence of PD ranging from 7/100,000 in Ethiopia to 67/100,000 in Nigeria. The most recent community-based study reported a mean age at onset of 69.4 years. The infrequent occurrence of mutations in established PD genes was also observed in the region. Treatments were non-existent or at best irregular. Additionally, there is a lack of well-trained medical personnel and multidisciplinary teams in most countries in this region. Drugs for treating PD are either not available or unaffordable. Large-scale genetic and epidemiological studies are therefore needed in SSA to provide further insights into the roles of genetics and other etiological factors in the pathogenesis of PD. The quality of care also requires urgent improvement to meet the basic level of care required by PD patients.
Original Articles
Amantadine and the Risk of Dyskinesia in Patients with Early Parkinson’s Disease: An Open-Label, Pragmatic Trial
Aryun Kim, Young Eun Kim, Ji Young Yun, Han-Joon Kim, Hui-Jun Yang, Woong-Woo Lee, Chae Won Shin, Hyeyoung Park, Yu Jin Jung, Ahro Kim, Yoon Kim, Mihee Jang, Beomseok Jeon
J Mov Disord. 2018;11(2):65-71.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.18005
  • 6,953 View
  • 217 Download
  • 12 Citations
AbstractAbstract PDFSupplementary Material
Objective
We examined whether amantadine can prevent the development of dyskinesia.
Methods
Patients with drug-naïve Parkinson’s disease (PD), younger than 70 years of age and in the early stage of PD (Hoehn and Yahr scale < 3), were recruited from April 2011 to December 2014. The exclusion criteria included the previous use of antiparkinsonian medication, the presence of dyskinesia, significant psychological disorders, and previous history of a hypersensitivity reaction. Patients were consecutively assigned to one of 3 treatment groups in an open label fashion: Group A-1, amantadine first and then levodopa when needed; Group A-2, amantadine first, dopamine agonist when needed, and then levodopa; and Group B, dopamine agonist first and then levodopa when needed. The primary endpoint was the development of dyskinesia, which was analyzed by the Kaplan-Meier survival rate.
Results
A total of 80 patients were enrolled: Group A-1 (n = 27), Group A-2 (n = 27), and Group B (n = 26). Twenty-four patients were excluded from the analysis due to the following: withdrawal of amantadine or dopamine agonist (n = 9), alternative diagnosis (n = 2), withdrawal of consent (n = 1), and breach in the protocol (n = 12). After exclusion, 5 of the 56 (8.93%) patients developed dyskinesia. Patients in Group A-1 and A-2 tended to develop dyskinesia less often than those in Group B (cumulative survival rates of 0.933, 0.929, and 0.700 for A-1, A-2, and B, respectively; p = 0.453).
Conclusion
Amantadine as an initial treatment may decrease the incidence of dyskinesia in patients with drug-naïve PD.
Prospective Characterization of Cognitive Function in Typical and ‘Brainstem Predominant’Progressive Supranuclear Palsy Phenotypes
Young-Eun C Lee, David R Williams, Jacqueline F I Anderson
J Mov Disord. 2018;11(2):72-77.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.17067
  • 5,880 View
  • 105 Download
  • 6 Citations
AbstractAbstract PDF
Objective
Clinicopathological studies over the last decade have broadened the clinical spectrum of progressive supranuclear palsy (PSP) to include several distinct clinical syndromes. We examined the cognitive profiles of patients with PSP-Richardson’s syndrome (PSP-RS) and two atypical ‘brainstem predominant’ PSP phenotypes (PSP-parkinsonism, PSP-P; and PSP-pure akinesia with gait freezing, PSP-PAGF) using a comprehensive neuropsychological battery.
Methods
Fourteen patients diagnosed as PSP-RS, three patients with PSP-P and four patients with PSP-PAGF were assessed using a comprehensive battery of neuropsychological tests.
Results
The typical PSP-RS subgroup demonstrated greater impairments in processing speed [t(19) = -4.10, p = 0.001 (d =1.66)] and executive function [t(19) = -2.63, p = 0.02 (d = 1.20)] compared to the ‘brainstem predominant’ PSP phenotype.
Conclusion
This is the first prospective study to demonstrate that PSP-RS and ‘brainstem predominant’ PSP phenotypes can be differentiated on cognitive grounds. These differences correspond with variations in pathological profiles reported in the literature.
Oromandibular Dystonia: Demographics and Clinical Data from 240 Patients
Linda Slaim, Myriam Cohen, Patrick Klap, Marie Vidailhet, Alain Perrin, Daniel Brasnu, Denis Ayache, Marie Mailly
J Mov Disord. 2018;11(2):78-81.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.17065
  • 5,351 View
  • 198 Download
  • 21 Citations
AbstractAbstract PDF
Objective
To report demographic data from a large cohort of patients with oromandibular dystonia (OMD).
Methods
This is a retrospective review of patients with OMD referred to our institution between 1989 and 2015. Demographic (age of onset, gender, and familial history of dystonia) and clinical (type of OMD, associated dystonia, and etiology of dystonia) data were collected from a cohort of 240 individuals.
Results
The mean age of onset of OMD was 51.6 years old, with a female predominance (2:1). A family history of dystonia was found in 6 patients (2.5%). One hundred and forty-nine patients (62.1%) had the jaw-opening type of OMD, 48 patients (20.0%) had the jaw-closing type, and 43 patients (17.9%) had a mixed form of OMD. Lingual dystonia was also present in 64 (26.7%) of these patients. Eighty-two patients (34.2%) had a focal dystonia, 131 patients (54.6%) had a segmental dystonia, and 27 patients (11.3%) had a generalized dystonia. One hundred and seventy-one patients (71.3%) had idiopathic OMD.
Conclusion
OMD is a chronic and disabling focal dystonia. Our study found a prevalence of female patients, an onset in middle age and a predominantly idiopathic etiology. Unlike other studies, jaw-opening was found to be the most frequent clinical type of OMD.
Case Reports
Transcranial Direct Current Stimulation Combined with Action Observation and Electromyographic Biofeedback Training in a Patient with Writer’s Cramp
Yohei Okada, Chiharu Shibamoto, Yukari Osumi, Chihiro Asano, Riho Takeuchi, Sachio Nabeshima, Shu Morioka, Koji Shomoto
J Mov Disord. 2018;11(2):82-86.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.18007
  • 7,011 View
  • 78 Download
  • 4 Citations
AbstractAbstract PDFSupplementary Material
We present a 47-year-old right-handed woman with a 15-year history of writer’s cramp who was provided with six sessions of cathodal transcranial direct current stimulation (tDCS) combined with observation of writing actions performed by a healthy subject and electromyographic (EMG) biofeedback training to decrease EMG activities in her right forehand muscles while writing for 30 min for 4 weeks. She showed improvement in dystonic posture and writing speed after the intervention. The writing movement and writing speed scores on a writer’s cramp rating scale decreased, along with writing time. Our findings demonstrated that cathodal tDCS combined with action observation and EMG biofeedback training might improve dystonic writing movements in a patient with writer’s cramp.
Purposeless Groaning in Parkinson’s Disease
Shen-Yang Lim, Ai Huey Tan, Jia Lun Lim, Azlina Ahmad-Annuar
J Mov Disord. 2018;11(2):87-88.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.18004
  • 5,878 View
  • 101 Download
  • 4 Citations
AbstractAbstract PDFSupplementary Material
Purposeless groaning has been reported in advanced progressive supranuclear palsy. We present a case of purposeless groaning occurring as a primary complaint in a patient with advanced Parkinson’s disease. Purposeless groaning is thought to be a manifestation of disinhibition and perseveration due to frontal-subcortical dysfunction. Proper recognition of this phenomenon will help clinicians to avoid unnecessary investigations and treatment (e.g., prescription of opioid medications).
A Patient with Beta-Propeller Protein-Associated Neurodegeneration: Treatment with Iron Chelation Therapy
Shen-Yang Lim, Ai Huey Tan, Azlina Ahmad-Annuar, Susanne A. Schneider, Ping Chong Bee, Jia Lun Lim, Norlisah Ramli, Mohamad Imran Idris
J Mov Disord. 2018;11(2):89-92.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.17082
  • 23,759 View
  • 142 Download
  • 9 Citations
AbstractAbstract PDFSupplementary Material
We present a case of beta-propeller protein-associated neurodegeneration, a form of neurodegeneration with brain iron accumulation. The patient harbored a novel mutation in the WDR45 gene. A detailed video and description of her clinical condition are provided. Her movement disorder phenomenology was characterized primarily by limb stereotypies and gait dyspraxia. The patient’s disability was advanced by the time iron-chelating therapy with deferiprone was initiated, and no clinical response in terms of cognitive function, behavior, speech, or movements were observed after one year of treatment.

JMD : Journal of Movement Disorders