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Prof. Joseph Jankovic
Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA

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0 Dystonia
0 TICs
0 botulinum toxin
0 hemifacial spasm
0 Tremor

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Journal article
Published: 24 August 2021 in Current Medical Research and Opinion
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Objective: Blepharospasm is a focal dystonia whereby excessive eyelid muscle contractions cause involuntary eye closure. Botulinum neurotoxin type A (BoNT-A) injections are an approved treatment. This randomized placebo-controlled trial (NCT01896895; EudraCT number 2012-004821-26) assessed the efficacy, safety, and treatment effect duration of incobotulinumtoxinA (Xeomin®, Merz Pharmaceuticals GmbH), a BoNT-A formulation without complexing proteins, in BoNT-A-naïve adults with blepharospasm. Methods: Subjects received incobotulinumtoxinA 50 U, 25 U (total dose) or placebo during a main study period (MP; 6–20 weeks). Patients needing a second injection received incobotulinumtoxinA ≤70 U in an open-label extension period (EP; 6–20 weeks). Treatment effect durations were time from first injection to EP injection or final MP visit and from EP injection to end-of-study visit. Times to effect onset and to waning of effect (MP) were time from injection to first subject-assessed onset effect and time from injection to subject-reported waning of effect, respectively. Results: Of 61 subjects, 39 entered the EP. During the MP, median duration of treatment effect was longer with incobotulinumtoxinA 50 U (20 weeks) versus incobotulinumtoxinA 25 U (11 weeks) or placebo (6 weeks). Median duration of treatment effect was 20 weeks during the EP. Median time to effect onset was 5, 7 and 14 days with 50 U, 25 U and placebo, respectively (p = 0.022 for 50 U versus placebo). Median time to waning of treatment effect was comparable between groups. Conclusion: Subjects reported an effect onset from 5 days after injection lasting up to 20 weeks (maximum observation period). Data indicate that incobotulinumtoxinA re-treatment of blepharospasm may not be required at fixed 12-week intervals and provide evidence for a patient-tailored approach. Blepharospasm is a condition in which involuntary contractions of the eyelid muscles cause the eye to close. The condition can be treated with botulinum neurotoxin type A (BoNT-A) injections. This study assessed the duration of effect and time to onset of effect for the BoNT-A formulation incobotulinumtoxinA (Xeomin®, Merz Pharmaceuticals GmbH) in adults with blepharospasm. Subjects received a single injection of one of two doses of incobotulinumtoxinA (total dose 25 or 50 U) or placebo and received a second injection of incobotulinumtoxinA only (total dose ≤70 U; the second injection was not compared with placebo) if needed 6–20 weeks after the first injection. After the first injection, the median (mid-point of values from all subjects) duration of treatment effect was longer with the higher incobotulinumtoxinA dose (20 weeks) than with the lower dose (11 weeks) and was longer with both incobotulinumtoxinA doses than with placebo (6 weeks). After the second incobotulinumtoxinA injection, the median duration of treatment effect was 20 weeks. The time to onset of effect was quicker with both incobotulinumtoxinA doses (5 and 7 days for the higher and lower doses, respectively) than with placebo (14 days) and the difference was statistically significant for the higher incobotulinumtoxinA dose compared with placebo. The time to waning of treatment effect was similar for the two incobotulinumtoxinA doses and placebo. This study shows that incobotulinumtoxinA is an effective treatment for blepharospasm, with a fast onset of action. In addition, the effects of one injection can last for up to 20 weeks.

ACS Style

Dimos D. Mitsikostas; Andrzej Dekundy; Angelika Hanschmann; Michael Althaus; Astrid Scheschonka; Fernando Pagan; Joseph Jankovic. Duration and onset of effect of incobotulinumtoxinA for the treatment of blepharospasm in botulinum toxin-naïve subjects. Current Medical Research and Opinion 2021, 1 -8.

AMA Style

Dimos D. Mitsikostas, Andrzej Dekundy, Angelika Hanschmann, Michael Althaus, Astrid Scheschonka, Fernando Pagan, Joseph Jankovic. Duration and onset of effect of incobotulinumtoxinA for the treatment of blepharospasm in botulinum toxin-naïve subjects. Current Medical Research and Opinion. 2021; ():1-8.

Chicago/Turabian Style

Dimos D. Mitsikostas; Andrzej Dekundy; Angelika Hanschmann; Michael Althaus; Astrid Scheschonka; Fernando Pagan; Joseph Jankovic. 2021. "Duration and onset of effect of incobotulinumtoxinA for the treatment of blepharospasm in botulinum toxin-naïve subjects." Current Medical Research and Opinion , no. : 1-8.

Review article
Published: 26 July 2021 in Frontiers in Neurology
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Tremor is the most commonly encountered movement disorder in clinical practice. A wide range of pathologies may manifest with tremor either as a presenting or predominant symptom. Considering the marked etiological and phenomenological heterogeneity, it would be desirable to develop a classification of tremors that reflects their underlying pathophysiology. The tremor task force of the International Parkinson Disease and Movement Disorders Society has worked toward this goal and proposed a new classification system. This system has remained a prime topic of scientific communications on tremor in recent times. The new classification is based on two axes: 1. based on the clinical features, history, and tremor characteristics and 2. based on the etiology of tremor. In this article, we discuss the key aspects of the new classification, review various tremor syndromes, highlight some of the controversies in the field of tremor, and share the potential future perspectives.

ACS Style

Abhishek Lenka; Joseph Jankovic. Tremor Syndromes: An Updated Review. Frontiers in Neurology 2021, 12, 1 .

AMA Style

Abhishek Lenka, Joseph Jankovic. Tremor Syndromes: An Updated Review. Frontiers in Neurology. 2021; 12 ():1.

Chicago/Turabian Style

Abhishek Lenka; Joseph Jankovic. 2021. "Tremor Syndromes: An Updated Review." Frontiers in Neurology 12, no. : 1.

Journal article
Published: 23 June 2021 in Tremor and Other Hyperkinetic Movements
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A defining characteristic of dystonia is its position-dependence. In cervical dystonia (CD), sensory tricks ameliorate head tremor (HT). But it remains unknown whether raising the arms alone has the same impact. We analyzed data collected from patients enrolled by the Dystonia Coalition. For 120 patients with HT, we assessed how raising their arms without touching their head changed their HT severity. Forty-eight out of 120 patients exhibited changes in HT severity when raising their arms. These patients were more likely to exhibit decreases in HT severity (N = 35) than increases (N = 13, χ2 (1, N = 48) = 10.1, p = 0.002). Demographic factors and sensory trick efficacy were not significant predictors of whether HT severity changed when raising their arms. Raising the arms without touching the head is a posture that can reduce HT severity in some CD patients. Our results extend the concept of position-dependent motor symptoms in CD to include the position of the arms. Head tremor (HT) is a prevalent symptom of cervical dystonia (CD) that can often be disabling. This study demonstrates that raising the arms without touching the head is a posture that can reduce HT severity in some CD patients. Our findings also identify a novel form of position-dependence in CD.

ACS Style

Elizabeth Cisneros; Jeanne P. Vu; Ha Yeon Lee; Qiyu Chen; Casey N. Benadof; Zheng Zhang; Emily A. Pettitt; Subhagya K. Joshi; Richard L. Barbano; Joseph Jankovic; Hyder A. Jinnah; Joel S. Perlmutter; Brian D. Berman; Abhimanyu Mahajan; Christopher G. Goetz; Glenn T. Stebbins; Cynthia L. Comella; David A. Peterson. Does Raising the Arms Modify Head Tremor Severity in Cervical Dystonia? Tremor and Other Hyperkinetic Movements 2021, 11, 1 .

AMA Style

Elizabeth Cisneros, Jeanne P. Vu, Ha Yeon Lee, Qiyu Chen, Casey N. Benadof, Zheng Zhang, Emily A. Pettitt, Subhagya K. Joshi, Richard L. Barbano, Joseph Jankovic, Hyder A. Jinnah, Joel S. Perlmutter, Brian D. Berman, Abhimanyu Mahajan, Christopher G. Goetz, Glenn T. Stebbins, Cynthia L. Comella, David A. Peterson. Does Raising the Arms Modify Head Tremor Severity in Cervical Dystonia? Tremor and Other Hyperkinetic Movements. 2021; 11 (1):1.

Chicago/Turabian Style

Elizabeth Cisneros; Jeanne P. Vu; Ha Yeon Lee; Qiyu Chen; Casey N. Benadof; Zheng Zhang; Emily A. Pettitt; Subhagya K. Joshi; Richard L. Barbano; Joseph Jankovic; Hyder A. Jinnah; Joel S. Perlmutter; Brian D. Berman; Abhimanyu Mahajan; Christopher G. Goetz; Glenn T. Stebbins; Cynthia L. Comella; David A. Peterson. 2021. "Does Raising the Arms Modify Head Tremor Severity in Cervical Dystonia?" Tremor and Other Hyperkinetic Movements 11, no. 1: 1.

Case series
Published: 14 April 2021 in Movement Disorders Clinical Practice
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Background Driving ability may be impaired in patients with various movement disorders, but it has not been studied in patients with Tourette syndrome (TS). Cases We describe a series of 6 patients from our large cohort of TS patients followed in our movement disorders clinic in whom severe tics have had interfered with their driving abilities. The motor tics involved facial muscles and caused visual impairment because of frequent blinking and transient blepharospasm (dystonic tic), but complex limb and truncal tics also seriously impacted their driving. Conclusions Although majority of patients with TS have no functional impairment, severe motor tics in some patients may adversely affect their driving ability, potentially causing danger to themselves and others. Screening for such troublesome tics should be considered in patients with TS, particularly in teenagers who are being evaluated for driver's licensing.

ACS Style

Karim Makhoul; Joseph Jankovic. Tourette Syndrome and Driving. Movement Disorders Clinical Practice 2021, 8, 763 -768.

AMA Style

Karim Makhoul, Joseph Jankovic. Tourette Syndrome and Driving. Movement Disorders Clinical Practice. 2021; 8 (5):763-768.

Chicago/Turabian Style

Karim Makhoul; Joseph Jankovic. 2021. "Tourette Syndrome and Driving." Movement Disorders Clinical Practice 8, no. 5: 763-768.

Journal article
Published: 31 March 2021 in Movement Disorders Clinical Practice
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Background Pimavanserin is a serotonin 2A receptor inverse agonist and antagonist used for the treatment of hallucinations and delusions in Parkinson's disease psychosis. Numerous studies support a modulatory role of serotonin in Tourette syndrome (TS). Objectives To determine whether or not pimavanserin affects TS symptoms. Methods In this open‐label study of TS adult patients, pimavanserin was titrated to 34 mg/day over 1 week and continued for an additional 7 weeks followed by a 2‐week washout. Tic severity, the primary outcome measure, was assessed by the Yale Global Tic Severity Scale Total Tic Severity score (YGTSS‐TTS). Secondary outcome measures included changes in the Yale‐Brown Obsessive Compulsive Scale (Y‐BOCS), the Tourette Syndrome Clinical Global Impression of Change (TS‐CGIC), the Tourette Syndrome‐Patient Global Impression of Impact (TS‐PGII), and the Gilles de la Tourette Syndrome – Quality of Life scale (GTS‐QOL). Results We enrolled 12 patients, but 2 dropped out after week 2 due to non‐serious side effects. In the 10 patients, mean (standard deviation (SD) age 34 (12.9) who completed the study the mean (SD) baseline YGTSS‐TTS was 34 (9.3). This decreased by 3.6 (4.9) points at week 8, a 12% reduction in tic severity (p = 0.03). This improvement is small and may not be clinically important. Significant improvement was noted in the TS‐CGIC, TS‐PGII and GTS‐QO. No serious adverse events were reported. Conclusions The results of this study suggest that pimavanserin is safe and associated with improvement in motor and non‐motor TS symptoms. These findings warrant further study by a larger, placebo‐controlled, trial.

ACS Style

Andrew Billnitzer; Joseph Jankovic. Pilot study to evaluate pimavanserin for the treatment of motor and behavioral symptoms of Tourette syndrome. Movement Disorders Clinical Practice 2021, 8, 694 -700.

AMA Style

Andrew Billnitzer, Joseph Jankovic. Pilot study to evaluate pimavanserin for the treatment of motor and behavioral symptoms of Tourette syndrome. Movement Disorders Clinical Practice. 2021; 8 (5):694-700.

Chicago/Turabian Style

Andrew Billnitzer; Joseph Jankovic. 2021. "Pilot study to evaluate pimavanserin for the treatment of motor and behavioral symptoms of Tourette syndrome." Movement Disorders Clinical Practice 8, no. 5: 694-700.

Journal article
Published: 29 March 2021 in Tremor and Other Hyperkinetic Movements
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Patients with essential tremor were initially considered to have isolated tremor, but additional motor and non-motor features have been increasingly recognized. The term “essential tremor plus” was adopted by the Task Force on Tremor of the International Parkinson and Movement Disorder Society to describe essential tremor patients with additional neurologic signs. To characterize essential tremor patients and their phenotypes in a movement disorders clinic population in the context of the new tremor classification. Demographic, clinical, historical, treatment, and diagnostic data were retrospectively collected on 300 patients diagnosed by movement disorder experts with essential tremor. Patients were classified as having essential tremor, essential tremor plus, or essential tremor-Parkinson’s disease combination, and features between these groups were compared. Of the 300 patients, 20.7% were classified as isolated essential tremor, 53.3% as essential tremor plus, and 26.0% as essential tremor-Parkinson’s disease. There was no significant difference in the duration of tremor symptoms. Essential tremor plus patients were more likely to have dystonia, tandem gait abnormalities, head tremor and greater tremor severity. Essential tremor-Parkinson’s disease patients were more likely to have RBD symptoms. There was no significant difference in cognitive impairment between essential tremor plus and essential tremor-Parkinson’s disease patients. Additional motor and non-motor features, including parkinsonism, are common in patients with essential tremor. Further studies are needed to clarify essential tremor phenotypes and to provide insights into possible subtypes. 300 patients with essential tremor from a movement disorders clinic were re-classified based on the Movement Disorder Society Consensus Statement on the Classification of Tremors. Additional motor and non-motor features, including parkinsonism, were common, and only 20.7% of patients remained classified as isolated essential tremor.

ACS Style

Steven T. Bellows; Joseph Jankovic. Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson’s Disease in a Movement Disorders Clinic. Tremor and Other Hyperkinetic Movements 2021, 11, 1 .

AMA Style

Steven T. Bellows, Joseph Jankovic. Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson’s Disease in a Movement Disorders Clinic. Tremor and Other Hyperkinetic Movements. 2021; 11 (1):1.

Chicago/Turabian Style

Steven T. Bellows; Joseph Jankovic. 2021. "Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson’s Disease in a Movement Disorders Clinic." Tremor and Other Hyperkinetic Movements 11, no. 1: 1.

Journal article
Published: 12 February 2021 in Toxins
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Opisthotonus refers to abnormal axial extension and arching of the trunk produced by excessive contractions of the paraspinal muscles. In childhood, the abnormal posture is most often related to dystonia in the setting of hypoxic injury or a number of other acquired and genetic etiologies. The condition is often painful, interferes with ambulation and quality of life, and is challenging to treat. Therapeutic options include oral benzodiazepines, oral and intrathecal baclofen, botulinum neurotoxin injections, and deep brain stimulation. Management of opisthotonus within the pediatric population has not been systematically reviewed. Here, we describe a series of seven children who presented to our institution with opisthotonus in whom symptom relief was achieved following administration of botulinum neurotoxin injections.

ACS Style

Mariam Hull; Mered Parnes; Joseph Jankovic. Botulinum Neurotoxin Injections in Childhood Opisthotonus. Toxins 2021, 13, 137 .

AMA Style

Mariam Hull, Mered Parnes, Joseph Jankovic. Botulinum Neurotoxin Injections in Childhood Opisthotonus. Toxins. 2021; 13 (2):137.

Chicago/Turabian Style

Mariam Hull; Mered Parnes; Joseph Jankovic. 2021. "Botulinum Neurotoxin Injections in Childhood Opisthotonus." Toxins 13, no. 2: 137.

Journal article
Published: 28 January 2021 in Neurology Genetics
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Objective To discover genetic determinants of Parkinson disease (PD) motor subtypes, including tremor dominant (TD) and postural instability/gait difficulty (PIGD) forms. Methods In 3,212 PD cases of European ancestry, we performed a genome-wide association study (GWAS) examining 2 complementary outcome traits derived from the Unified Parkinson's Disease Rating Scale, including dichotomous motor subtype (TD vs PIGD) or a continuous tremor/PIGD score ratio. Logistic or linear regression models were adjusted for sex, age at onset, disease duration, and 5 ancestry principal components, followed by meta-analysis. Results Among 71 established PD risk variants, we detected multiple suggestive associations with PD motor subtype, including GPNMB (rs199351, p subtype = 0.01, p ratio = 0.03), SH3GL2 (rs10756907, p subtype = 0.02, p ratio = 0.01), HIP1R (rs10847864, p subtype = 0.02), RIT2 (rs12456492, p subtype = 0.02), and FBRSL1 (rs11610045, p subtype = 0.02). A PD genetic risk score integrating all 71 PD risk variants was also associated with subtype ratio (p = 0.026, ß = −0.04, 95% confidence interval = −0.07–0). Based on top results of our GWAS, we identify a novel suggestive association at the STK32B locus (rs2301857, p ratio = 6.6 × 10−7), which harbors an independent risk allele for essential tremor. Conclusions Multiple PD risk alleles may also modify clinical manifestations to influence PD motor subtype. The discovery of a novel variant at STK32B suggests a possible overlap between genetic risk for essential tremor and tremor-dominant PD.

ACS Style

Isabel Alfradique-Dunham; Rami Al-Ouran; Rainer von Coelln; Cornelis Blauwendraat; Emily Hill; Lan Luo; Amanda Stillwell; Emily Young; Anita Kaw; Manuela Tan; Calwing Liao; Dena Hernandez; Lasse Pihlstrom; Donald Grosset; Lisa M. Shulman; Zhandong Liu; Guy A. Rouleau; Mike Nalls; Andrew B. Singleton; Huw Morris; Joseph Jankovic; Joshua M. Shulman. Genome-Wide Association Study Meta-Analysis for Parkinson Disease Motor Subtypes. Neurology Genetics 2021, 7, e557 .

AMA Style

Isabel Alfradique-Dunham, Rami Al-Ouran, Rainer von Coelln, Cornelis Blauwendraat, Emily Hill, Lan Luo, Amanda Stillwell, Emily Young, Anita Kaw, Manuela Tan, Calwing Liao, Dena Hernandez, Lasse Pihlstrom, Donald Grosset, Lisa M. Shulman, Zhandong Liu, Guy A. Rouleau, Mike Nalls, Andrew B. Singleton, Huw Morris, Joseph Jankovic, Joshua M. Shulman. Genome-Wide Association Study Meta-Analysis for Parkinson Disease Motor Subtypes. Neurology Genetics. 2021; 7 (2):e557.

Chicago/Turabian Style

Isabel Alfradique-Dunham; Rami Al-Ouran; Rainer von Coelln; Cornelis Blauwendraat; Emily Hill; Lan Luo; Amanda Stillwell; Emily Young; Anita Kaw; Manuela Tan; Calwing Liao; Dena Hernandez; Lasse Pihlstrom; Donald Grosset; Lisa M. Shulman; Zhandong Liu; Guy A. Rouleau; Mike Nalls; Andrew B. Singleton; Huw Morris; Joseph Jankovic; Joshua M. Shulman. 2021. "Genome-Wide Association Study Meta-Analysis for Parkinson Disease Motor Subtypes." Neurology Genetics 7, no. 2: e557.

Correspondence
Published: 27 January 2021 in Journal of the Neurological Sciences
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ACS Style

Karim Makhoul; Joseph Jankovic. Parkinson's disease after COVID-19. Journal of the Neurological Sciences 2021, 422, 117331 .

AMA Style

Karim Makhoul, Joseph Jankovic. Parkinson's disease after COVID-19. Journal of the Neurological Sciences. 2021; 422 ():117331.

Chicago/Turabian Style

Karim Makhoul; Joseph Jankovic. 2021. "Parkinson's disease after COVID-19." Journal of the Neurological Sciences 422, no. : 117331.

Review
Published: 08 January 2021 in Toxins
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Since its initial approval in 1989 by the US Food and Drug Administration for the treatment of blepharospasm and other facial spasms, botulinum toxin (BoNT) has evolved into a therapeutic modality for a variety of neurological and non-neurological disorders. With respect to neurologic movement disorders, BoNT has been reported to be effective for the treatment of dystonia, bruxism, tremors, tics, myoclonus, restless legs syndrome, tardive dyskinesia, and a variety of symptoms associated with Parkinson’s disease. More recently, research with BoNT has expanded beyond its use as a powerful muscle relaxant and a peripherally active drug to its potential central nervous system applications in the treatment of neurodegenerative disorders. Although BoNT is the most potent biologic toxin, when it is administered by knowledgeable and experienced clinicians, it is one of the safest therapeutic agents in clinical use. The primary aim of this article is to provide an update on recent advances in BoNT research with a focus on novel applications in the treatment of movement disorders. This comprehensive review of the literature provides a critical review of evidence-based clinical trials and highlights recent innovative pilot studies.

ACS Style

Charenya Anandan; Joseph Jankovic. Botulinum Toxin in Movement Disorders: An Update. Toxins 2021, 13, 42 .

AMA Style

Charenya Anandan, Joseph Jankovic. Botulinum Toxin in Movement Disorders: An Update. Toxins. 2021; 13 (1):42.

Chicago/Turabian Style

Charenya Anandan; Joseph Jankovic. 2021. "Botulinum Toxin in Movement Disorders: An Update." Toxins 13, no. 1: 42.

Review article
Published: 10 November 2020 in Faculty Reviews
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Though primarily a sporadic condition, Parkinson’s disease is increasingly recognized to be a multifactorial disease with a strong genetic component. At a cellular level, disruptions of protein trafficking and recycling, particularly by misfolding, accumulation, and aggregation of α-synuclein, mitochondrial dysfunction, oxidative stress, and other etiopathogenic mechanisms, have been found to result in the death of vulnerable neuronal populations and appear to drive the neurodegeneration underlying Parkinson’s disease. The improved understanding of these mechanisms has led to the development of novel pathogenesis-targeted and potentially disease-modifying therapeutic approaches in Parkinson’s disease. Until these treatments are fully developed and approved, clinicians must rely on therapies designed to improve quality of life of patients by treating various motor and non-motor symptoms of the disease.

ACS Style

Arjun Tarakad; Joseph Jankovic. Recent advances in understanding and treatment of Parkinson’s disease. Faculty Reviews 2020, 9, 1 .

AMA Style

Arjun Tarakad, Joseph Jankovic. Recent advances in understanding and treatment of Parkinson’s disease. Faculty Reviews. 2020; 9 ():1.

Chicago/Turabian Style

Arjun Tarakad; Joseph Jankovic. 2020. "Recent advances in understanding and treatment of Parkinson’s disease." Faculty Reviews 9, no. : 1.

Journal article
Published: 30 October 2020 in Journal of the Neurological Sciences
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Objectives To investigate hypothesized sources of error when quantifying the effect of the sensory trick in cervical dystonia (CD) with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS-2), test strategies to mitigate them, and provide guidance for future research on the sensory trick. Methods Previous analyses suggested the sensory trick (or "alleviating maneuver", AM) item be removed from the TWSTRS-2 because of its poor clinimetric properties. We hypothesized three sources of clinimetric weakness for rating the AM: 1) whether patients were given sufficient time to demonstrate their AM; 2) whether patients' CD was sufficiently severe for detecting AM efficacy; and 3) whether raters were inadvertently rating the item in reverse of scale instructions. We tested these hypotheses with video recordings and TWSTRS-2 ratings by one "site rater" and a panel of five "video raters" for each of 185 Dystonia Coalition patients with isolated CD. Results Of 185 patients, 23 (12%) were not permitted sufficient testing time to exhibit an AM, 23 (12%) had baseline CD too mild to allow confident rating of AM effect, and 1 site- and 1 video-rater each rated the AM item with a reverse scoring convention. When these confounds were eliminated in step-wise fashion, the item's clinimetric properties improved. Conclusions The AM's efficacy can contribute to measuring CD motor severity by addressing identified sources of error during its assessment and rating. Given the AM's sensitive diagnostic and potential pathophysiologic significance, we also provide guidance on modifications to how AMs can be assessed in future CD research.

ACS Style

Elizabeth Cisneros; Glenn T. Stebbins; Qiyu Chen; Jeanne P. Vu; Casey N. Benadof; Zheng Zhang; Richard L. Barbano; Susan H. Fox; Christopher G. Goetz; Joseph Jankovic; Hyder A. Jinnah; Joel S. Perlmutter; Charles H. Adler; Stewart A. Factor; Stephen G. Reich; Ramon Rodriguez; Lawrence L. Severt; Natividad P. Stover; Brian D. Berman; Cynthia L. Comella; David A. Peterson. It's tricky: Rating alleviating maneuvers in cervical dystonia. Journal of the Neurological Sciences 2020, 419, 117205 .

AMA Style

Elizabeth Cisneros, Glenn T. Stebbins, Qiyu Chen, Jeanne P. Vu, Casey N. Benadof, Zheng Zhang, Richard L. Barbano, Susan H. Fox, Christopher G. Goetz, Joseph Jankovic, Hyder A. Jinnah, Joel S. Perlmutter, Charles H. Adler, Stewart A. Factor, Stephen G. Reich, Ramon Rodriguez, Lawrence L. Severt, Natividad P. Stover, Brian D. Berman, Cynthia L. Comella, David A. Peterson. It's tricky: Rating alleviating maneuvers in cervical dystonia. Journal of the Neurological Sciences. 2020; 419 ():117205.

Chicago/Turabian Style

Elizabeth Cisneros; Glenn T. Stebbins; Qiyu Chen; Jeanne P. Vu; Casey N. Benadof; Zheng Zhang; Richard L. Barbano; Susan H. Fox; Christopher G. Goetz; Joseph Jankovic; Hyder A. Jinnah; Joel S. Perlmutter; Charles H. Adler; Stewart A. Factor; Stephen G. Reich; Ramon Rodriguez; Lawrence L. Severt; Natividad P. Stover; Brian D. Berman; Cynthia L. Comella; David A. Peterson. 2020. "It's tricky: Rating alleviating maneuvers in cervical dystonia." Journal of the Neurological Sciences 419, no. : 117205.

Other
Published: 02 September 2020
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Objective To better characterize oromandibular dystonia (OMD) to facilitate early diagnosis and test the hypothesis that botulinum toxin treatment alleviates symptoms, regardless of etiology, to provide guidance on treatment strategies. Methods To better characterize this condition we utilize a three-pronged approach. First, we provide a comprehensive summary of the world’s literature encompassing 1157 cases in 27 separate manuscripts. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter database. Finally, we provide details of the treatment approach and response from two expert centers where large numbers of OMD patients are followed. Cases from expert centers were utilized to analyze whether response to botulinum toxin varied by etiology of OMD. Results In all cohorts, typical age at onset was in the 50s and approximately 70 % of cases were female. Although the literature OMD more commonly described as a focal dystonia, analysis of the DC database revealed it more commonly appears as part of a segmental dystonia. Expert center review of 173 cases revealed botulinum toxin injections improved symptom severity by more than 50% in approximately 78% of subjects. Among the patients at expert centers, analysis revealed that treatment response did not vary by etiology. Conclusions Botulinum toxin injections are an effective treatment for OMD, regardless of etiology. By providing a more comprehensive description of OMD and the therapeutic efficacy of botulinum toxin for this type of dystonia, we hope to improve clinical recognition to aid in timely diagnosis and inform treatment strategies.

ACS Style

Laura M. Scorr; Stewart A. Factor; Sahyli Perez Parra; Rachel Kaye; Randal C. Paniello; Scott A. Norris; Joel Perlmutter; Tobias Bäumer; Tatiana Usnich; Brian Berman; Marie Mailly; Emmanuel Roze; Marie Vidailhet; Joseph Jankovic; Mark S. LeDoux; Richard Barbano; Florence Chang; Victor Fung; Sarah Pirio Richardson; Andrew Blitzer; Hyder A. Jinnah; for the Dystonia Coalition Investigators. Delineation of the Clinical Features and Treatment Response of Oromandibular Dystonia: A Multicenter Summary of 2,057 Cases. 2020, 1 .

AMA Style

Laura M. Scorr, Stewart A. Factor, Sahyli Perez Parra, Rachel Kaye, Randal C. Paniello, Scott A. Norris, Joel Perlmutter, Tobias Bäumer, Tatiana Usnich, Brian Berman, Marie Mailly, Emmanuel Roze, Marie Vidailhet, Joseph Jankovic, Mark S. LeDoux, Richard Barbano, Florence Chang, Victor Fung, Sarah Pirio Richardson, Andrew Blitzer, Hyder A. Jinnah, for the Dystonia Coalition Investigators. Delineation of the Clinical Features and Treatment Response of Oromandibular Dystonia: A Multicenter Summary of 2,057 Cases. . 2020; ():1.

Chicago/Turabian Style

Laura M. Scorr; Stewart A. Factor; Sahyli Perez Parra; Rachel Kaye; Randal C. Paniello; Scott A. Norris; Joel Perlmutter; Tobias Bäumer; Tatiana Usnich; Brian Berman; Marie Mailly; Emmanuel Roze; Marie Vidailhet; Joseph Jankovic; Mark S. LeDoux; Richard Barbano; Florence Chang; Victor Fung; Sarah Pirio Richardson; Andrew Blitzer; Hyder A. Jinnah; for the Dystonia Coalition Investigators. 2020. "Delineation of the Clinical Features and Treatment Response of Oromandibular Dystonia: A Multicenter Summary of 2,057 Cases." , no. : 1.

Review
Published: 27 August 2020 in Neurotherapeutics
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Tourette syndrome is a heterogeneous neurobehavioral disorder manifested by childhood-onset motor and phonic tics, often accompanied by a variety of behavioral comorbidities, including attention deficit and obsessive compulsive disorder. Treatment must be tailored to the needs and goals of the individual patients and their families. All patients should receive education on the condition and, if possible, engage behavioral therapy targeted towards tics and/or comorbidities. Pharmacological therapies, such as alpha agonists, topiramate, and vesicular monoamine transport type 2 inhibitors, are generally used as first-line therapies in patients with troublesome tics that are not controlled by behavioral therapy or when the latter is not available or accessible. Botulinum toxin injections can be used in patients with bothersome focal tics. Second-line therapy includes antipsychotics, such as fluphenazine, aripiprazole, risperidone, and ziprasidone. These medications are generally efficacious but carry the risk of metabolic syndrome, tardive dyskinesia, and other side effects. Much more research is needed before novel therapies such as cannabis-derived products or transcranial magnetic stimulation can be recommended. There is promise in ongoing clinical trials with D1 receptor antagonist ecopipam and other experimental therapeutics. Patients with tics that are refractory to conventional treatments may be candidates for deep brain stimulation, but further studies are needed to determine the optimal target selection.

ACS Style

Andrew Billnitzer; Joseph Jankovic. Current Management of Tics and Tourette Syndrome: Behavioral, Pharmacologic, and Surgical Treatments. Neurotherapeutics 2020, 17, 1681 -1693.

AMA Style

Andrew Billnitzer, Joseph Jankovic. Current Management of Tics and Tourette Syndrome: Behavioral, Pharmacologic, and Surgical Treatments. Neurotherapeutics. 2020; 17 (4):1681-1693.

Chicago/Turabian Style

Andrew Billnitzer; Joseph Jankovic. 2020. "Current Management of Tics and Tourette Syndrome: Behavioral, Pharmacologic, and Surgical Treatments." Neurotherapeutics 17, no. 4: 1681-1693.

Other
Published: 17 August 2020
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Introduction Emerging technologies show promise for enhanced characterization of Parkinson’s Disease (PD) motor manifestations. We evaluated quantitative mobility measures from a wearable device compared to the conventional motor assessment, the Movement Disorders Society-Unified PD Rating Scale part III (motor MDS-UPDRS). Methods We evaluated 176 subjects with PD (mean age 65, 65% male, 66% H&Y stage 2) at the time of routine clinic visits using the motor MDS-UPDRS and a structured 10-minute motor protocol, which included a 32-ft walk, Timed Up and Go (TUG), and standing posture with eyes closed, while wearing a body-fixed sensor (DynaPort MT, McRoberts BV). Regression models examined 12 quantitative mobility measures for associations with (i) motor MDS-UPDRS, (ii) motor subtype (tremor dominant vs. postural instability/gait difficulty), (iii) Montreal Cognitive Assessment (MoCA), and (iv) physical functioning disability (PROMIS-29). All analyses included age, gender, and disease duration as covariates. Models iii-iv were secondarily adjusted for motor MDS-UPDRS. Results Quantitative mobility measures from gait, TUG transitions, turning, and posture were significantly associated with motor MDS-UPDRS (7 of 12 measures, p< 0.05) and subtype (6 of 12 measures, p< 0.05). Compared with motor MDS-UPDRS, several quantitative mobility measures accounted for ∼1.5-fold increased variance in either cognition or physical functioning disability. Among minimally-impaired subjects within the bottom quartile of motor MDS-UPDRS, including subjects with normal gait exam, the measures captured substantial residual motor heterogeneity. Conclusion Clinic-based quantitative mobility assessments using a wearable sensor captured features of motor performance beyond those obtained with the motor MDS-UPDRS and may offer enhanced characterization of disease heterogeneity.

ACS Style

Emily J. Hill; Carl Grant Mangleburg; Isabel Alfradique-Dunham; Brittany Ripperger; Amanda Stillwell; Hiba Saade; Sindhu Rao; Oluwafunmiso Fagbongbe; Rainer Von Coelln; Arjun Tarakad; Christine Hunter; Robert J. Dawe; Joseph Jankovic; Lisa M. Shulman; Aron S. Buchman; Joshua M. Shulman. Quantitative mobility measures complement the MDS-UPDRS for characterization of Parkinson’s disease heterogeneity. 2020, 1 .

AMA Style

Emily J. Hill, Carl Grant Mangleburg, Isabel Alfradique-Dunham, Brittany Ripperger, Amanda Stillwell, Hiba Saade, Sindhu Rao, Oluwafunmiso Fagbongbe, Rainer Von Coelln, Arjun Tarakad, Christine Hunter, Robert J. Dawe, Joseph Jankovic, Lisa M. Shulman, Aron S. Buchman, Joshua M. Shulman. Quantitative mobility measures complement the MDS-UPDRS for characterization of Parkinson’s disease heterogeneity. . 2020; ():1.

Chicago/Turabian Style

Emily J. Hill; Carl Grant Mangleburg; Isabel Alfradique-Dunham; Brittany Ripperger; Amanda Stillwell; Hiba Saade; Sindhu Rao; Oluwafunmiso Fagbongbe; Rainer Von Coelln; Arjun Tarakad; Christine Hunter; Robert J. Dawe; Joseph Jankovic; Lisa M. Shulman; Aron S. Buchman; Joshua M. Shulman. 2020. "Quantitative mobility measures complement the MDS-UPDRS for characterization of Parkinson’s disease heterogeneity." , no. : 1.

Preprint content
Published: 27 June 2020
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OBJECTIVETo discover genetic determinants of Parkinson disease (PD) motor subtypes, including Tremor Dominant (TD) and Postural Instability/Gait Difficulty (PIGD) forms.METHODSIn 3,212 PD cases of European ancestry, we performed a genome-wide association study (GWAS) examining two complementary outcome traits derived from the Unified Parkinson’s Disease Rating Scale (UPDRS), including dichotomous motor subtype (TD vs. PIGD) or a continuous tremor / PIGD score ratio. Logistic or linear regression models were adjusted for sex, age of onset, disease duration, and 5 ancestry principal components, followed by meta-analysis.RESULTSAmong 71 established PD risk variants, we detected multiple suggestive associations with PD motor subtype, including GPNMB (rs199347, psubtype = 0.01, pratio = 0.03), SH3GL2 (rs10756907, psubtype = 0.02, pratio = 0.01), HIP1R (rs10847864, psubtype = 0.02), RIT2 (rs12456492, psubtype = 0.02), and FBRSL1 (rs11610045, psubtype = 0.02). A PD genetic risk score integrating all 71 PD risk variants was also associated with subtype ratio (p = 0.026, ß = −0.04, 95% CI = −0.07, 0). Based on top results of our GWAS, we identify a novel suggestive association at the STK32B locus (rs2301857, pratio = 6.6×10−7), which harbors an independent risk allele for essential tremor.CONCLUSIONSMultiple PD risk alleles may also modify clinical manifestations to influence PD motor subtype. The discovery of a novel variant at STK32B suggests a possible overlap between genetic risk for essential tremor and tremor-dominant PD.

ACS Style

Isabel Alfradique-Dunham; Rami Al-Ouran; Rainer Von Coelln; Cornelis Blauwendraat; Emily Hill; Lan Luo; Amanda Stillwell; Emily Young; Manuela Tan; Calwing Liao; Dena G. Hernandez; Lasse Pihlstrom; Donald Grosset; Lisa M. Shulman; Zhandong Liu; Guy A. Rouleau; Mike A. Nalls; Andrew B. Singleton; Huw Morris; Joseph Jankovic; Joshua M. Shulman; International Parkinson’s Disease Genomics Consortium (IPDGC). Genome-Wide Association Study Meta-Analysis for Parkinson’s Disease Motor Subtypes. 2020, 1 .

AMA Style

Isabel Alfradique-Dunham, Rami Al-Ouran, Rainer Von Coelln, Cornelis Blauwendraat, Emily Hill, Lan Luo, Amanda Stillwell, Emily Young, Manuela Tan, Calwing Liao, Dena G. Hernandez, Lasse Pihlstrom, Donald Grosset, Lisa M. Shulman, Zhandong Liu, Guy A. Rouleau, Mike A. Nalls, Andrew B. Singleton, Huw Morris, Joseph Jankovic, Joshua M. Shulman, International Parkinson’s Disease Genomics Consortium (IPDGC). Genome-Wide Association Study Meta-Analysis for Parkinson’s Disease Motor Subtypes. . 2020; ():1.

Chicago/Turabian Style

Isabel Alfradique-Dunham; Rami Al-Ouran; Rainer Von Coelln; Cornelis Blauwendraat; Emily Hill; Lan Luo; Amanda Stillwell; Emily Young; Manuela Tan; Calwing Liao; Dena G. Hernandez; Lasse Pihlstrom; Donald Grosset; Lisa M. Shulman; Zhandong Liu; Guy A. Rouleau; Mike A. Nalls; Andrew B. Singleton; Huw Morris; Joseph Jankovic; Joshua M. Shulman; International Parkinson’s Disease Genomics Consortium (IPDGC). 2020. "Genome-Wide Association Study Meta-Analysis for Parkinson’s Disease Motor Subtypes." , no. : 1.

2020 hindsight
Published: 23 June 2020 in Journal of Neurology, Neurosurgery & Psychiatry
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The concept of ‘idiopathic’ Parkinson’s disease (PD) as a single entity has been challenged with the identification of several clinical subtypes, pathogenic genes and putative causative environmental agents. In addition to classic motor symptoms, non-motor manifestations (such as rapid eye movement sleep disorder, anosmia, constipation and depression) appear at prodromic/premotor stage and evolve, along with cognitive impairment and dysautonomia, as the disease progresses, often dominating the advanced stages of the disease. The key molecular pathogenic mechanisms include α-synuclein misfolding and aggregation, mitochondrial dysfunction, impairment of protein clearance (associated with deficient ubiquitin-proteasome and autophagy-lysosomal systems), neuroinflammation and oxidative stress. The involvement of dopaminergic as well as noradrenergic, glutamatergic, serotonergic and adenosine pathways provide insights into the rich and variable clinical phenomenology associated with PD and the possibility of alternative therapeutic approaches beyond traditional dopamine replacement therapies.One of the biggest challenges in the development of potential neuroprotective therapies has been the lack of reliable and sensitive biomarkers of progression. Immunotherapies such as the use of vaccination or monoclonal antibodies directed against aggregated, toxic α-synuclein.as well as anti-aggregation or protein clearance strategies are currently investigated in clinical trials. The application of glucagon-like peptide one receptor agonists, specific PD gene target agents (such as GBA or LRRK2 modifiers) and other potential disease modifying drugs provide cautious optimism that more effective therapies are on the horizon. Emerging therapies, such as new symptomatic drugs, innovative drug delivery systems and novel surgical interventions give hope to patients with PD about their future outcomes and prognosis.

ACS Style

Joseph Jankovic; Eng King Tan. Parkinson’s disease: etiopathogenesis and treatment. Journal of Neurology, Neurosurgery & Psychiatry 2020, 91, 795 -808.

AMA Style

Joseph Jankovic, Eng King Tan. Parkinson’s disease: etiopathogenesis and treatment. Journal of Neurology, Neurosurgery & Psychiatry. 2020; 91 (8):795-808.

Chicago/Turabian Style

Joseph Jankovic; Eng King Tan. 2020. "Parkinson’s disease: etiopathogenesis and treatment." Journal of Neurology, Neurosurgery & Psychiatry 91, no. 8: 795-808.

Viewpoint
Published: 12 June 2020 in Movement Disorders
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Joseph Jankovic; Michael S. Okun; Jeffrey H. Kordower. Stem Cells: Scientific and Ethical Quandaries of a Personalized Approach to Parkinson's Disease. Movement Disorders 2020, 35, 1312 -1314.

AMA Style

Joseph Jankovic, Michael S. Okun, Jeffrey H. Kordower. Stem Cells: Scientific and Ethical Quandaries of a Personalized Approach to Parkinson's Disease. Movement Disorders. 2020; 35 (8):1312-1314.

Chicago/Turabian Style

Joseph Jankovic; Michael S. Okun; Jeffrey H. Kordower. 2020. "Stem Cells: Scientific and Ethical Quandaries of a Personalized Approach to Parkinson's Disease." Movement Disorders 35, no. 8: 1312-1314.

Editorial
Published: 09 April 2020 in Neurologic Clinics
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Joseph Jankovic. Treatment of Movement Disorders. Neurologic Clinics 2020, 38, 1 .

AMA Style

Joseph Jankovic. Treatment of Movement Disorders. Neurologic Clinics. 2020; 38 (2):1.

Chicago/Turabian Style

Joseph Jankovic. 2020. "Treatment of Movement Disorders." Neurologic Clinics 38, no. 2: 1.

Review article
Published: 28 February 2020 in Neurologic Clinics
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Tardive dyskinesia (TD) is an iatrogenic condition that encompasses a wide phenomenological spectrum of movement disorders caused by exposure to dopamine receptor blocking agents (DRBAs). TD may cause troublesome or disabling symptoms that impair quality of life. Due to frequent, often inappropriate, use of DRBAs, TD prevalence rates among patients exposed to DRBAs continue to be high. The judicious use of DRBAs is key to the prevention of TD, reduction of disease burden, and achieving lasting remission. Dopamine-depleting vesicular monoamine transporter type 2 inhibitors are considered the treatment of choice of TD.

ACS Style

Hassaan H. Bashir; Joseph Jankovic. Treatment of Tardive Dyskinesia. Neurologic Clinics 2020, 38, 379 -396.

AMA Style

Hassaan H. Bashir, Joseph Jankovic. Treatment of Tardive Dyskinesia. Neurologic Clinics. 2020; 38 (2):379-396.

Chicago/Turabian Style

Hassaan H. Bashir; Joseph Jankovic. 2020. "Treatment of Tardive Dyskinesia." Neurologic Clinics 38, no. 2: 379-396.