A. J. Reyes, K. Ramcharan, Sean Perot, S. Giddings, F. Rampersad, R. Gobin
{"title":"Subacute Sclerosing Panencephalitis Causing Rapidly Progressive Dementia and Myoclonic Jerks in a Sexagenarian Woman","authors":"A. J. Reyes, K. Ramcharan, Sean Perot, S. Giddings, F. Rampersad, R. Gobin","doi":"10.7916/tohm.v0.680","DOIUrl":"https://doi.org/10.7916/tohm.v0.680","url":null,"abstract":"Background Subacute sclerosing panencephalitis (SSPE) is a disease of childhood and adolescence, but can affect adults. Rapidly progressive cognitive decline, seizures including myoclonic jerks, spasticity, ataxia, visual disturbances, and incontinence are typical manifestations. Case report A 62-year-old woman who presented with rapidly progressive dementia and myoclonus was diagnosed with SSPE. There was resolution of the movement disorder with clonazepam and valproic acid treatment and some amelioration of cognitive decline after 3 months of therapy with interferon alfa and isoprinosine. Discussion With the recent rise in measles cases worldwide, any increased incidence of SSPE would require vigilance for early interventions.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41813417","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Review of the Clinical Evidence for Complementary and Alternative Medicine in Huntington’s Disease","authors":"Margaret Yu, D. Bega","doi":"10.7916/tohm.v0.678","DOIUrl":"https://doi.org/10.7916/tohm.v0.678","url":null,"abstract":"Background There is a lack of published guidelines related to the use of complementary and alternative medicine (CAM) for Huntington’s disease (HD). We conducted a review of the literature to summarize the available evidence for various mind–body practices and nutraceuticals. Methods PubMed and Cochrane Library electronic databases were searched independently from inception to February 2019 by two independent raters. Studies were classified for the level of evidence (Class I, II, III, or IV) according to the American Academy of Neurology (AAN) classification scale. Results Randomized controlled trials in HD were reviewed for mind–body interventions (dance therapy, music therapy, and exercise), alternative systems (traditional Chinese medicine [TCM]), and nutraceuticals/diet (aminooxyacetic acid [AOAA], coenzyme q10, creatine, cannabinoids, alpha-tocopherol, eicosapentaenoic acid, idebenone, levocarnitine, and triheptanoin). Few studies met AAN Class I or II level of evidence for benefits, and these are highlighted. Discussion There is a relative paucity of clinical trials examining CAM modalities in HD when compared to other neurodegenerative disorders. Currently, there is no evidence supporting disease modification or symptom improvement with any specific dietary or nutraceutical supplement for HD. Supervised exercise and contemporary dance are safe for people with HD, but more robust studies are warranted to guide specific recommendations for these and other mind–body interventions.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44011913","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neurodegeneration with Brain Iron Accumulation: Two Additional Cases with Dystonic Opisthotonus","authors":"S. Mehta, V. Lal","doi":"10.7916/tohm.v0.683","DOIUrl":"https://doi.org/10.7916/tohm.v0.683","url":null,"abstract":"Background Specific phenomenology and pattern of involvement in movement disorders point toward a probable clinical diagnosis. For example, forehead chorea usually suggests Huntington’s disease; feeding dystonia suggests neuroacanthocytosis and risus sardonicus is commonly seen in Wilson’s disease. Dystonic opisthotonus has been described as a characteristic feature of neurodegeneration with brain iron accumulation (NBIA) related to PANK2 and PLA2G6 mutations. Case report We describe two additional patients in their 30s with severe extensor truncal dystonia causing opisthotonic posturing in whom evaluation revealed the diagnosis of NBIA confirmed by genetic testing. Discussion Dystonic opisthotonus may be more common in NBIA than it is reported and its presence especially in a young patient should alert the neurologists to a possibility of probable NBIA.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43343235","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Top 50 Most-Cited Articles in Orthostatic Tremor: A Bibliometric Review","authors":"M. L. Ruiz, Julián Benito-León","doi":"10.7916/tohm.v0.679","DOIUrl":"https://doi.org/10.7916/tohm.v0.679","url":null,"abstract":"Background Article-level citation count is a hallmark indicating scientific impact. We aimed to pinpoint and evaluate the top 50 most-cited articles in orthostatic tremor (OT). Methods The ISI Web of Knowledge database and 2017 Journal Citation Report Science Edition were used to retrieve the 50 top-cited OT articles published from 1984 to April 2019. Information was collected by the Analyze Tool on the Web of Science, including number of citations, publication title, journal name, publication year, and country and institution of origin. Supplementary analyses were undertaken to clarify authorship, study design, level of evidence, and category. Results Up to 66% of manuscripts were recovered from five journals: Movement Disorders (n = 18), Brain (n = 4), Journal of Clinical Neurophysiology (n = 4), Neurology (n = 4), and Clinical Neurophysiology (n = 3). Articles were published between 1984 and 2018, with expert opinion as the predominant design (n = 22) and review as category (n = 17). Most articles had level 5 evidence (n = 26). According to their countries of origin, 34% of articles belonged to the United States (n = 17) leading the list, followed by United Kingdom (n = 15). University College London yielded the greater number of articles (n = 12), followed by the University of Kiel (n = 9). Most popular authors were G. Deuschl (n = 10), C.D. Marsden (n = 6), J. Jankovic (n = 5), P.D. Thompson (n = 5), J.C. Rothwell (n = 5), L.J. Findley (n = 4), and P. Brown (n = 4), who together accounted for 48% of them. All papers were in English. Discussion Publishing high-cited OT articles could be facilitated by source journal, study design, category, publication language, and country and institution of origin.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42464530","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Gait Impairment in Myoclonus–Dystonia (DYT-SGCE)","authors":"G. Haeri, G. Shahidi, A. Fasano, M. Rohani","doi":"10.7916/tohm.v0.656","DOIUrl":"https://doi.org/10.7916/tohm.v0.656","url":null,"abstract":"Background Myoclonus–dystonia usually presents variable combination of myoclonus and dystonia mainly affecting the neck and arms, but leg involvement, especially as the presenting sign, is not common. Case report A 29-year-old lady with a heterozygous mutation in Epsilon-sarcoglycan (SGCE) gene is presented with rapid jerks of the right leg interfering with walking. She has also manifested dystonic posture and jerks of the trunk and proximal upper limbs. Discussion Although it is not typical, leg involvement could be a manifestation of myoclonus–dystonia either at presentation or during disease progression.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44682529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. L. Sousa, P. Salgado, J. Alves, S. Silva, S. Ferreira, M. Magalhães
{"title":"Uncommon Movement Disorders in Chronic Hepatic Disease with Response to Rifaximin","authors":"A. L. Sousa, P. Salgado, J. Alves, S. Silva, S. Ferreira, M. Magalhães","doi":"10.7916/tohm.v0.649","DOIUrl":"https://doi.org/10.7916/tohm.v0.649","url":null,"abstract":"Background Chronic hepatic disease can present with extrapyramidal symptoms. We describe two cases that presented with highly unusual movement disorders: ballism and gait freezing. Case report Patient 1 is a 42-year-old man with previous episodes of hepatic encephalopathy (HE) who presented with upper limb dystonia and generalized chorea that progressed to ballism. Patient 2 is a 55-year-old woman who presented with pronounced gait freezing. In both patients, features of HE and acquired hepatocerebral degeneration coexisted. They improved markedly, though transiently, with rifaximin. Discussion Ammonia-reducing treatments should be considered in patients presenting with movement disorders due to chronic liver disease.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48789849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful Treatment of Primary Orthostatic Tremor Using Perampanel","authors":"Anant Wadhwa, S. Schaefer","doi":"10.7916/tohm.v0.681","DOIUrl":"https://doi.org/10.7916/tohm.v0.681","url":null,"abstract":"Background Primary orthostatic tremor (POT) remains a therapeutic conundrum. Various medication classes have been tried, yielding modest results at best. Case Report A 62-year-old female with a 13-year history of POT, refractory to clonazepam up to 20 mg/day, was treated with perampanel 1–2 mg/day. She reported 90% subjective symptomatic improvement. Discussion This case highlights the potential for use of perampanel, a novel AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor antagonist for the treatment of POT. There has been one prior report citing its use for POT with complete resolution of symptoms. We encourage further studies to highlight its efficacy for POT.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46223888","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Facio-Oculo-Palatal Myoclonus Complicated by a Recurrent Brainstem Stroke","authors":"Y. Pitakpatapee, P. Srivanitchapoom","doi":"10.7916/tohm.v0.658","DOIUrl":"https://doi.org/10.7916/tohm.v0.658","url":null,"abstract":"Background A 54-year-old Thai male who has suffered from multiple episodes of ischemic and hemorrhagic strokes developed facio-oculo-palatal myoclonus (FOPM) 1 month after the latest episode of the brainstem stroke. Phenomenology Shown The patient presented with semirhythmic, involuntary, horizontal jerky, and rotatory ocular oscillation concomitant with asymmetrical palatal and perioral myoclonus consistent with FOPM. Educational value FOPM is a useful clinical clue for diagnosing brainstem lesions, specifically in the Guillain–Mollaret triangle. The commonest etiology is cerebrovascular diseases.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45536944","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Functional Movement Disorders in Elderly","authors":"A. Chouksey, S. Pandey","doi":"10.7916/tohm.v0.691","DOIUrl":"https://doi.org/10.7916/tohm.v0.691","url":null,"abstract":"Background Functional movement disorders (FMDs) have been rarely described in the elderly population. Methods This is a retrospective chart review of elderly patients with FMDs (onset >60 years) attending the movement disorders clinic at a tertiary care teaching institute in India. Results Out of 117 patients diagnosed with FMD at our center, 18 patients had an onset after the age of 60 years. The male-to-female ratio was 10:8 and the duration of symptoms ranged from 1 day to 5 years. Social (10/18) and physical factors (5/18) with an evident temporal relationship with the onset of FMD were identified in 15 out of 18 patients. Six of them had a past history of depression, anxiety, or other psychiatric illnesses. The tremor was the most frequent phenomenology seen in 11 (61.1%) patients, followed by dystonia in seven (38.8%), choreoballism and tics in two each, and hemifacial spasm and functional gait in one each. Seven patients had more than one phenotype. Discussion Tremor was the most frequent movement disorder seen in our patients with FMD. Surprisingly, tics (n = 2) and choreoballistic (n = 2) movements were also found in our patients with FMD, which has not been reported previously in an elderly population. Both physical and social factors were identified preceding the development of FMDs in majority of our patients.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45840281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Kreisler, Benoîte Bouchain, L. Defebvre, P. Krystkowiak
{"title":"Treatment with Botulinum Neurotoxin Improves Activities of Daily Living and Quality of Life in Patients with Upper Limb Tremor","authors":"A. Kreisler, Benoîte Bouchain, L. Defebvre, P. Krystkowiak","doi":"10.7916/tohm.v0.640","DOIUrl":"https://doi.org/10.7916/tohm.v0.640","url":null,"abstract":"Background Botulinum neurotoxin’s degree of effectiveness on upper limb tremor is subject to debate; although this treatment reduces the tremor’s amplitude, a clear functional benefit has not been demonstrated. The objective of this study was to assess the effect of botulinum neurotoxin type A treatment on activities of daily living and quality of life in patients with upper limb tremor. Methods We retrospectively examined the medical records of 50 consecutive patients treated with botulinum neurotoxin for upper limb tremor that was refractory to oral medication. One month after the injection, the patient was evaluated according to the Quality of Life in Essential Tremor Questionnaire, and the Essential Tremor Embarrassment Assessment. Results Full data sets were available for 38 patients suffering variously from essential tremor (n = 21), Holmes tremor secondary to a focal brain lesion (n = 8), idiopathic dystonic tremor (n = 4), primary writing tremor (n = 4), and Parkinson’s disease (n = 1). The Quality of Life Essential Tremor Questionnaire and the Essential Tremor Embarrassment Assessment scores improved significantly (p < 0.001) in the study population as a whole, and in the essential tremor and Holmes tremor subgroups. Discussion Botulinum neurotoxin treatment of patients with upper limb tremor is associated with improved quality of life and activities of daily living, irrespective of the tremor’s etiology. Long-term treatment enables the physician to adjust the injection strategy to the patient’s needs. Our study was limited by its retrospective design. The results must therefore be confirmed in a prospective, double-blind, placebo-controlled, randomized clinical trial.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41535875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}