Tess E. K. Cersonsky, Daniel Trujillo Diaz, S. Kellner, R. Hickman, Maria Anna Zdrodowska, J. Monin, E. Louis
{"title":"Enfeeblement in Elders with Essential Tremor: Characterizing the Phenomenon and Its Role in Caregiver Burden","authors":"Tess E. K. Cersonsky, Daniel Trujillo Diaz, S. Kellner, R. Hickman, Maria Anna Zdrodowska, J. Monin, E. Louis","doi":"10.7916/tohm.v0.687","DOIUrl":"https://doi.org/10.7916/tohm.v0.687","url":null,"abstract":"Background Individuals with essential tremor (ET), a common movement disorder, experience functional impairment, which contributes to burden experienced by their loved ones and caregivers. Some burdened caregivers report their loved ones as seeming debilitated or prematurely old, a concept that we have called enfeeblement. Using the Essential Tremor Enfeeblement Survey (ETES), we seek to characterize enfeeblement in elders with ET and assess its contribution to caregiver burden. Methods We administered the ETES (range = 8–40, higher scores indicating more enfeeblement) and other scales to 98 caregivers of individuals with ET. Individuals with ET were also queried regarding tremors, cognitive abilities, and overall health. We then identified demographic and clinical correlates of ETES and modeled the contribution of ETES to caregiver burden (assessed using the Zarit 12-item Burden Interview [ZBI-12]). Results Mean ETES score was 14.2 ± 6.2 (median = 12.0, range = 8.0–32.0); 26.5% of respondents endorsed at least one of the eight ETES items. Older age, greater tremor severity and disability, more functional and gait disability, more cognitive difficulty, and more depressive symptoms were associated with higher ETES scores. ETES was the strongest contributor to caregiver burden (ZBI-12) and substantially increased the variance explained in models of caregiver burden. Discussion Enfeeblement seems to describe a previously unexplained component of caregiver burden in elders with ET. The presence of enfeeblement may contribute to greater burden and should be factored into assessments of patient and caregiver needs.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49036455","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}
Jongmok Ha, B. Na, J. H. Ahn, Minkyeong Kim, Jae Woo Kim, Jae Hyeok Lee, J. Cho, Ji Sun Kim, J. Youn
{"title":"Anti-CV2/CRMP5 Paraneoplastic Chorea Effectively Managed with Intravenous Amantadine","authors":"Jongmok Ha, B. Na, J. H. Ahn, Minkyeong Kim, Jae Woo Kim, Jae Hyeok Lee, J. Cho, Ji Sun Kim, J. Youn","doi":"10.7916/tohm.v0.701","DOIUrl":"https://doi.org/10.7916/tohm.v0.701","url":null,"abstract":"Background Paraneoplastic chorea is typically a subacute progressive hyperkinetic movement disorder. The mainstay of treatment is managing the underlying neoplasm. However, the clinical course may be variable, and effective symptomatic management can precede the start of cancer treatment. Case report A 63-year-old man presented with insidious onset, slowly progressive generalized chorea for 1 year, later diagnosed as anti-CV2/CRMP5 autoantibody positive paraneoplastic chorea. His chorea was markedly improved with intravenous amantadine. Discussion In patients with anti-CV2/CRMP5 autoantibody-related chorea, sequential follow-up of brain magnetic resonance imaging reveals progression from active inflammation to atrophy. Our report highlights the efficacy of intravenous amantadine in paraneoplastic chorea.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43779692","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}
M. Paucar, Alexander M. R. Taylor, M. Hadjivassiliou, B. Fogel, P. Svenningsson
{"title":"Progressive Ataxia with Elevated Alpha-Fetoprotein: Diagnostic Issues and Review of the Literature","authors":"M. Paucar, Alexander M. R. Taylor, M. Hadjivassiliou, B. Fogel, P. Svenningsson","doi":"10.7916/tohm.v0.708","DOIUrl":"https://doi.org/10.7916/tohm.v0.708","url":null,"abstract":"Background Ataxias represent a challenging group of disorders due to significant clinical overlap. Here, we present a patient with early-onset progressive ataxia, polyneuropathy and discuss how elevation of alpha fetoprotein (AFP) narrows the differential diagnosis. Case report Ataxia, polyneuropathy, and mild elevation of AFP are features compatible with ataxia with oculomotor apraxia type 2 (AOA2) but also with ataxia with oculomotor apraxia type 4 (AOA4). A genetic analysis demonstrated biallelic mutations in senataxin (SETX), confirming the diagnosis of AOA2. Discussion Mild elevation of AFP is found in patients with AOA2 and AOA4, and higher levels are commonly seen in ataxia-telangiectasia. AFP is a useful diagnostic tool but not a biomarker for disease progression in AOA2.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42667157","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. Rachdi, J. Dupouy, M. Benaiteau, C. Bost, M. Moreau, C. B. Courbon, O. Rascol, F. Magne
{"title":"Leucine-Rich Glioma-Inactivated 1 Encephalitis: Broadening the Sphere","authors":"A. Rachdi, J. Dupouy, M. Benaiteau, C. Bost, M. Moreau, C. B. Courbon, O. Rascol, F. Magne","doi":"10.7916/tohm.v0.663","DOIUrl":"https://doi.org/10.7916/tohm.v0.663","url":null,"abstract":"Background Leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a rare entity. Its typical features are seizures, faciobrachial dystonic seizures (FBDS), cognitive impairment, and personality changes. Case report We report the case of a 66-year-old man with an unusual presentation, consisting of two types of FBDS, one starting in the foot and the other consisting of asynchronous myoclonic and dystonic jerks of the face triggered by noise and chin stimulation. The patient displayed no personality changes or cognitive impairment. Discussion LGI1 encephalitis is a heterogeneous disease. Many different forms of FBDS may be observed, and these seizures can be the only symptom. This type of encephalitis should be suspected in presenting very frequent episodic events with dystonic features, regardless of the part of the body affected.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46720527","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}
F. Cavallieri, F. Valzania, L. Vercueil, E. Moro, V. Fraix
{"title":"A Case of Peripherally Induced Task-Specific “Lipstick Dystonic Tremor”","authors":"F. Cavallieri, F. Valzania, L. Vercueil, E. Moro, V. Fraix","doi":"10.7916/tohm.v0.689","DOIUrl":"https://doi.org/10.7916/tohm.v0.689","url":null,"abstract":"Background Peripherally induced movement disorders (PIMDs) represent a rare and debated complication of peripheral trauma. Phenomenology Shown We report a case of task-specific “lipstick” jerky dystonic tremor as a consequence of traumatic shoulder injury, successfully treated with EMG-guided botulinum toxin injections. Educational Value This case expands the phenotypic spectrum of PIMDs, with a visual example of a task-specific dystonic tremor after peripheral trauma, and the efficacy of EMG-guided botulinum toxin treatment in the setting of posttraumatic dystonic tremor.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49553224","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. T. Meira, W. Arruda, S. Ono, A. C. Neto, S. Raskin, C. Camargo, H. Teive
{"title":"Neuroradiological Findings in the Spinocerebellar Ataxias","authors":"A. T. Meira, W. Arruda, S. Ono, A. C. Neto, S. Raskin, C. Camargo, H. Teive","doi":"10.7916/tohm.v0.682","DOIUrl":"https://doi.org/10.7916/tohm.v0.682","url":null,"abstract":"Background The spinocerebellar ataxias (SCAs) are a group of autosomal dominant degenerative diseases characterized by cerebellar ataxia. Classified according to gene discovery, specific features of the SCAs – clinical, laboratorial, and neuroradiological (NR) – can facilitate establishing the diagnosis. The purpose of this study was to review the particular NR abnormalities in the main SCAs. Methods We conducted a literature search on this topic. Results The main NR characteristics of brain imaging (magnetic resonance imaging or computerized tomography) in SCAs were: (1) pure cerebellar atrophy; (2) cerebellar atrophy with other findings (e.g., pontine, olivopontocerebellar, spinal, cortical, or subcortical atrophy; “hot cross bun sign”, and demyelinating lesions); (3) selective cerebellar atrophy; (4) no cerebellar atrophy. Discussion The main NR abnormalities in the commonest SCAs, are not pathognomonic of any specific genotype, but can be helpful in limiting the diagnostic options. We are progressing to a better understanding of the SCAs, not only genetically, but also pathologically; NR is helpful in the challenge of diagnosing the specific genotype of SCA.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42372251","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}
Bisena Bulica, Christos Sidiropoulos, A. Mahajan, Andrew Zillgitt, Patricia Kaminski, S. Bowyer
{"title":"Sensorimotor Integration and GABA-ergic Activity in Embouchure Dystonia: An Assessment with Magnetoencephalography","authors":"Bisena Bulica, Christos Sidiropoulos, A. Mahajan, Andrew Zillgitt, Patricia Kaminski, S. Bowyer","doi":"10.7916/tohm.v0.709","DOIUrl":"https://doi.org/10.7916/tohm.v0.709","url":null,"abstract":"Background Embouchure dystonia (ED) is a task-specific dystonia affecting musicians thought to be related to alteration in sensorimotor processing and loss of cortical inhibition. Case Report Magnetoencephalography-coherence source imaging (MEG-CSI) was used to map connectivity between brain regions by imaging neuronal oscillations that are coherent across the brain in patient with ED at rest and while using the index finger to evoke dystonia normally triggered by playing the flute. Discussion During rest, there was increased coherence in the bilateral frontal and parietal regions that became more focal during dystonia. Diffuse hyperexcitability and increased coherence persisted in bilateral parietal regions as well as the bilateral frontal regions.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":"3 3","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41296976","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}
Nester Mitchell, Gaynel A. LaTouche, Beverly Nelson, K. Figueroa, R. Walker, Andrew K. Sobering
{"title":"Fast Orthostatic Tremor in Parkinson’s Disease: Case Report and Comprehensive Review of Literature","authors":"Nester Mitchell, Gaynel A. LaTouche, Beverly Nelson, K. Figueroa, R. Walker, Andrew K. Sobering","doi":"10.7916/tohm.v0.670","DOIUrl":"https://doi.org/10.7916/tohm.v0.670","url":null,"abstract":"Background Orthostatic tremor (OT) is a rare symmetric tremor disorder occasionally observed in association with other movement disorders. Case report We report the presence of a fast OT in a case of Parkinson’s disease (PD), and provide a comprehensive review of the literature. Discussion A fast OT presenting as unsteadiness may be a presenting symptom of PD. This symptom may be nonresponsive to levodopa, and benzodiazepines should be prescribed to adequately control the OT and reduce disability.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41388114","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}
M. Higuchi, R. Roemmich, D. Martinez-Ramirez, Jaimie A. Roper, Lisa A. Zukowski, K. Foote, M. Okun, C. Hass
{"title":"Changes in Midline Tremor and Gait Following Deep Brain Stimulation for Essential Tremor","authors":"M. Higuchi, R. Roemmich, D. Martinez-Ramirez, Jaimie A. Roper, Lisa A. Zukowski, K. Foote, M. Okun, C. Hass","doi":"10.7916/tohm.v0.684","DOIUrl":"https://doi.org/10.7916/tohm.v0.684","url":null,"abstract":"Background Essential tremor (ET) is a common movement disorder characterized by kinetic and postural tremor in the upper extremities and frequently in the midline. Persons with ET often also exhibit gait ataxia. Previous studies have observed associations between midline tremor severity and gait ataxia in persons with ET, suggesting a common pathophysiology distinct from that of upper extremity tremor. However, a causal link between midline tremor and gait impairment has not been established. Methods We investigated tremor and gait in 24 persons with ET before and after implantation of unilateral deep brain stimulation into the ventralis intermedius nucleus of the thalamus. Results Stimulation significantly improved tremor in the targeted upper extremity and midline. However, gait was unaffected at the cohort level. Furthermore, improvement in midline tremor was not significantly associated with gait improvement. Discussion These findings revealed that midline tremor and gait impairment may be dissociable in persons with ET.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47575094","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}
Ahmad Elkouzi, T. Tsuboi, Matthew R Burns, R. Eisinger, Amar Patel, Wissam Deeb
{"title":"Dorsal GPi/GPe Stimulation Induced Dyskinesia in a Patient with Parkinson’s Disease","authors":"Ahmad Elkouzi, T. Tsuboi, Matthew R Burns, R. Eisinger, Amar Patel, Wissam Deeb","doi":"10.7916/tohm.v0.685","DOIUrl":"https://doi.org/10.7916/tohm.v0.685","url":null,"abstract":"Clinical vignette A 68-year-old man with Parkinson’s disease (PD) had bilateral GPi DBS placed for management of his motor fluctuations. He developed stimulation-induced dyskinesia (SID) with left dorsal GPi stimulation. Clinical dilemma What do we know about SID in PD patients with GPi DBS? What are the potential strategies used to maximize the DBS therapeutic benefit and minimize the side effects of stimulation? Clinical solution Avoiding the contact implicated in SID and programming more ventral contacts, using lower voltage, frequency and pulse width and programming in bipolar configuration all appear to help minimize the SID and provide appropriate symptomatic motor control. Gap in knowledge Little is known about SID in patients with PD who had GPi DBS therapy. More studies using volume of tissue activated and diffusion tensor imaging MRI are needed to localize specific tracts in or around the GPi that may be implicated in SID.","PeriodicalId":23317,"journal":{"name":"Tremor and Other Hyperkinetic Movements","volume":" ","pages":""},"PeriodicalIF":2.2,"publicationDate":"2019-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45964288","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}