scholarly journals Oromandibular Dystonia: A Clinical Examination of 2,020 Cases

2021 ◽  
Vol 12 ◽  
Author(s):  
Laura M. Scorr ◽  
Stewart A. Factor ◽  
Sahyli Perez Parra ◽  
Rachel Kaye ◽  
Randal C. Paniello ◽  
...  
Keyword(s):  
Clinical Features ◽  
Age At Onset ◽  
Treatment Strategies ◽  
Oromandibular Dystonia ◽  
Cross Sectional ◽  
Psychiatric Disturbances ◽  
Segmental Dystonia ◽  
Expert Center ◽  
Sectional Analysis ◽  
Botulinum Toxin Injections

Objective: The goal of this study is to better characterize the phenotypic heterogeneity of oromandibular dystonia (OMD) for the purpose of facilitating early diagnosis.Methods: First, we provide a comprehensive summary of the literature encompassing 1,121 cases. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter cohort. Finally, we summarize clinical features and treatment outcomes from cross-sectional analysis of 172 OMD subjects from two expert centers.Results: In all cohorts, typical age at onset was in the 50s and 70% of cases were female. The Dystonia Coalition cohort revealed perioral musculature was involved most commonly (85%), followed by jaw (61%) and tongue (17%). OMD more commonly appeared as part of a segmental dystonia (43%), and less commonly focal (39%) or generalized (10%). OMD was found to be associated with impaired quality of life, independent of disease severity. On average, social anxiety (LSA score: 33 ± 28) was more common than depression (BDI II score: 9.7 ± 7.8). In the expert center cohorts, botulinum toxin injections improved symptom severity by more than 50% in ~80% of subjects, regardless of etiology.Conclusions: This comprehensive description of OMD cases has revealed novel insights into the most common OMD phenotypes, pattern of dystonia distribution, associated psychiatric disturbances, and effect on QoL. We hope these findings will improve clinical recognition to aid in timely diagnosis and inform treatment strategies.

scholarly journals Delineation of the Clinical Features and Treatment Response of Oromandibular Dystonia: A Multicenter Summary of 2,057 Cases.

2020 ◽  
Author(s):  
Laura M. Scorr ◽  
Stewart A. Factor ◽  
Sahyli Perez Parra ◽  
Rachel Kaye ◽  
Randal C Paniello ◽  
...  
Keyword(s):  
Botulinum Toxin ◽  
Treatment Response ◽  
Clinical Features ◽  
Age At Onset ◽  
Treatment Strategies ◽  
Oromandibular Dystonia ◽  
Large Numbers ◽  
Segmental Dystonia ◽  
Botulinum Toxin Treatment ◽  
Botulinum Toxin Injections

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 worlds 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.

Patient Selection Criteria for Deep Brain Stimulation for Dystonia

2020 ◽  
pp. 175-184
Author(s):  
Laura S. Surillo Dahdah ◽  
Rasheda El-Nazer ◽  
Richard B. Dewey ◽  
Padraig O’Suilleabhain ◽  
Shilpa Chitnis
Keyword(s):  
Botulinum Toxin ◽  
Patient Selection ◽  
Muscle Activation ◽  
Age At Onset ◽  
Voluntary Action ◽  
Body Distribution ◽  
Segmental Dystonia ◽  
Generalized Dystonia ◽  
Patient Selection Criteria ◽  
Botulinum Toxin Injections

Dystonia is defined as a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Dystonic movements are typically patterned and twisting and may be tremulous. Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation. A recent revision now classifies dystonia into two axes: (1) clinical characteristics (age at onset, temporal pattern, body distribution, whether focal, segmental, or generalized; and associated features) and (2) etiology, whether idiopathic/genetic or secondary to other neurological/medical diseases. Pharmacological treatments for dystonia remain generally unsatisfactory and consist of various combinations of levodopa, anticholinergics, muscle-relaxing drugs as well as botulinum toxin injections in focal and segmental dystonia. Overall in outcomes are poor because of limited efficacy and the potential for significant side effects such as sedation and cognitive impairment. A humanitarian-device exemption from the Food and Drug Administration was issued for the treatment of medically refractory symptoms of generalized dystonia with the use of DBS. Bilateral GPi DBS surgery is effective for both generalized and focal dystonia including cervical dystonia and tardive dystonia. DBS may be the best available treatment for disabling symptoms of generalized, cervical, tardive, and other dystonia that have failed to respond to oral drugs and botulinum toxin injections (when applicable) as long as contractures have not developed, because in this situation, DBS will be ineffective. Rigorous patient selection and careful management of comorbidities are essential for favorable outcomes.

scholarly journals Early variation of ultrasound halo sign with treatment and relation with clinical features in patients with giant cell arteritis

Rheumatology ◽  
2020 ◽  
Vol 59 (12) ◽  
pp. 3717-3726 ◽  
Author(s):  
Cristina Ponte ◽  
Ana Sofia Serafim ◽  
Sara Monti ◽  
Elisabete Fernandes ◽  
Ellen Lee ◽  
...  
Keyword(s):  
Clinical Features ◽  
Final Diagnosis ◽  
Newly Diagnosed ◽  
Glucocorticoid Treatment ◽  
Cross Sectional ◽  
Halo Sign ◽  
Early Use ◽  
Diagnostic Role ◽  
Sectional Analysis

Abstract Objectives To compare the ultrasound characteristics with clinical features, final diagnosis and outcome; and to evaluate the halo size following glucocorticoid treatment in patients with newly diagnosed GCA. Methods Patients with suspected GCA, recruited from an international cohort, had an ultrasound of temporal (TA) and axillary (AX) arteries performed within 7 days of commencing glucocorticoids. We compared differences in clinical features at disease presentation, after 2 weeks and after 6 months, according to the presence or absence of halo sign. We undertook a cross-sectional analysis of the differences in halo thickness using Pearson’s correlation coefficient (r) and Analysis of Variance (ANOVA). Results A total of 345 patients with 6 months follow-up data were included; 226 (65.5%) had a diagnosis of GCA. Jaw claudication and visual symptoms were more frequent in patients with halo sign (P =0.018 and P =0.003, respectively). Physical examination abnormalities were significantly associated with the presence of ipsilateral halo (P <0.05). Stenosis or occlusion on ultrasound failed to contribute to the diagnosis of GCA. During 7 days of glucocorticoid treatment, there was a consistent reduction in halo size in the TA (maximum halo size per patient: r=−0.30, P =0.001; and all halos r=−0.23, P <0.001), but not in the AX (P >0.05). However, the presence of halo at baseline failed to predict future ischaemic events occurring during follow-up. Conclusion In newly diagnosed GCA, TA halo is associated with the presence of ischaemic features and its size decreases following glucocorticoid treatment, supporting its early use as a marker of disease activity, in addition to its diagnostic role.

scholarly journals POS0950 ARE THERE GENDER DIFFERENCES IN AXIAL SPONDYLOARTHRITIS: DATA FROM A PORTUGUESE SPONDYLOARTHRITIS COHORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 738.2-738
Author(s):  
A. R. Cunha ◽  
R. Aguiar ◽  
M. J. Santos ◽  
V. Afreixo ◽  
A. Barcelos
Keyword(s):  
Gender Differences ◽  
Axial Spondyloarthritis ◽  
Age At Onset ◽  
Multivariable Analysis ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
First Record ◽  
National Database ◽  
Cross Sectional ◽  
Radiographic Findings

Background:Historically, axial spondyloarthritis (particularly ankylosing spondylitis) was considered a men’s disease and has been under-recognized in women.Emerging evidence reveals gender differences in patophysiology, disease presentation and therapeutic efficacy.Objectives:To determine if there are differences between genders in a Portuguese cohort of patients with axSpA as regards clinical manifestations, disease activity, functional capacity, patient related outcomes and radiographic findings.Methods:Patients with ≥18 years fulfilling the ASAS- Assessment of Spondyloarthritis International Society classification criteria for axSpA and registered in the electronic national database- Reuma.pt were included in a multicentric cross-sectional study. Sociodemographic data, clinical features and radiographic findings were collected from the first record in Reuma.pt. These variables were compared between genders using Mann-Whitney test and Chi-Square test. Variables with a significant association with group variable (gender) were considered in the multiple variable analysis to adjust the gender effect on the outcome variables.Results:A total of 1995 patients were included, 1114 (55.9%) men and 881 (44.1%) women. Men had a lower median age at onset of disease (25.1 vs 28.4, p=0.000) and median age at diagnosis (26.9 vs 30.4, p=0.000) and were more frequently smokers (32.1% vs 15.7%, p=0.000). Comparing to women, men had worse BASMI scores (4.0 vs 3.4, p=0.000), higher levels of CPR (10.5 vs 6.9, p=0.000) and were more often HLA-B27 positive (67.8% vs 54%, p=0.000). In univariable analysis, sacroiliitis on radiograph or/and MRI (95.5% vs 91.7%, p=0.04) was more common in men, however that wasn’t confirmed in multivariable analysis.In contrast, women more frequently had more inflammatory bowel disease (8.8% vs 4.9%, p=0,004), higher levels of ESR (25.0 vs 21.0, p=0.003) and worse PROs- BASDAI (5.7 vs 4.5, p=0.000), PGA (60.0 vs 55.0, p=0.000) and fatigue (6.2 vs 5.4, p=0,000).Conclusion:Physicians must be aware of differences between genders in axial spondyloarthritis because this can result in less underdiagnosis and misdiagnosis, allow optimization of treatment strategies, and decrease overall disease burden in women with axSpA patients.References:[1]Rusman T, van Vollenhoven RF, van der Horst-Bruinsma IE. Gender Differences in Axial Spondyloarthritis: Women Are Not So Lucky. Curr Rheumatol Rep. 2018;20(6):35.[2]Tournadre A, Pereira B, Lhoste A, Dubost JJ, Ristori JM, Claudepierre P, et al. Differences between women and men with recent-onset axial spondyloarthritis: results from a prospective multicenter French cohort. Arthritis Care Res (Hoboken). 2013;65(9):1482-9.Disclosure of Interests:None declared

An observational study of clozapine-induced sedation and its pharmacological management

2016 ◽  
Vol 33 (S1) ◽  
pp. S106-S106
Author(s):  
S. Ramos Perdigues ◽  
A. Mane Santacana ◽  
R.B. Sauras Quetcuti ◽  
E. Fernandez-Egea
Keyword(s):  
Treatment Strategies ◽  
Schizophrenia Spectrum Disorder ◽  
Cross Sectional ◽  
Pharmacological Management ◽  
Efficacy Analysis ◽  
Schizophrenia Spectrum ◽  
The Hours ◽  
Competing Interest ◽  
Sectional Analysis ◽  
Prospective Analyses

IntroductionClozapine is the only drug approved for resistant schizophrenia, but remains underused because of its side effects. Sedation is common, but its management is unclear.ObjectivesTo analyze factors associated with clozapine-induced sedation and the efficacy of common treatment strategies.AimsTo determine clozapine-induced sedation factors and possible therapeutic strategies.MethodsUsing two years’ electronic records of a community cohort of resistant schizophrenia spectrum disorder cases on clozapine, we performed three analyses: a cross-sectional analysis of which factors were associated with number of hours slept (objective proxy of sedation), and two prospective analyses: which factors were associated with changes in hours slept, and the efficacy of the main pharmacological strategies for improving sedation.ResultsOne hundred and thirty-three patients were included; 64.7% slept at least 9 hours/daily. Among monotherapy patients (n = 30), only norclozapine levels (r = .367, P = .033) correlated with sleeping hours. Multiple regression analyses confirmed the findings (r = .865, P < .00001). Using the cohort prospectively assessed (n = 107), 42 patients decreased the number of hours slept between two consecutive appointments. Decreasing clozapine (40%) or augmenting with aripiprazole (36%) were the most common factors. In the efficacy analysis, these two strategies were recommended to 22 (20.6%) and 23 (21.5%) subjects, respectively. The majority (81.8% and 73.9%) did not report differences in the hours slept.ConclusionsSedationis common and involves pharmacological and non-pharmacological factors. The only correlation was a weak correlation between norclozapine plasma levels and total sleeping hours. Reducing clozapine and aripiprazole augmentation were the most successful strategies to ameliorate sedation, although both strategies were effective only in a limited numbers of subjects.Disclosure of interestThe authors have not supplied their declaration of competing interest.

scholarly journals Publication bias in pediatric emergence delirium: a cross-sectional analysis of ClinicalTrials.gov and ClinicalTrialsRegister.eu

BMJ Open ◽  
2020 ◽  
Vol 10 (10) ◽  
pp. e037346
Author(s):  
Jochen Meyburg ◽  
Markus Ries
Keyword(s):  
Clinical Decision Making ◽  
Treatment Strategies ◽  
Clinical Decision ◽  
Selective Reporting ◽  
Publication Date ◽  
Cross Sectional ◽  
Emergence Delirium ◽  
Cross Sectional Analysis ◽  
Study Results ◽  
Sectional Analysis

ObjectivesEmergence delirium (ED) is a frequent and potentially serious complication of general anaesthesia in children. Although there are various treatment strategies, no general management recommendations can be made. Selective reporting of study results may impair clinical decision-making. We, therefore, analysed whether the results of completed registered clinical studies in patients with paediatric ED are publicly available or remain unpublished.DesignCross-sectional analysis.SettingClinicalTrials.gov and ClinicalTrialsRegister.eu.Participants and outcome measuresWe determined the proportion of published and unpublished studies registered at ClinicalTrials.gov and ClinicalTrialsRegister.eu that were marked as completed by 1st September 2018. The major trial and literature databases were used to search for publications. In addition, the study investigators were contacted directly. For published trials, time to publication was calculated as the difference in months between study completion date and publication date.ResultsOf the 44 registered studies on paediatric ED, only 24 (54%) were published by September 2019. Published trials contained data from n=2556 patients, whereas n=1644 patients were enrolled in unpublished trials. Median time to publication was 19 months. Studies completed in recent years were published faster, but still only 9 of 24 trials were published within 12 months of completion.ConclusionThere is a distinct publication gap in clinical research in paediatric ED that may have an impact on meta-analyses and clinical practice.

Clinical Features of Scleroderma Patients With or Without Prior or Current Ischemic Digital Ulcers: Post-Hoc Analysis of a Nationwide Multicenter Cohort (ItinérAIR-Sclérodermie)

2009 ◽  
Vol 36 (7) ◽  
pp. 1470-1476 ◽  
Author(s):  
KIET PHONG TIEV ◽  
ELISABETH DIOT ◽  
PIERRE CLERSON ◽  
FRÉDÉRIQUE DUPUIS-SIMÉON ◽  
ERIC HACHULLA ◽  
...  
Keyword(s):  
Clinical Features ◽  
Early Onset ◽  
Topoisomerase I ◽  
Age At Onset ◽  
Digital Ulcers ◽  
Cross Sectional ◽  
Prospective Longitudinal Study ◽  
Multicenter Cohort ◽  
Post Hoc ◽  
Prospective Longitudinal

Objective.Digital ulcers are the most frequent vascular manifestations of systemic sclerosis (SSc). Clinical features of patients with prior or current digital ulcers have not been extensively described. This cross-sectional analysis of a large multicenter cohort compared the characteristics of SSc patients with prior or current digital ulcers with those never affected.Methods.Patients with prior/current digital ulcers or never affected were identified in the cohort of SSc patients enrolled in the French ItinérAIR-Sclérodermie registry. Rodnan skin scores, pulmonary function test results, and clinical and immunological data were analyzed to identify digital ulcerassociated clinical features.Results.Of 599 SSc patients, 317 had prior or current digital ulcers. These patients were more frequently male, with impaired diffusing capacity for carbon monoxide (DLCO), and higher Rodnan skin scores than patients never affected by digital ulcers. In a multivariate analysis, male gender, early onset of SSc, increased duration of SSc, high Rodnan skin score, and presence of anti-topoisomerase I antibodies (anti-topo I) were associated with prior or current digital ulcers. Comparison of patients with current digital ulcers versus patients never affected indicated that affected patients had increased duration of SSc, impaired DLCO, increased Rodnan score, and younger age at onset of SSc.Conclusion.Male patients with early onset SSc, more severe skin fibrosis, impaired DLCO, and anti-topo I were most likely to exhibit prior or current digital ulcers. Confirmation of these results in a prospective longitudinal study may enable identification of patients at greatest risk of developing digital ulcers, facilitating management of this disabling complication.

scholarly journals Stable Angina Treatment Strategies and Current Practices in Lahore, Pakistan: A Cross-Sectional Analysis

2016 ◽  
Vol 6 (1) ◽  
pp. 1-5
Author(s):  
Ali Ahmed ◽  
Maria Tanveer ◽  
Naheed Bano ◽  
Gul Majid Khan
Keyword(s):  
Stable Angina ◽  
Drug Class ◽  
Chronic Stable Angina ◽  
Treatment Strategies ◽  
Pharmaceutical Journal ◽  
Therapeutic Agents ◽  
Cross Sectional ◽  
Drug Classes ◽  
Evidence Based Guidelines ◽  
Sectional Analysis

The purpose of the study was to analyze the prescription trends in the treatment of stable angina and to identify the therapeutic agents being prescribed majorly In Pakistan and to analyze that whether the current prescription patterns are in accordance with evidence based guidelines. A concurrent type of study was conducted from 24-01-2015 to 24-04-2015, through collection of data from prescriptions of patients suffering from stable angina in public sector hospitals of Lahore Pakistan. Data observations from the total 200 prescriptions collected from the hospitals of Lahore graphically demonstrate the trends of drug classes per prescription. The study identified that in the treatment of stable angina Antiplatelet are the most frequently prescribed drug class in Lahore and adjacent areas. Followed by CCBs on 2nd rank and nitrates on 3rd rank, ACE inhibitors and ARBs on 4th number, after which statins occupy the 5th position, lastly comes the position of beta blocker i.e. 6th and diuretics are the least prescribed class. The anti-platelet blocker drugs were the most prescribed drug class in the management of chronic stable angina followed by calcium channel and vasodilators occupying the second and third rank respectively. The study also revealed that men are 1.5 times more vulnerable to the development of angina than the females. The study is also showing concrete evidence that there is strong need to follow guidelines for proper treatment.Ahmed et al., International Current Pharmaceutical Journal, December 2016, 6(1): 1-5http://www.icpjonline.com/documents/Vol6Issue1/01.pdf

Ultramicrotomy as a Technique for Materials Specimen Preparation

1990 ◽  
Vol 48 (2) ◽  
pp. 428-429
Author(s):  
S.R. Glanvill
Keyword(s):  
Materials Science ◽  
Structural Information ◽  
Specimen Preparation ◽  
Preparation Technique ◽  
Cross Sectional ◽  
Surfaces And Interfaces ◽  
Beam Analysis ◽  
Flat Embedding ◽  
20 Nm ◽  
Sectional Analysis

This paper summarizes the application of ultramicrotomy as a specimen preparation technique for some of the Materials Science applications encountered over the past two years. Specimens 20 nm thick by hundreds of μm lateral dimension are readily prepared for electron beam analysis. Materials examined include metals, plastics, ceramics, superconductors, glassy carbons and semiconductors. We have obtain chemical and structural information from these materials using HRTEM, CBED, EDX and EELS analysis. This technique has enabled cross-sectional analysis of surfaces and interfaces of engineering materials and solid state electronic devices, as well as interdiffusion studies across adjacent layers.Samples are embedded in flat embedding moulds with Epon 812 epoxy resin / Methyl Nadic Anhydride mixture, using DY064 accelerator to promote the reaction. The embedded material is vacuum processed to remove trapped air bubbles, thereby improving the strength and sectioning qualities of the cured block. The resin mixture is cured at 60 °C for a period of 80 hr and left to equilibrate at room temperature.

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