Publisher: BMC (Biomed Central)   (Total: 316 journals)

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Showing 201 - 316 of 316 Journals sorted by number of followers
Fisheries and Aquatic Sciences     Open Access   (Followers: 3, SJR: 0.199, CiteScore: 0)
Cancers of the Head & Neck     Open Access   (Followers: 3)
Clinical and Translational Allergy     Open Access   (Followers: 3, SJR: 1.425, CiteScore: 4)
J. of Clinical Movement Disorders     Open Access   (Followers: 3)
Advances in Rheumatology     Open Access   (Followers: 3)
J. of Animal Science and Technology     Open Access   (Followers: 3)
Flavour     Open Access   (Followers: 3)
Biomarker Research     Open Access   (Followers: 3)
Cancer Imaging     Open Access   (Followers: 3, SJR: 1.012, CiteScore: 3)
Inflammation and Regeneration     Open Access   (Followers: 2)
Asthma Research and Practice     Open Access   (Followers: 2)
Public Health Reviews     Open Access   (Followers: 2, SJR: 0.454, CiteScore: 1)
Intl. J. of Retina and Vitreous     Open Access   (Followers: 2)
Biology of Sex Differences     Open Access   (Followers: 2, SJR: 1.902, CiteScore: 4)
Tropical Diseases, Travel Medicine and Vaccines     Open Access   (Followers: 2)
J. of Angiogenesis Research     Open Access   (Followers: 2)
Animal Biotelemetry     Open Access   (Followers: 2, SJR: 1.067, CiteScore: 2)
BMC Materials     Open Access   (Followers: 2)
Urban Transformations     Open Access   (Followers: 2)
CABI Agriculture and Bioscience     Open Access   (Followers: 2)
J. of Biomedical Semantics     Open Access   (Followers: 2, SJR: 0.952, CiteScore: 2)
One Health Outlook     Open Access   (Followers: 2)
NeuroCommons     Open Access   (Followers: 2)
Signals     Open Access   (Followers: 2)
Contraception and Reproductive Medicine     Open Access   (Followers: 2)
Fertility Research and Practice     Open Access   (Followers: 2)
Chinese Medicine     Open Access   (Followers: 2, SJR: 0.57, CiteScore: 2)
J. of Inflammation     Open Access   (Followers: 2, SJR: 1.101, CiteScore: 3)
Molecular Neurodegeneration     Open Access   (Followers: 2, SJR: 3.418, CiteScore: 7)
Fluids and Barriers of the CNS     Open Access   (Followers: 2, SJR: 2.054, CiteScore: 5)
Cell Communication and Signaling     Open Access   (Followers: 2, SJR: 2.211, CiteScore: 4)
BMC Pharmacology     Open Access   (Followers: 2)
Cancer Nanotechnology     Open Access   (Followers: 2, SJR: 1.168, CiteScore: 4)
Neural Development     Open Access   (Followers: 2, SJR: 1.821, CiteScore: 2)
J. of Neuroinflammation     Open Access   (Followers: 2, SJR: 2.336, CiteScore: 5)
J. of Experimental & Clinical Cancer Research     Open Access   (Followers: 2, SJR: 2, CiteScore: 6)
Particle and Fibre Toxicology     Open Access   (Followers: 2, SJR: 2.253, CiteScore: 8)
Hereditary Cancer in Clinical Practice     Open Access   (Followers: 2, SJR: 0.848, CiteScore: 2)
BMC Proceedings     Full-text available via subscription   (Followers: 2, SJR: 0.302, CiteScore: 1)
Italian J. of Pediatrics     Open Access   (Followers: 2, SJR: 0.685, CiteScore: 2)
Reproductive Health     Open Access   (Followers: 2, SJR: 1.228, CiteScore: 2)
Plant Methods     Open Access   (Followers: 2, SJR: 1.885, CiteScore: 4)
Acta Neuropathologica Communications     Open Access   (Followers: 1, SJR: 2.683, CiteScore: 5)
Canine Genetics and Epidemiology     Open Access   (Followers: 1)
Cerebellum & Ataxias     Open Access   (Followers: 1)
Infectious Diseases of Poverty     Open Access   (Followers: 1, SJR: 1.212, CiteScore: 3)
Longevity & Healthspan     Open Access   (Followers: 1)
J. of Environmental Health Science & Engineering     Open Access   (Followers: 1, SJR: 0.802, CiteScore: 3)
Translational Neurodegeneration     Open Access   (Followers: 1, SJR: 1.901, CiteScore: 5)
Eye and Vision     Open Access   (Followers: 1)
Cancer Convergence     Open Access   (Followers: 1)
BMC Zoology     Open Access   (Followers: 1)
Bioelectronic Medicine     Open Access   (Followers: 1)
European J. of Medical Research     Open Access   (Followers: 1, SJR: 0.55, CiteScore: 1)
Scoliosis and Spinal Disorders     Open Access   (Followers: 1, SJR: 0.843, CiteScore: 2)
Women's Midlife Health     Open Access   (Followers: 1)
COPD Research and Practice     Open Access   (Followers: 1, SJR: 0.755, CiteScore: 2)
Genes and Environment     Open Access   (Followers: 1, SJR: 0.516, CiteScore: 1)
Gynecologic Oncology Research and Practice     Open Access   (Followers: 1)
Pilot and Feasibility Studies     Open Access   (Followers: 1)
Investigative Genetics     Open Access   (Followers: 1, SJR: 1.809, CiteScore: 3)
J. of Venomous Animals and Toxins including Tropical Diseases     Open Access   (Followers: 1, SJR: 0.573, CiteScore: 2)
Orphanet J. of Rare Diseases     Open Access   (Followers: 1, SJR: 1.413, CiteScore: 3)
Skeletal Muscle     Open Access   (Followers: 1, SJR: 2.32, CiteScore: 4)
J. of Medical Case Reports     Open Access   (Followers: 1, SJR: 0.331, CiteScore: 1)
Head & Face Medicine     Open Access   (Followers: 1, SJR: 0.62, CiteScore: 2)
BMC Ear, Nose and Throat Disorders     Open Access   (Followers: 1, SJR: 0.653, CiteScore: 2)
Cell Division     Open Access   (Followers: 1, SJR: 2.445, CiteScore: 4)
Respiratory Research     Open Access   (Followers: 1, SJR: 1.644, CiteScore: 4)
Proteome Science     Open Access   (Followers: 1, SJR: 0.792, CiteScore: 2)
Theoretical Biology and Medical Modelling     Open Access   (Followers: 1, SJR: 0.783, CiteScore: 2)
Biological Research     Open Access   (Followers: 1, SJR: 0.654, CiteScore: 2)
Hereditas     Open Access   (Followers: 1, SJR: 0.278, CiteScore: 1)
Thyroid Research     Open Access   (Followers: 1, SJR: 0.329, CiteScore: 1)
World Allergy Organization J.     Open Access   (Followers: 1, SJR: 1.936, CiteScore: 6)
World J. of Surgical Oncology     Open Access   (Followers: 1, SJR: 0.688, CiteScore: 2)
J. of Cardiovascular Magnetic Resonance     Open Access   (Followers: 1, SJR: 2.292, CiteScore: 5)
Molecular Cytogenetics     Open Access   (Followers: 1, SJR: 0.623, CiteScore: 1)
Measurement Instruments for the Social Sciences     Open Access  
BMC Energy     Open Access  
Sustainable Earth     Open Access  
BMC Biomedical Engineering     Open Access  
BMC Chemical Engineering     Open Access  
ExRNA     Open Access  
J. of Cotton Research     Open Access  
Biomedical Dermatology     Open Access  
Cancer Communications     Open Access  
Diagnostic and Prognostic Research     Open Access  
Porcine Health Management     Open Access  
Neurovascular Imaging     Open Access  
NeuroMetals     Open Access  
Chinese Neurosurgical J.     Open Access  
Cardio-Oncology     Open Access  
Neuropsychiatric Electrophysiology     Open Access  
Research Involvement and Engagement     Open Access  
J. of Biological Research - Thessaloniki     Open Access   (SJR: 0.32, CiteScore: 2)
J. of Therapeutic Ultrasound     Open Access   (SJR: 0.906, CiteScore: 3)
Cilia     Open Access   (SJR: 0.732, CiteScore: 1)
Israel J. of Health Policy Research     Open Access   (SJR: 0.488, CiteScore: 1)
Vascular Cell     Open Access   (SJR: 1.349, CiteScore: 4)
Clinical Sarcoma Research     Open Access  
Environmental Microbiome     Open Access   (SJR: 0.768, CiteScore: 2)
Mobile DNA     Open Access   (SJR: 3.783, CiteScore: 5)
J. of Neurodevelopmental Disorders     Open Access   (SJR: 1.71, CiteScore: 4)
Biological Procedures Online     Open Access   (SJR: 1.352, CiteScore: 4)
Basic and Clinical Andrology     Open Access   (SJR: 0.564, CiteScore: 2)
PMC Biophysics     Open Access  
Fibrogenesis & Tissue Repair     Open Access   (SJR: 1.531, CiteScore: 4)
J. of Ovarian Research     Open Access   (SJR: 1.008, CiteScore: 3)
Source Code for Biology and Medicine     Open Access   (SJR: 0.784, CiteScore: 2)
Retrovirology     Open Access   (SJR: 1.855, CiteScore: 3)
Lipids in Health and Disease     Open Access   (SJR: 0.915, CiteScore: 2)
J. of Negative Results in BioMedicine     Open Access   (SJR: 0.483, CiteScore: 1)
J. of Ethnobiology and Ethnomedicine     Open Access   (SJR: 0.693, CiteScore: 3)
Infectious Agents and Cancer     Open Access   (SJR: 0.855, CiteScore: 2)
Harm Reduction J.     Open Access   (SJR: 1.445, CiteScore: 3)

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Journal of Clinical Movement Disorders
Number of Followers: 3  

  This is an Open Access Journal Open Access journal
ISSN (Online) 2054-7072
Published by BMC (Biomed Central) Homepage  [316 journals]
  • AbobotulinumtoxinA using 2-mL dilution (500 U/2-mL) maintains durable
           improvement across multiple treatment cycles

    • Abstract: Background Cervical dystonia (CD), the most common focal dystonia, is a chronic neurological movement disorder characterized by sustained involuntary contractions of the neck muscles, leading to abnormal postures. AbobotulinumtoxinA (aboBoNT-A) was approved in the US initially as a 500 U per 1-mL dilution and subsequently, as a 500 U/2-mL dilution (or 250 U/mL), thereby providing clinicians with more flexible dosing options to better meet individual patient needs. The objective of this open-label extension study was to evaluate the longer term safety and efficacy of repeat treatments with aboBoNT-A using 2-mL dilutions in adults with cervical dystonia. Methods Patients (N = 112) from a 12-week, double-blind lead-in study (NCT01753310) received up to three additional treatments of aboBoNT-A, with re-treatment every 12–16 weeks based on clinical judgment. Safety was assessed through treatment-emergent adverse events (TEAEs). The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total and subscale scores were measured at day 1 of each treatment cycle (C), 4 weeks after each treatment, and 12 weeks after the third treatment. Descriptive statistics were used for all analyses. Results In cycles 1, 2, 3, and 4, respectively, 35.7, 25.9, 30.2, and 22.8% of patients reported TEAEs. Dysphagia, muscular weakness, and neck pain were each reported by 10.7% of patients, over the full study duration. Mean TWSTRS total score decreased from 37.7 (SD 13.6 [C1, day 1]) to 30.1 (SD 12.8 [C3, week 12]). In each cycle, TWSTRS total and subscale scores decreased from day 1 to week 4 and increased between weeks 4 and 12, though the week 12 scores remained lower than day 1 scores. Conclusion Extended treatment of cervical dystonia with aboBoNT-A (up to 3 additional treatment cycles) using a 2-mL dilution is effective, with a positive risk-benefit profile. Trial registration ClinicalTrials.gov Identifier: NCT01753336. Registered 17 Dec 2012.
      PubDate: 2020-08-31
       
  • Potential impact and challenges associated with Parkinson’s disease
           patient care amidst the COVID-19 global pandemic

    • Abstract: Background COVID-19 has made itself known to health care providers and families across the world in a matter of months. While primarily a respiratory disorder, it has also been shown to cause neurological symptoms, which can be a concern for Parkinson’s disease (PD) patients. Although PD is not as common as other conditions such as cardiovascular diseases, it affects millions of patients around the world whose care has been affected by the global pandemic. Objectives The aim of this review is to provide insight into the direct and indirect associations between COVID-19 and PD patient care. Results Potential direct effects of COVID-19 include possible neurodegeneration, concerns of symptom self-management with over-the-counter (OTC) products and ICU challenges that can arise in PD patients. In addition, a subset of PD patients may be at higher risk of severe COVID-19 infection. The indirect effects of the pandemic are associated with the social distancing measures and disruptions in health care systems and PD clinical trials, which may negatively affect PD patients’ mental wellbeing and create barriers in controlling their PD symptoms. On a more positive note, telemedical care is quickly emerging as a primary communication tool for virtual patient care. However, further research should be conducted to examine the applicability of telemedicine across the entire PD population, such as those with more severe symptoms living in less developed areas. With all the uncertainty during this time, it is hopeful to hear many promising COVID-19 treatments being researched, one of them being a PD drug therapy, amantadine. Conclusion Hopefully, we can consider this pandemic an opportunity to strengthen the PD community and learn more about the impact of the SARS-COV-2 virus. This review provides an overview of the interaction between COVID-19 and PD patients and future investigational retrospective studies are suggested to validate the observations.
      PubDate: 2020-08-08
       
  • Correction to: Economic evaluation of AbobotulinumtoxinA vs
           OnabotulinumtoxinA in real-life clinical management of cervical dystonia

    • Abstract: An amendment to this paper has been published and can be accessed via the original article.
      PubDate: 2020-07-29
       
  • Objective measurement in Parkinson’s disease: a descriptive analysis of
           Parkinson’s symptom scores from a large population of patients across
           the world using the Personal KinetiGraph®

    • Abstract: Background The Personal KinetiGraph® (PKG®) Movement Recording System provides continuous, objective, ambulatory movement data during routine daily activities and provides information on medication compliance, motor fluctuations, immobility, and tremor for patients with Parkinson’s disease (PD). Recent evidence has proposed targets for treatable symptoms. Indications for PKG vary by country and patient selection varies by physician. Methods The analyses were based upon 27,834 complete and de-identified PKGs from January 2012 to August 2018 used globally for routine clinical care. Median scores for bradykinesia (BKS) and dyskinesia (DKS) as well as percent time with tremor (PTT) and percent time immobile (PTI) were included as well as proportions of PKGs above published PKG summary score target values (BKS > 25, DKS > 9, PTT > 1%, PTI > 10%). Two sub-analyses included subjects who had 2+ PKG records and scores above proposed BKS and DKS targets, respectively, on their first PKG. Median BKS and DKS scores for subsequent PKGs (1st, 2nd, etc.) were summarized and limited to those with 100+ subsequent PKGs for each data point. Results Significant differences between countries were found for all 4 PKG parameter median scores (all p < 0.0001). Overall, 54% of BKS scores were > 25 and ranged from 46 to 61% by country. 10% of all DKS scores were > 9 and ranged from 5 to 15% by country. Sub-analysis for BKS showed global median BKS and DKS scores across subsequent PKGs for subjects who had 2+ PKGs and had BKS > 25 on their first PKG. There were significant changes in BKS from 1st to 2nd-6th PKGs (all p < 0.0001). Sub-analysis for DKS showed global median BKS & DKS scores across subsequent PKGs for subjects who had 2+ PKGs and had DKS > 9 on their first PKG. There were significant changes in DKS from 1st to 2nd and 3rd PKGs (both p < 0.0001). Conclusions This analysis shows that in every country evaluated a meaningful proportion of patients have sub-optimal PD motor symptoms and substantial variations exist across countries. Continuous objective measurement (COM) in routine care of PD enables identification and quantification of PD motor symptoms, which can be used to enhance clinical decision making, track symptoms over time and improve PD symptom scores. Thus, clinicians can use these PKG scores during routine clinical management to identify PD symptoms and work to move patients into a target range or a more controlled symptom state.
      PubDate: 2020-04-30
       
  • Quantification of tremor using consumer product accelerometry is feasible
           in patients with essential tremor and Parkinson’s disease: a comparative
           study

    • Abstract: Background To quantify pharmacological effects on tremor in patients with essential tremor (ET) or Parkinson’s Disease (PD), laboratory-grade accelerometers have previously been used. Over the last years, consumer products such as smartphones and smartwatches have been increasingly applied to measure tremor in an easy way. However, it is unknown how the technical performance of these consumer product accelerometers (CPAs) compares to laboratory-grade accelerometers (LGA). This study was performed to compare the technical performance of CPAs with LGA to measure tremor in patients with Parkinson’s Disease (PD) and essential tremor (ET). Methods In ten patients with PD and ten with ET, tremor peak frequency and corresponding amplitude were measured with 7 different CPAs (Apple iPhone 7, Apple iPod Touch 5, Apple watch 2, Huawei Nexus 6P, Huawei watch, mbientlabMetaWear (MW) watch, mbientlab MW clip) and compared to a LGA (Biometrics ACL300) in resting and extended arm position. Results Both in PD and ET patients, the peak frequency of CPAs did not significantly differ from the LGA in terms of limits of agreement. For the amplitude at peak frequency, only the iPhone and MW watch performed comparable to the LGA in ET patients, while in PD patients all methods performed comparable except for the iPod Touch and Huawei Nexus. Amplitude was higher when measured with distally-located CPAs (Clip, iPhone, iPod) compared with proximally-located CPAs (all watches). The variability between subjects was higher than within subjects for frequency (25.1% vs. 13.4%) and amplitude measurement (331% vs. 53.6%). Resting arm position resulted in lower intra-individual variability for frequency and amplitude (13.4 and 53.5%) compared to extended arm position (17.8 and 58.1%). Conclusions Peak frequencies of tremor could be measured with all tested CPAs, with similar performance as LGA. The amplitude measurements appeared to be driven by anatomical location of the device and can therefore not be compared. Our results show that the tested consumer products can be used for tremography, allowing at-home measurements, in particular in studies with a cross-over or intra-individual comparison design using the resting arm position. Trial registration This trial was registered in the Dutch Competent Authority (CCMO) database with number NL60672.058.17 on May 30th 2017.
      PubDate: 2020-04-07
       
  • Vitamin B12 measurements across neurodegenerative disorders

    • Abstract: Background Vitamin B12 deficiency causes a number of neurological features including cognitive and psychiatric disturbances, gait instability, neuropathy, and autonomic dysfunction. Clinical recognition of B12 deficiency in neurodegenerative disorders is more challenging because it causes defects that overlap with expected disease progression. We sought to determine whether B12 levels at the time of diagnosis in patients with Parkinson’s disease (PD) differed from those in patients with other neurodegenerative disorders. Methods We performed a cross-sectional analysis of B12 levels obtained around the time of diagnosis in patients with PD, Multiple System Atrophy (MSA), Dementia with Lewy Bodies (DLB), Alzheimer’s disease (AD), Progressive Supranuclear Palsy (PSP), Frontotemporal Dementia (FTD), or Mild Cognitive Impairment (MCI). We also evaluated the rate of B12 decline in PD, AD, and MCI. Results In multivariable analysis adjusted for age, sex, and B12 supplementation, we found that B12 levels were significantly lower at time of diagnosis in patients with PD than in patients with PSP, FTD, and DLB. In PD, AD, and MCI, the rate of B12 decline ranged from − 17 to − 47 pg/ml/year, much greater than that reported for the elderly population. Conclusions Further studies are needed to determine whether comorbid B12 deficiency affects progression of these disorders.
      PubDate: 2020-03-12
       
  • Economic evaluation of AbobotulinumtoxinA vs OnabotulinumtoxinA in
           real-life clinical management of cervical dystonia

    • Abstract: Background Botulinum neurotoxins type A (BoNT-As) are commonly used treatments for cervical dystonia (CD). Clinical trials have demonstrated the benefits of them in these patients, but data from real-life clinical practice as well as comparative data on the cost and outcome of different BoNT-A formulations are limited. The aim of this study was to compare abobotulinumtoxinA (aboBoNT-A) and onabotulinumtoxinA (onaBoNT-A) on their clinical outcomes and drug costs in real-life clinical practice. Methods This analysis included 356 adult patients with idiopathic CD treated with aboBoNT-A (n = 253) or onaBoNT-A (n = 103) from 38 centres across Europe and Australia (NCT00833196). The clinical outcome measures were treatment responses, changes in TWSTRS scores and changes in health utility scores from baseline to study visit 2 and 3. Health utility score was mapped from the TWSTRS total scale, using a previous publication. Costs included drug cost for France. Results The aboBoNT-A treated group had 2.06 (95% CI: 1.15 to 3.69) times higher odds of achieving treatment response than the onaBoNT-A treated group. The adjusted mean change in TWSTRS total score from baseline to visit 3 were − 6.42 (95% CI: − 7.52 to − 5.33) for aboBoNT-A and − 3.94 (95% CI: − 5.68 to − 2.2) for onaBoNT-A, with a difference of − 2.48 (95% CI: − 4.57 to − 0.39). The corresponding difference in the adjusted mean change for health utility score was 0.008 (95% CI: 0.001 to 0.014). Mean treatment costs for aboBoNT-A and onaBoNT-A were 314.1 (95% CI: 299.1 to 329.0) and 346.6 (95% CI: 322.9 to 370.4) Euros, respectively. Conclusions This comparative analysis indicated that treatment with aboBoNT-A may be less costly and lead to improved clinical outcomes when compared with onaBoNT-A, from a French healthcare system perspective. Additional comparative clinical data from larger patient cohorts, as well as more information about cost consequences of an improvement in clinical outcome would be of value to further confirm the findings.
      PubDate: 2020-02-11
       
  • Case study on the use of intensive pediatric neurorehabilitation in the
           treatment of kernicterus

    • Abstract: Background Kernicterus Spectrum Disorder (KSD) is the result of prolonged bilirubin toxicity resulting in widespread neurological injury. Once the bilirubin levels are normalized the encephalopathy becomes static, however the consequences of the injury can have life-long effects. The sequelae of KSD include motor impairments, auditory deficits, dental dysplasia, and potentially cognitive impairments. While KSD is a rare diagnosis, particularly in developed countries, there is evidence that there may be a global increase in incidence (Hansen, Semin Neonatol 7:103–9, 2002; Johnson, J Perinatol 29:S25–45, 2009; Kaplan etal. Neonatology 100:354–62, 2011; Maisels, Early Hum Dev 85:727–32, 2009; Olusanya etal., Arch Dis Child 99:1117–21, 2014; Steffensrud, Newborn Infant Nurs Rev 4:191–200, 2004). The literature on the treatment of various specific sequelae of KSD is varied, but in general specific therapeutic efforts to improve motor skills are not evidenced-based. The following is a case report on the use of Acquire therapy, an intensive neuromotor intervention, to ameliorate some of the motor-function deficits secondary to KSD. Case presentation This case-report presents the results of two intensive therapeutic intervention sessions in one male child with KSD. Treatments occurred at 28 and 34 months. The child presented with fine and gross motor deficits as well as communication delays. Each session consisted of daily therapy for 4 h each weekday for 3 weeks. The child was assessed before and after treatment with 2 standardized measures, the Gross Motor Function Measure (GMFM) and The Bayley Scales of Infant and Toddler Development (Bayley). Conclusions The GMFM at the 1st assessment was 34, 74at the 2nd assessment (after intervention 1), and 64 at the third assessment and 104 at the 4th assessment (after intervention 2). The Bayley at the 3rd assessment was 18, and 38 at the 4th assessment (after intervention 2).
      PubDate: 2020-02-03
       
  • A case of Gerstmann-Straussler-Scheinker (GSS) disease with supranuclear
           gaze palsy

    • Abstract: Background Gerstmann-Straussler-Scheinker disease (GSS), an autosomal dominant prion disorder, usually presents as a slowly progressive cerebellar ataxia followed by later cognitive decline. We present a member of the GSS Indiana Kindred with supranuclear palsy, a less common feature in GSS. Case presentation A 42-year-old man presented with 12 months of progressive gait and balance difficulty. Exam was notable for ataxia and cerebellar eye movement abnormalities. Genetic testing revealed a F198S variant in the prion protein (PRNP) gene, the pathological variant of GSS associated with his family, the Indiana kindred. Eighteen months after initial presentation supranuclear palsy developed. Conclusions GSS is a neurodegenerative prion disease with diverse clinical presentations, and exhibits greater variability in disease phenotype compared to other inherited spongiform encephalopathies. GSS should be on the differential for patients with ataxia and supranuclear palsy, and it is important to assess both horizontal and vertical saccades and optokinetic nystagmus in patients with ataxia.
      PubDate: 2019-12-11
       
  • “Feasibility and utility of a simple computerized test for measuring
           saccade latency in progressive supranuclear palsy- a proof-of-concept
           study”

    • Abstract: Background Reliable detection of slowed vertical saccades may help discriminate progressive supranuclear palsy (PSP) from the subset of Parkinson’s disease patients who lack tremor (akinetic-rigid or PD-postural instability and gait disorder PIGD subtype), and from age-related oculomotor changes. We investigated the feasibility of a camera-less computerized behavioral saccade latency paradigm previously validated in PD to discriminate probable PSP-Richardson syndrome (PSP-RS) from PD-PIGD and age-matched controls. Methods In this proof-of-concept case-control study, reflexive saccade latencies were measured in 5 subjects with probable PSP-RS, 5 subjects with PD-PIGD subtype, and 5 age-matched controls using the behavioral paradigm. The battery was repeated approximately one month later. All subjects were examined off levodopa by a movement disorders neurologist and by an ophthalmologist, who also performed a dilated eye exam. Results Vertical prosaccade latencies were longer in the PSP group (median = 903 ms) relative to PD (median = 268 ms) and control groups (median = 235 ms), with no overlap between groups (100% accuracy). PSP subjects also had larger vertical-horizontal discrepancies than comparison groups. Test-retest reliability for the behavioral saccade measures was good (interclass correlation coefficient = 0.948; 95% confidence interval [0.856, 0.982]), and the measures strongly correlated with clinical ratings. Conclusions Computerized behavioral measurement of reflexive saccade latency is feasible in PSP, and potentially discriminates probable PSP-RS from the PD-PIGD subtype. Findings from this proof-of-concept study support utility of the approach for obtaining objective saccade metrics in clinical evaluations and for tracking change in future, larger trials of moderately advanced PSP. Future studies should also examine the behavioral paradigm in earlier presentations of PSP and other subtypes of PSP.
      PubDate: 2019-12-06
       
  • Tongue involvement in embouchure dystonia: new piloting results using
           real-time MRI of trumpet players

    • Abstract: Background The embouchure of trumpet players is of utmost importance for tone production and quality of playing. It requires skilled coordination of lips, facial muscles, tongue, oral cavity, larynx and breathing and has to be maintained by steady practice. In rare cases, embouchure dystonia (EmD), a highly task specific movement disorder, may cause deterioration of sound quality and reduced control of tongue and lip movements. In order to better understand the pathophysiology of this movement disorder, we use real-time MRI to analyse differences in tongue movements between healthy trumpet players and professional players with embouchure dystonia. Methods Real-time MRI videos (with sound recording) were acquired at 55 frames per second, while 10 healthy subjects and 4 patients with EmD performed a defined set of exercises on an MRI-compatible trumpet inside a 3 Tesla MRI system. To allow for a comparison of tongue movements between players, temporal changes of MRI signal intensities were analysed along 7 standardized positions of the tongue using a customised MATLAB toolkit. Detailed results of movements within the oral cavity during performance of an ascending slurred 11-note harmonic series are presented. Results Playing trumpet in the higher register requires a very precise and stable narrowing of the free oral cavity. For this purpose the anterior section of the tongue is used as a valve in order to speed up airflow in a controlled manner. Conversely, the posterior part of the tongue is much less involved in the regulation of air speed. The results further demonstrate that healthy trumpet players control movements of the tongue rather precisely and stable during a sustained tone, whereas trumpet players with EmD exhibit much higher variability in tongue movements. Conclusion Control of the anterior tongue in trumpet playing emerges as a critical feature for regulating air speed and, ultimately, achieving a high-quality performance. In EmD the observation of less coordinated tongue movements suggests the presence of compensatory patterns in an attempt to regulate (or correct) pitch. Increased variability of the anterior tongue could be an objective sign of dystonia that has to be examined in further studies and extended to other brass instruments and may be also a potential target for therapy options.
      PubDate: 2019-11-12
       
  • Publisher Correction to: Journal of Clinical Movement Disorders, volume 6

    • Abstract: An error occurred during the publication of an article in Journal of Clinical Movement Disorders. This article was published in volume 6 with a duplicate citation number.
      PubDate: 2019-08-07
       
  • Huntington’s disease: a forensic risk factor in women

    • Abstract: Background Huntington’s disease (HD) is an autosomal dominant, neurodegenerative disorder. Associated cognitive deficits including impulsivity and disinhibition are the same factors that also predispose to forensic risk. Men tend to be perpetrators of more severe violent behaviours than women and women are less likely than men to be arrested for violence. This finding is not applicable in the case of women with Huntington’s disease and explored in the three clinical cases of women with HD and their forensic histories that are subsequently described. Case presentation ‘A’ was admitted from court following a charge of arson and reckless behavior, with increasing severity and frequency of self-harm and attempted suicide. This case demonstrates someone who had previously presented to psychiatric services on multiple occasions for various reasons, culminating in a serious criminal charge of arson due to psychiatric symptoms associated with HD. ‘B’ was arrested and imprisoned after having been charged with actual bodily harm (ABH) for assaulting her partner and young daughter then breaking her bail conditions. Although she was gene positive for HD she had no neurological symptoms of the disease. B was given leave but needed to be recalled to hospital by police. Six weeks later the medical recommendation for a court imposed hospital order was overturned as B presented and articulated her case so convincingly in court. This case demonstrates that even in the absence of psychiatric history or movement disorder there may be substantial forensic risk indicated by subtle underlying cognitive deficits due to changes in executive function affecting the frontal lobes. ‘C’ was admitted to acute psychiatric services after being found wandering in traffic wanting to die. She had been diagnosed with HD in the previous year and had a long criminal record on a background of alcohol dependency. Following transfer to a specialist psychiatric unit, she engaged well with a neurobehavioural levels system which rewards desirable and appropriate behaviours and she responded well to a highly structured environment resulting in discharge to a community placement. Conclusions These three case studies aim to highlight the need to raise awareness of the increased forensic risk in women with HD. Although criminal behaviour is less frequently observed in women than men and usually violence is less severe in women, HD may cause or contribute to criminal behaviour that can be violent in nature in women who are gene carriers for HD even in the absence of movement disorder, psychiatric symptoms or overt cognitive deficits. Assessment and earlier treatment in appropriate hospital settings may successfully contain and modify behaviours leading to reduced levels of risk and recidivism in this vulnerable patient group.
      PubDate: 2019-07-24
       
  • Telepsychiatry for patients with movement disorders: a feasibility and
           patient satisfaction study

    • Abstract: Background Telemedicine is a convenient health service delivery modality for patients with movement disorders, including Parkinson’s disease (PD), but is currently underutilized in the management of associated psychiatric symptoms. This study explored the feasibility of and patient satisfaction with telepsychiatry services at an academic movement disorders center. Methods All patients seen by telepsychiatry between January and December 2017 at the UCSF Movement Disorders and Neuromodulation Center were invited to participate. Participation was voluntary. Patients received an initial survey after the first telepsychiatry visit and satisfaction surveys after each visit. Survey responses were collected online via Research Electronic Data Capture (REDCap). Frequencies were calculated for categorical variables, and means and standard deviations were generated for continuous variables. Results Thirty-three patients (79% with PD; 72% Medicare recipients; 64% men; mean age, 61.1 ± 10.5 years; mean distance to clinic, 79.9 ± 81.3 miles) completed a total of 119 telepsychiatry and 62 in-person visits. Twenty-two initial surveys and 50 satisfaction surveys (from 21 patients) were collected. Patients were very satisfied with the care (95%), convenience (100%), comfort (95%), and overall visit (95%). Technical quality was somewhat lower rated, with 76% patients reporting they were very satisfied, while 19% were satisfied. All patients would recommend telemedicine to friends or family members. Conclusions Telepsychiatry is a feasible option for patients with movement disorders, leading to high patient satisfaction and improved access to care. Technical aspects still need optimization. Whenever available, telepsychiatry can be considered in addition to in-person visits. Future studies with larger samples should explore its impact on patient care outcomes and caregiver burden.
      PubDate: 2019-06-06
       
  • Transcranial magnetic stimulation therapy for focal leg dystonia: a case
           report

    • Abstract: Background Dystonia is a debilitating disease that causes abnormal, often repetitive, movements, postures or both. The pathophysiology is unknown but related to loss of neuronal inhibition, aberrant sensorimotor integration, and/or derangements of synaptic plasticity. Current treatments include pharmacotherapy, botulinum toxin injections and deep brain stimulation (DBS). The response to these treatments are often limited and carry the risk of side effects requiring alternative therapies such as non-invasive brain stimulation. Case presentation We present a case report of a 65-year -old man with refractory focal ‘task-specific’ dystonia. The treatment plan included 10-daily sessions of 1 Hz, 2600 pulses of repetitive transcranial magnetic stimulation (rTMS) targeting the primary motor cortex. Conclusion There were no clinical benefits noticed. Currently, there are no rTMS protocol treatments for dystonia. Publication of negative results will help in refining the optimal stimulation parameters, thus maximizing the effectiveness and reproducibility of future therapeutic protocols.
      PubDate: 2019-03-08
       
  • Correction to: Medical treatment of dystonia

    • Abstract: Following publication of the original article [1], the authors reported that the videos referred to in their article were not accessible to readers.
      PubDate: 2018-11-16
       
  • Characterization of vitamin D supplementation and clinical outcomes in a
           large cohort of early Parkinson’s disease

    • Abstract: Background Vitamin D (VitD) deficiency is common in Parkinson’s disease (PD) and has been raised as a possible PD risk factor. In the past decade, VitD supplementation for potential prevention of age related conditions has become more common. In this study, we sought to characterize VitD supplementation in early PD and determine as an exploratory analysis whether baseline characteristics or disease progression differed according to reported VitD use. Methods We analyzed data from the National Institutes of Health Exploratory Trials in Parkinson’s Disease (NET-PD) Long-term study (LS-1), a longitudinal study of 1741 participants. Subjects were divided into following supplement groups according to subject exposure (6 months prior to baseline and during the study): no VitD supplement, multivitamin (MVI), VitD ≥400 IU/day, and VitD + multivitamin (VitD+MVI). Clinical status was followed using the Unified Parkinson’s Disease Rating Scale, Symbol Digit Modalities Test, total daily levodopa equivalent dose, and Parkinson’s Disease Questionnaire. Results About 5% of subjects took VitD alone, 7% took VitD+MVI, 34% took MVI alone, while 54% took no supplement. Clinical outcomes at 3 years were similar across all groups. Conclusion This study shows VitD supplementation ≥400 IU/day was not common in early PD and that its use was similar to that seen in the US population. At 3 years, there was no difference in disease progression according to vitamin D supplement use.
      PubDate: 2018-10-31
       
  • SCA2 presenting as a focal dystonia

    • Abstract: Background Spinocerebellar ataxia 2 (SCA2) is an autosomal dominant neurodegenerative disorder caused by CAG repeat expansions in ATXN2 on chromosome 12q24. Patients present with adult-onset progressive gait ataxia, slow saccades, nystagmus, dysarthria and peripheral neuropathy. Dystonia is known to occur as SCA2 advances, but is rarely the presenting symptom. Case presentation A 43-year-old right handed woman presented with focal dystonia of the right hand which started two years earlier with difficulty writing. There were only mild cerebellar signs. Her mother was reported to have a progressive gait disorder and we subsequently learned that she had SCA2. A total of 10 maternal family members were similarly affected. Over the course of 10 years, the patient’s cerebellar signs progressed only mildly however the dystonia worsened to the extent of inability to use her right hand. Dystonia did not improve significantly with botulinum toxin, levodopa or trihexyphenidyl, but has shown marked improvement since DBS implantation in the GPi. Conclusions We describe a patient with SCA2 who presented with focal dystonia of the right upper extremity. Subtle cerebellar signs as well as the family history became especially important given the absence of predominant gait ataxia. Our case emphasizes that focal dystonia is not only a feature of SCA2, but can also rarely be the presenting sign as well as the most prominent feature during the disease course.
      PubDate: 2018-08-13
       
  • Inpatient care for stiff person syndrome in the United States: a
           nationwide readmission study

    • Abstract: Background Stiff person syndrome (SPS) is a progressive neurological disorder characterized by axial muscle rigidity and involuntary spasms. Autoimmune and neoplastic diseases are associated with SPS. Our study objectives were to describe inpatient care for SPS in the United States and characterize 30-day readmissions. Methods We queried the 2014 Nationwide Readmission Database for hospitalizations where a diagnosis of SPS was recorded. For readmission analyses, we excluded encounters with missing length of stay, hospitalization deaths, and out-of-state and December discharges. National estimates of index hospitalizations and 30-day readmissions were computed using survey weighting methods. Unconditional logistic regression was used to examine associations between demographic, clinical, and hospital characteristics and readmission. Results There were 836 patients with a recorded diagnosis of SPS during a 2014 hospitalization. After exclusions, 703 patients remained, 9.4% of which were readmitted within 30 days. Frequent reasons for index hospitalization were SPS (27.8%) and diabetes with complications (5.1%). Similarly, readmissions were predominantly for diabetes complications (24.2%) and SPS. Most readmissions attributed to diabetes complications (87.5%) were to different hospitals. Female sex (OR, 3.29; CI: 1.22–8.87) and routine discharge (OR, 0.26; CI: 0.10–0.64) were associated with readmission, while routine discharge (OR, 0.18; CI: 0.04–0.89) and care at for-profit hospitals (OR, 10.87; CI: 2.03–58.25) were associated with readmission to a different hospital. Conclusions Readmissions in SPS may result from disease complications or comorbid conditions. Readmissions to different hospitals may reflect specialty care, gaps in discharge planning, or medical emergencies. Studies are required to determine if readmissions in SPS are preventable.
      PubDate: 2018-08-06
       
  • Spectrum of practice in the routine management of cervical dystonia with
           abobotulinumtoxinA: findings from three prospective open-label
           observational studies

    • Abstract: Background Cervical dystonia is a heterogeneous disorder with several possible presentations, for which first-line therapy is often botulinum toxin (BoNT). In routine clinical practice the success of each BoNT injection is dependent on several variables, including individual presentation and injection technique. Large multicenter, observational studies provide important information on individualized administration strategies that cannot be otherwise ascertained from controlled clinical trials. In this meta-analysis of patient level data, we aimed to evaluate the clinical characteristics of patients with cervical dystonia undergoing routine treatment with botulinum toxin, specifically abobotulinumtoxinA. We also aimed to characterize current abobotulinumtoxinA injection techniques and parameters and to explore international differences in patient presentation and treatment. Methods This was a meta-analysis of baseline data from three prospective, international, multicenter, observational studies (NCT01314365, NCT00833196 and NCT01753349) of botulinum toxin treatment for the routine management of adult cervical dystonia. Results Data presented illustrate the significant heterogeneity of CD presentation in routine practice. Most subjects presented with a complex pattern of dystonic movements and the majority had additional components of shoulder elevation, tremor and/or jerk. Dosing was generally in accordance with that recommended in the abobotulinumtoxinA prescribing information, although the range of dosing also indicates that injections are tailored to individual presentation. Sub-group analyses at the country level revealed distinct differences in injection practice. Conclusions This meta-analysis is based on the largest dataset of subjects with cervical dystonia studied to date. The heterogeneity revealed in our baseline findings support the need to develop consistent, practical and comprehensive best practice guidelines.
      PubDate: 2018-07-09
       
 
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