Journal of Clinical Movement Disorders最新文献

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Are we missing non-motor seizures in Parkinson's disease? Two case reports. 帕金森氏症的非运动性癫痫发作我们是否遗漏了?两例报告。
Journal of Clinical Movement Disorders Pub Date : 2017-09-05 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0061-3
Andre Y Son, Alberto Cucca, Shashank Agarwal, Anli Liu, Alessandro Di Rocco, Milton C Biagioni
{"title":"Are we missing non-motor seizures in Parkinson's disease? Two case reports.","authors":"Andre Y Son,&nbsp;Alberto Cucca,&nbsp;Shashank Agarwal,&nbsp;Anli Liu,&nbsp;Alessandro Di Rocco,&nbsp;Milton C Biagioni","doi":"10.1186/s40734-017-0061-3","DOIUrl":"https://doi.org/10.1186/s40734-017-0061-3","url":null,"abstract":"<p><strong>Background: </strong>Parkinson's disease (PD) is predominantly recognized for its motor symptoms, but patients struggle from a morbid and heterogeneous collection of non-motor symptoms (NMS-PD) that can affect their quality of life even more. NMS-PD is a rather generalized term and the heterogeneity and non-specific nature of many symptoms poses a clinical challenge when a PD patient presents with non-motor complaints that may not be NMS-PD.</p><p><strong>Case presentation: </strong>We report two patients with idiopathic PD who presented with acute episodes of cognitive changes. Structural brain images, cardiovascular and laboratory assessment were unremarkable. Both patients experienced a considerable delay before receiving an epilepsy-evaluation, at which point electroencephalogram abnormalities supported the diagnosis of focal non-motor seizures with alteration of awareness. Antiepileptic therapy was implemented and was effective in both cases.</p><p><strong>Conclusions: </strong>Diagnosing non-motor seizures can be challenging. However, PD patients pose an even greater challenge given their eclectic non-motor clinical manifestations and other disease-related complications that could confound and mislead adequate clinical interpretation. Our two cases provide examples of non-motor seizures that may mimic non-motor symptoms of PD. Treating physicians should always consider other possible causes of non-motor symptoms that may coexist in PD patients. Epilepsy work-up should be contemplated in the differential of acute changes in cognition, behavior, or alertness.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"14"},"PeriodicalIF":0.0,"publicationDate":"2017-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0061-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35479319","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 6
A Computerized Cognitive behavioral therapy Randomized, Controlled, pilot trial for insomnia in Parkinson Disease (ACCORD-PD). 计算机认知行为疗法治疗帕金森病失眠症的随机、对照、先导试验(ACCORD-PD)。
Journal of Clinical Movement Disorders Pub Date : 2017-08-21 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0062-2
Shnehal Patel, Oluwadamilola Ojo, Gencer Genc, Srivadee Oravivattanakul, Yang Huo, Tanaporn Rasameesoraj, Lu Wang, James Bena, Michelle Drerup, Nancy Foldvary-Schaefer, Anwar Ahmed, Hubert H Fernandez
{"title":"<i>A C</i>omputerized <i>Co</i>gnitive behavioral therapy <i>R</i>andomized, Controlle<i>d</i>, pilot trial for insomnia in <i>P</i>arkinson <i>D</i>isease (<i>ACCORD-PD</i>).","authors":"Shnehal Patel,&nbsp;Oluwadamilola Ojo,&nbsp;Gencer Genc,&nbsp;Srivadee Oravivattanakul,&nbsp;Yang Huo,&nbsp;Tanaporn Rasameesoraj,&nbsp;Lu Wang,&nbsp;James Bena,&nbsp;Michelle Drerup,&nbsp;Nancy Foldvary-Schaefer,&nbsp;Anwar Ahmed,&nbsp;Hubert H Fernandez","doi":"10.1186/s40734-017-0062-2","DOIUrl":"https://doi.org/10.1186/s40734-017-0062-2","url":null,"abstract":"<p><strong>Background: </strong>Parkinson disease (PD) is associated with a high prevalence of insomnia, affecting up to 88% of patients. Pharmacotherapy studies in the literature addressing insomnia in PD reveal disappointing and inconsistent results. Cognitive behavioral therapy (CBT) is a novel treatment option with durable effects shown in primary insomnia. However, the lack of accessibility and expense can be limiting. For these reasons, computerized CBT for insomnia (CCBT-I) has been developed. The CCBT-I program is a 6-week web-based course consisting of daily \"lessons\" providing learnable skills and appropriate recommendations to help patients improve their sleep habits and patterns.</p><p><strong>Methods: </strong>We conducted a single-center, pilot, randomized controlled trial comparing CCBT-I versus standardized sleep hygiene instructions to treat insomnia in PD. Twenty-eight subjects with PD experiencing insomnia, with a score > 11 on the Insomnia Severity Index (ISI) were recruited. Based on a 6-point improvement in ISI in treatment group when compared to controls and an alpha = 0.05 and beta of 0.1 (power = 90%) a sample size of 11 patients (on active treatment) were required to detect this treatment effect using a dependent sample t-test.</p><p><strong>Results: </strong>In total, 8/14 (57%) subjects randomized to CCBT-I versus 13/14 (93%) subjects randomized to standard education completed the study. Among completers, the improvement in ISI scores was greater with CCBT-I as compared to standard education (-7.9 vs -3.5; <i>p</i> = 0.03). However, in an intention-to-treat analysis, where all enrolled subjects were included, the change in ISI between groups was not significant (-.4.5 vs -3.3; <i>p</i> = 0.48), likely due to the high dropout rate in the CCBT-I group (43%).</p><p><strong>Conclusion: </strong>This pilot study suggests that CCBT-I can be an effective treatment option for PD patients with insomnia when the course is thoroughly completed. High drop-out rate in our study shows that although effective, it may not be a generalizable option; however, larger studies are needed for further evaluation.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"16"},"PeriodicalIF":0.0,"publicationDate":"2017-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0062-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35308893","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 29
Pathophysiology of writer's cramp: an exploratory study on task-specificity and non-motor symptoms using an extended fine-motor testing battery. 作家痉挛的病理生理学:使用扩展精细运动测试电池对任务特异性和非运动症状进行探索性研究。
Journal of Clinical Movement Disorders Pub Date : 2017-08-08 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0060-4
Ali Amouzandeh, Michael Grossbach, Joachim Hermsdörfer, Eckart Altenmüller
{"title":"Pathophysiology of writer's cramp: an exploratory study on task-specificity and non-motor symptoms using an extended fine-motor testing battery.","authors":"Ali Amouzandeh, Michael Grossbach, Joachim Hermsdörfer, Eckart Altenmüller","doi":"10.1186/s40734-017-0060-4","DOIUrl":"10.1186/s40734-017-0060-4","url":null,"abstract":"<p><strong>Background: </strong>Writer's cramp (WC) is a task-specific focal dystonia which manifests itself as abnormal postures interfering with motor performance. As the spread of motor symptoms remains controversial and non-motor symptoms are widely discussed, in this exploratory study, we explore the pathophysiology of WC, focusing on task-specificity and the psychological profiles of WC patients.</p><p><strong>Methods: </strong>In 14 right-handed WC patients and matched controls, we assessed motor control by applying motor performance tests (Vienna Test Series), as well as using writing analysis and grip-force measurements. Moreover, detailed psychological factors were assessed. Classification trees were used to distinguish patients from controls.</p><p><strong>Results: </strong>The total duration of writing and the vertical writing frequency of the pen are the most important variables to split the data set successfully into patients and controls. No other variables concerning motor performance tests, grip-force measurements or psychological factors correctly separated patients and controls.</p><p><strong>Conclusions: </strong>Only variables from the writing tasks successfully separated patients and controls, indicating a strong task-specificity of WC in our patient group. Future research should be performed with larger samples of untreated WC patients in early stages of impairment, without any secondary motor disturbances, to verify our findings.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"13"},"PeriodicalIF":0.0,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5547537/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35309820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Italian validation of the Belastungsfragebogen Parkinson kurzversion (BELA-P-k): a disease-specific questionnaire for evaluation of the subjective perception of quality of life in parkinson's disease. 意大利验证Belastungsfragebogen帕金森kurzversion (BELA-P-k):一种评估帕金森病患者主观生活质量感知的疾病特异性问卷。
Journal of Clinical Movement Disorders Pub Date : 2017-07-25 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0059-x
Paola Ortelli, Roberto Maestri, Marianna Zarucchi, Veronica Cian, Elisa Urso, Francesca Giacomello, Davide Ferrazzoli, Giuseppe Frazzitta
{"title":"Italian validation of the Belastungsfragebogen Parkinson kurzversion (BELA-P-k): a disease-specific questionnaire for evaluation of the subjective perception of quality of life in parkinson's disease.","authors":"Paola Ortelli, Roberto Maestri, Marianna Zarucchi, Veronica Cian, Elisa Urso, Francesca Giacomello, Davide Ferrazzoli, Giuseppe Frazzitta","doi":"10.1186/s40734-017-0059-x","DOIUrl":"10.1186/s40734-017-0059-x","url":null,"abstract":"<p><strong>Background: </strong>Quality of life (QoL) is the sense of well-being perceived by people. The improvement of parkinsonian patient's QoL is a crucial goal for clinicians involved in rehabilitative care. In order to provide an appropriate endpoint for the assessment of the effectiveness of rehabilitation treatments on QoL of patients with Parkinson's Disease (PD), in this study we have first translated and then validated the Belastungsfragebogen Parkinson kurzversion (BELA-P-k). This tool allows evaluating separately two crucial aspects: i) the loss of personal autonomy in activities of daily life and ii) the psychological and psychosocial impact of the disease.</p><p><strong>Methods: </strong>The BELA-P-k was translated from Dutch into Italian. Subsequently 202 PD patients filled out the questionnaire. Patients were also evaluated by using the Parkinson Disease Questionnaire -39 (PDQ39), the Unified Parkinson's Disease Rating Scale (UPDRS), the Mini Mental State Examination (MMSE) and the Frontal Assessment Battery (FAB).</p><p><strong>Results: </strong>The internal consistency for total of two different scores <i>Bothered by</i> (Bb) and <i>Need for Help</i> (NfH) was excellent (<i>p</i> = 0.91) for both categories. The correlation between Bb and NfH categories was significant and strong, very-strong, ranging from 0.78 to 0.88 (all <i>p</i> < 0.0001). Finally, the value of Spearman r for the relationship between Bb and NfH items and PDQ 39 values were significant (<i>p</i> ≤ 0.003).</p><p><strong>Conclusions: </strong>In conclusion, we validated the BELA-P-k and demonstrated that it is an appropriate and potentially useful tool for assessing QoL in the management of PD.</p><p><strong>Trials registration: </strong>This trial was retrospectively registered with ClinicalTrials.gov, NCT03073044.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"12"},"PeriodicalIF":0.0,"publicationDate":"2017-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0059-x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35238968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Effects of a sensory-motor orthotic on postural instability rehabilitation in Parkinson's disease: a pilot study. 感觉-运动矫形器对帕金森病患者姿势不稳定康复的影响:一项初步研究。
Journal of Clinical Movement Disorders Pub Date : 2017-07-06 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0058-y
Daniele Volpe, Elisa Pelosin, Leila Bakdounes, Stefano Masiero, Giannettore Bertagnoni, Chiara Sorbera, Maria Giulia Giantin
{"title":"Effects of a sensory-motor orthotic on postural instability rehabilitation in Parkinson's disease: a pilot study.","authors":"Daniele Volpe,&nbsp;Elisa Pelosin,&nbsp;Leila Bakdounes,&nbsp;Stefano Masiero,&nbsp;Giannettore Bertagnoni,&nbsp;Chiara Sorbera,&nbsp;Maria Giulia Giantin","doi":"10.1186/s40734-017-0058-y","DOIUrl":"https://doi.org/10.1186/s40734-017-0058-y","url":null,"abstract":"<p><strong>Background: </strong>Proprioceptive deficits have been largely documented in PD patients, thus external sensory signals (peripheral sensory feedback) are often used to compensate the abnormalities of proprioceptive integration. This pilot study aims to evaluate the feasibility and the effectiveness of a rehabilitation-training program, combined with the use of a sensory-motor orthotic in improving balance in a small sample of PD patients.</p><p><strong>Methods: </strong>Twenty PD patients were randomly allocated into two groups: (i) <i>the Experimental group</i>, where participants were asked to wear a sensory-motor orthotic during the balance training program and (ii) <i>the Control group</i>, where subjects performed an identical training program without wearing any kind of orthotics. In all, the training program lasted 10 sessions (5 days a week for 2 weeks) and the clinical and instrumental assessments were performed at baseline, immediately after the end of the training and 4 weeks after the rehabilitative program was stopped.</p><p><strong>Results: </strong>All clinical outcome measures tested improved significantly at post and follow-up evaluations in both groups. Interestingly, at the end of the training, only the experimental group obtained a significant improvement in the functional reaching test (sway area - eyes closed) measured by means of stabilometric platform and this result was maintained in the follow-up evaluation.</p><p><strong>Conclusions: </strong>Our preliminary results suggested that the use of a sensory-motor orthotic, in combination with a tailored balance training, is feasible and it seems to positively impact on balance performance in Parkinson's disease.</p><p><strong>Trial registration: </strong>EudraCT N. 003020-36 - 2013.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"11"},"PeriodicalIF":0.0,"publicationDate":"2017-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0058-y","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35157186","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Functional neuroimaging and chorea: a systematic review. 功能性神经影像学与舞蹈病:系统综述。
Journal of Clinical Movement Disorders Pub Date : 2017-06-21 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0056-0
Debra J Ehrlich, Ruth H Walker
{"title":"Functional neuroimaging and chorea: a systematic review.","authors":"Debra J Ehrlich,&nbsp;Ruth H Walker","doi":"10.1186/s40734-017-0056-0","DOIUrl":"https://doi.org/10.1186/s40734-017-0056-0","url":null,"abstract":"<p><p>Chorea is a hyperkinetic movement disorder consisting of involuntary irregular, flowing movements of the trunk, neck or face. Although Huntington's disease is the most common cause of chorea in adults, chorea can also result from many other neurodegenerative, metabolic, and autoimmune conditions. While the pathophysiology of these different conditions is quite variable, recent advances in functional imaging have enabled the development of new methods for analysis of brain activity and neuronal dysfunction. In this paper we review the growing body of functional imaging data that has been performed in chorea syndromes and identify particular trends, which can be used to better understand the underlying network changes within the basal ganglia. While it can be challenging to identify whether changes are primary, secondary, or compensatory, identification of these trends can ultimately be useful in diagnostic testing and treatment in many of the conditions that cause chorea.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"8"},"PeriodicalIF":0.0,"publicationDate":"2017-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0056-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35119747","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 36
RimabotulinumtoxinB in sialorrhea: systematic review of clinical trials. 治疗霰粒肿的 RimabotulinumtoxinB:临床试验系统回顾。
Journal of Clinical Movement Disorders Pub Date : 2017-06-06 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0055-1
Khashayar Dashtipour, Roongroj Bhidayasiri, Jack J Chen, Bahman Jabbari, Mark Lew, Diego Torres-Russotto
{"title":"RimabotulinumtoxinB in sialorrhea: systematic review of clinical trials.","authors":"Khashayar Dashtipour, Roongroj Bhidayasiri, Jack J Chen, Bahman Jabbari, Mark Lew, Diego Torres-Russotto","doi":"10.1186/s40734-017-0055-1","DOIUrl":"10.1186/s40734-017-0055-1","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study was to examine the efficacy, safety and dosing practices of rimabotulinumtoxinB (BoNT-B) for the treatment of patients with sialorrhea based on a systematic review of clinical trials.</p><p><strong>Methods: </strong>A systematic literature review was performed to identify randomized controlled trials and other comparative clinical studies of BoNT-B for the treatment of sialorrhea published in English between January 1999 and December 2015. Medical literature databases (PubMed, Cochrane Library, and EMBASE) were searched and a total of 41 records were identified. Of these, six primary publications that evaluated BoNT-B for the treatment of sialorrhea met criteria and were included in the final data report.</p><p><strong>Synthesis: </strong>Total BoNT-B doses ranged from 1500 to 4000 units for sialorrhea. Most of the studies in sialorrhea showed statistically significant benefits of BoNT-B versus placebo (range 4-19.2 weeks). BoNT-B was generally well tolerated across the individual studies; most adverse events reported were considered unrelated to treatment. Adverse events considered potentially associated with BoNT-B included: dry mouth, change in saliva thickness, mild transient dysphagia, mild weakness of chewing and diarrhea.</p><p><strong>Conclusions: </strong>BoNT-B significantly reduces sialorrhea at doses between 1500 and 4000 units. The relatively mild dose-dependent adverse events suggest both direct and remote toxin effects.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"9"},"PeriodicalIF":0.0,"publicationDate":"2017-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5460542/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35070122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Parkinson disease in Gaucher disease. 戈歇病中的帕金森病。
Journal of Clinical Movement Disorders Pub Date : 2017-05-23 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0054-2
Federico Rodriguez-Porcel, Alberto J Espay, Miryam Carecchio
{"title":"Parkinson disease in Gaucher disease.","authors":"Federico Rodriguez-Porcel,&nbsp;Alberto J Espay,&nbsp;Miryam Carecchio","doi":"10.1186/s40734-017-0054-2","DOIUrl":"https://doi.org/10.1186/s40734-017-0054-2","url":null,"abstract":"<p><strong>Background: </strong>Gaucher disease (GD) is an inborn error of metabolism caused by mutations in the gene (<i>GBA</i>) coding for glucocerebrosidase (GCase), inherited in an autosomal recessive pattern. GD patients have up to 9% risk of developing PD.</p><p><strong>Case presentation: </strong>We report two patients with GD that developed PD at different disease stages.</p><p><strong>Conclusion: </strong>We reviewed the literature on the coexistence of PD and GD and speculate that the severity of symptoms may be related to the type of GBA mutation inherited.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"7"},"PeriodicalIF":0.0,"publicationDate":"2017-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0054-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35030231","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 12
A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies. 与抗谷氨酸脱羧酶抗体相关的僵硬犬综合征1例。
Journal of Clinical Movement Disorders Pub Date : 2017-05-10 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0053-3
Theresa E Pancotto, John H Rossmeisl
{"title":"A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies.","authors":"Theresa E Pancotto,&nbsp;John H Rossmeisl","doi":"10.1186/s40734-017-0053-3","DOIUrl":"https://doi.org/10.1186/s40734-017-0053-3","url":null,"abstract":"<p><strong>Background: </strong>The stiff person syndrome (SPS) is a rare and debilitating autoimmune disorder with an unknown pathogenesis and variable clinical presentation that can present a diagnostic challenge. Although entities that clinically mimic stiff-person spectrum disorders (SPSD) have manifested in horses, they have not been reported in dogs.</p><p><strong>Case presentation: </strong>We describe a 2-year-old beagle dog presented for progressive attacks of muscular rigidity and lordosis with superimposed spasms of the appendicular muscles triggered by tactile stimulation which resulted in marked gait impairment. Resting electromyography revealed continuous motor unit activity in the axial musculature. Compared to age-matched healthy beagle dogs, this patient had elevated glutamic acid decarboxylase antibody concentrations in serum and cerebrospinal fluid.</p><p><strong>Conclusion: </strong>This dog presented with phenotypic, electrodiagnostic, and immunologic criterion consistent with an SPSD, including elevated anti-GAD antibody titers, which we have termed the \"stiff dog syndrome (SDS)\". Durable clinical improvement was achieved with symptomatic and immunosuppressive treatments including baclofen, gabapentin, prednisone, and intravenous immunoglobulin.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"5"},"PeriodicalIF":0.0,"publicationDate":"2017-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0053-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34989150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 9
Dystonic storm: a practical clinical and video review. 失张力风暴:一个实用的临床和视频回顾。
Journal of Clinical Movement Disorders Pub Date : 2017-04-28 eCollection Date: 2017-01-01 DOI: 10.1186/s40734-017-0057-z
Pichet Termsarasab, Steven J Frucht
{"title":"Dystonic storm: a practical clinical and video review.","authors":"Pichet Termsarasab,&nbsp;Steven J Frucht","doi":"10.1186/s40734-017-0057-z","DOIUrl":"https://doi.org/10.1186/s40734-017-0057-z","url":null,"abstract":"<p><p>Dystonic storm is a frightening hyperkinetic movement disorder emergency. Marked, rapid exacerbation of dystonia requires prompt intervention and admission to the intensive care unit. Clinical features of dystonic storm include fever, tachycardia, tachypnea, hypertension, sweating and autonomic instability, often progressing to bulbar dysfunction with dysarthria, dysphagia and respiratory failure. It is critical to recognize early and differentiate dystonic storm from other hyperkinetic movement disorder emergencies. Dystonic storm usually occurs in patients with known dystonia, such as DYT1 dystonia, Wilson's disease and dystonic cerebral palsy. Triggers such as infection or medication adjustment are present in about one-third of all events. Due to the significant morbidity and mortality of this disorder, we propose a management algorithm that divides decision making into two periods: the first 24 h, and the next 2-4 weeks. During the first 24 h, supportive therapy should be initiated, and appropriate patients should be identified early as candidates for pallidal deep brain stimulation or intrathecal baclofen. Management in the next 2-4 weeks aims at symptomatic dystonia control and supportive therapies.</p>","PeriodicalId":15374,"journal":{"name":"Journal of Clinical Movement Disorders","volume":"4 ","pages":"10"},"PeriodicalIF":0.0,"publicationDate":"2017-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40734-017-0057-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34959174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 49
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