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Wanchun Cheng,1 Mohammad Mofatteh,2 José Fidel Baizabal-Carvallo,3,4 Shaohuan Lu,1 Ping Su,1 Yimin Chen,5,6 Luoming Li,1 Lizhi Qin,1 Xingmei Zuo,1 Yifeng Lan,7 Yue Huang,8 Zhihui Yu,9 Zirui Luo,10,&ast; Gang Chen10,&ast; 1Department of Neurology and National Stroke Center, The 5th People’s Hospital of Foshan City, Foshan, People’s Republic of China; 2School of Medicine, Dentistry and Biomedical Sciences, Queen’s University Belfast, Belfast, UK; 3Parkinson’s Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, USA; 4Department of Sciences and Engineering, University of Guanajuato, León, Mexico; 5Department of Neurology and Advanced National Stroke Center, Foshan Sanshui District People’s Hospital, Foshan, People’s Republic of China; 6Neuro International Collaboration (NIC), Foshan, People’s Republic of China; 7Department of Radiology, the 5th People’s Hospital of Foshan City, Foshan, People’s Republic of China; 8Department of Emergency, the 5th People’s Hospital of Foshan City, Foshan, People’s Republic of China; 9Department of Laboratory Medicine, the 5th People’s Hospital of Foshan City, Foshan, People’s Republic of China; 10Dean’s Office, the 5th People’s Hospital of Foshan City, Foshan, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Zirui Luo; Gang Chen, Dean’s Office, the 5th People’s Hospital of Foshan City, Foshan, Guangdong Province, 528211, People’s Republic of China, Email ziruiluo@126.com; vikingsaga@163.comPurpose: Intravenous thrombolysis has emerged as an effective approach to improve the long-term survival and functional status of patients with ischemic stroke. The aim of this study was to assess the impact of a national stroke project on the door-to-needle-time (DNT).Patients and Methods: The patients were divided into pre-construction and construction periods. Construction Measures were performed during the construction period. The DNT and onset-to-needle time (ONT) were compared in two period groups.Results: After participating in the National Stroke Center Project and effective measurements, the thrombolysis treatment metrics were improved significantly. The DNT (IQR) was shortened from 65.0 (54.5,85.0) minutes in the Pre-Construction period to 40.0 (33.0,53.0) minutes in the Construction period (p < 0.001). Similarly, the ONT was reduced from 157.0 (IQR) (115.0,184.0) minutes to 116.0 (87.8,170.0) minutes (p = 0.035).Conclusion: The DNT time and ONT time can be shortened by National Stroke Center Construction projects. More suitable hospitals should be encouraged to participate as the National Stroke Center.Keywords: acute ischemic stroke, intravenous thrombolysis, door-to-needle time, onset-to-needle time

Background: Tics are the hallmark of Tourette syndrome (TS) and chronic tic disorders (CTD). Although typically involving the face, especially at onset, tics may involve any muscle under voluntary control, including axial muscles of the neck (causing head movements), shoulders and trunk (thorax and abdomen). We aimed to characterize these tics and provide a clinical frame for their associations and complications., Materials and Methods: We reviewed video recordings and clinical history of 196 patients with TS or CTD according to DSM-5., Results: Any axial tic was identified in 75% of patients. Tic distribution were head (n = 113, 57.6%), shoulder (n = 91, 46.4%), and trunk (n = 63, 32.2%). There were no differences in sex, age at onset or at evaluation between patients with and without axial tics. The most common axial tics by anatomical distribution were head turning, bilateral synchronous shoulder elevation and trunk jerks; however, tic phenomenology was quite variable. A greater severity of tics (P = 0.018) was associated with axial tics in the multivariate regression analysis. Head/neck tics associated with simple phonic tics (P = 0.002); whereas shoulder and trunk tics associated with complex motor tics (P < 0.05) in a bivariate analysis. Neck pain, breathing interference, sleep limitation and radiculopathy, secondary to axial tics were complications observed in a proportion of these cases., Conclusions: Axial tics are commonly observed in patients with TS/CTD with variable phenomenology. They associate with greater tic severity, phonic tics and complex motor tics. They may result in neck pain, breathing interference, sleeping problems and cervical spine injuries., Competing Interests: Declarations Conflicts of interest On behalf of all the authors, the corresponding author states that there is no conflict of interest., (© 2024. Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Neuropathic tremor occurs with damage to the peripheral nervous system. Guillain-Barré syndrome (GBS) causes acute paralysis following nerve inflammation sometimes resulting in long-term disability. It is unclear how frequent and severe tremor is following GBS., Objectives: We aimed to assess the frequency and features of tremor following GBS., Methods: We enrolled 18 patients with GBS treated in a secondary care center within a 4-year period. Evaluations were done with the Fahn-Tolosa-Marin tremor rating scale (FTM-TRS). We compared these features with a cohort of consecutive patients with untreated essential tremor (ET)., Results: There were 13 males and 5 females with a mean age at evaluation (S.D.) of 41.5 ± 14.0 years and at GBS onset of 40.2 ± 13.7. No patient had history of tremor before GBS. Upper limb tremor was identified in 16 (89%) cases, 35.5% of patients had FTM-TRS score ≥10 points. Tremor was mostly kinetic, jerky with low amplitude with a total score of 10.94 ± 11.84 in the FTM-TRS. Compared with patients with ET, those with GBS-tremor were younger and had lower scores in all subscales of the FTM-TRS ( P value < 0.05 for all comparisons). In a multivariate linear regression analysis "days of hospitalization" had a positive association with the total FTM-TRS score ( P = 0.001)., Conclusions: Tremor was common following GBS. This tremor is mild compared with patients with ET, but adds functional impact., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2024 The Author(s).)

Objective: Functional neurological disorders (FNDs) are increasingly recognized in the general population and neurology clinics, and there is evidence that patients with neurological disorders are more likely to have a FND. This study was designed to identify the clinical features of FNDs among patients with movement disorders when the two disorders coexist., Methods: The clinical histories and video recordings of 150 consecutive patients with tremors were examined: the types of tremor included essential tremor; essential tremor plus; dystonic tremor; tremor associated with dystonia; and drug-induced, myoclonic, orthostatic, task-specific, parkinsonian, Holmes, and unclassified tremor. Using criteria for "possible" and "probable" coexistent FND, clinical features that helped differentiate between functional and other neurological tremors were identified., Results: There were 27 (18%) patients with functional manifestations, and 17 (11% of full sample) of these patients had signs suggestive of comorbid functional tremor. Patients with comorbid functional manifestations were younger at presentation, and these patients had greater severity of tremor, particularly upper limb postural tremor. Functional manifestations were not more commonly observed among patients with any particular type of tremor, except for patients with Holmes tremor, who were more likely to have comorbid functional neurological manifestations., Conclusions: About 18% of patients with diverse types of tremors also had comorbid functional neurological manifestations. Of the coexistent FNDs, functional tremor was the most common. Patients with co-occurring functional and other neurological tremors presented for evaluation at a younger age and had greater severity of arm tremor than those without comorbid functional neurological manifestations., Competing Interests: Dr. Baizabal-Carvallo has received royalties from Medlink Neurology. Dr. Jankovic has received research or training funding from Allergan, CHDI Foundation, Dystonia Coalition, F. Hoffmann-La Roche, Huntington Study Group, Medtronic Neuromodulation, Merz, Michael J. Fox Foundation for Parkinson Research, Neurocrine Biosciences, NIH, Nuvelution Pharma, Parkinson’s Foundation, Parkinson Study Group, Revance, and Teva; he has served as a consultant or adviser for Abide, Allergan, Lundbeck, Parexel, Retrophin, and Teva; he has served on editorial boards for several journals; and he has received royalties from Cambridge, Elsevier, Future Science Group, Hodder Arnold, Lippincott Williams and Wilkins, Medlink Neurology, and Wiley-Blackwell. Dr. Alonso-Juarez reports no financial relationships with commercial interests.

Immune-mediated cerebellar ataxias (IMCAs) have diverse etiologies. Patients with IMCAs develop cerebellar symptoms, characterized mainly by gait ataxia, showing an acute or subacute clinical course. We present a novel concept of latent autoimmune cerebellar ataxia (LACA), analogous to latent autoimmune diabetes in adults (LADA). LADA is a slowly progressive form of autoimmune diabetes where patients are often initially diagnosed with type 2 diabetes. The sole biomarker (serum anti-GAD antibody) is not always present or can fluctuate. However, the disease progresses to pancreatic beta-cell failure and insulin dependency within about 5 years. Due to the unclear autoimmune profile, clinicians often struggle to reach an early diagnosis during the period when insulin production is not severely compromised. LACA is also characterized by a slowly progressive course, lack of obvious autoimmune background, and difficulties in reaching a diagnosis in the absence of clear markers for IMCAs. The authors discuss two aspects of LACA: (1) the not manifestly evident autoimmunity and (2) the prodromal stage of IMCA's characterized by a period of partial neuronal dysfunction where non-specific symptoms may occur. In order to achieve an early intervention and prevent cell death in the cerebellum, identification of the time-window before irreversible neuronal loss is critical. LACA occurs during this time-window when possible preservation of neural plasticity exists. Efforts should be devoted to the early identification of biological, neurophysiological, neuropsychological, morphological (brain morphometry), and multimodal biomarkers allowing early diagnosis and therapeutic intervention and to avoid irreversible neuronal loss., (© 2023. The Author(s).)

Tourette syndrome (TS) is a complex neurodevelopmental disorder characterized by the presence of tics, frequently accompanied by a variety of neuropsychiatric comorbidities. A subset of patients with TS present with severe and disabling symptoms, requiring prompt therapeutic intervention. Some of these manifestations may result in medical emergencies when severe motor or phonic tics lead to damage of anatomical structures closely related to the tic. Examples include myelopathy or radiculopathy following severe neck ("whiplash") jerks or a variety of self-inflicted injuries. In addition to self-aggression or, less commonly, allo-aggression, some patients exhibit highly inappropriate behavior, suicidal tendencies, and rage attacks which increase the burden of the disease and are important components of "malignant TS". This subset of TS is frequently associated with comorbid obsessive-compulsive disorder. Therapeutic measures include intensive behavioral therapy, optimization of oral pharmacotherapy, botulinum toxin injections, and deep brain stimulation., Competing Interests: Declarations Informed consent for video/photography and publication in a scientific journal was provided by patients or legal proxy, approved by the Research Committee of Baylor College of Medicine., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background: Rising from a chair or the sit-to-stand (STS) task is frequently impaired in individuals with Parkinson's disease (PD). These patients commonly attribute such difficulties to weakness in the lower extremities. However, the role of muscle strength in the STS transfer task has not been fully elucidated., Objective: We aim at determining the role of muscle strength in the STS task., Methods: We studied 90 consecutive patients with PD and 52 sex- and age-matched controls. Lower limb strength was determined in both legs by clinical examination using the Medical Research Council Scale, dynamometric (leg flexion) and weighting machine (leg pressure) measures. Patients were interrogated regarding the presence of subjective lower limb weakness or allied sensations., Results: There were 20 patients (22.2%) with abnormal STS task (item 3.9 of the MDS-UPDRS-III ≥2 points). These patients had higher modified Hoehn and Yahr stage ( P   <  0.001) and higher total motor scores of the MDS-UPDRS( P   <  0.001), compared with 70 PD patients with normal STS task. Patients with abnormal STS task endorsed lower limb weakness more frequently and had lower muscle strength in the proximal lower extremities, compared to PD patients with normal STS task and normal controls. The presence of perceived lower limb weakness increased the risk of an abnormal STS task, OR: 11.93 (95% C.I. 1.51-94.32), whereas a hip extension strength ≤9 kg/pressure also increased the risk of abnormal STS task, OR: 4.45 (95% C.I. 1.49-13.23). In the multivariate regression analysis, bradykinesia and decreased hip strength were related to abnormal STS task., Conclusions: Patients with PD and abnormal STS task complain more commonly of lower limb weakness and have decreased proximal lower limb strength compared to patients with PD and normal STS task, likely contributing to abnormalities in performing the STS task., Competing Interests: The authors declare that there are no conflicts of interest., (Copyright © 2023 José Fidel Baizabal-Carvallo et al.)

Tics are rapid, recurrent, non-rhythmic movements or emitted sounds. Tics are the hallmark of Tourette syndrome (TS); however, a number of other disorders may be associated with tics, so-called secondary tic disorders (STD). We assessed clinical history and performed blinded evaluations of video-recordings from patients with TS and STD in order to identify features that may differentiate tics associated with TS vs STD. There were 156 patients with TS and 38 with STD, 21 of whom had functional (psychogenic) tics. Patients with TS were more frequently male and had a younger age at onset. Tics in TS tend to involve muscles in the cranial-cervical area more often and have greater severity and complexity than those in patients with STD. Similar findings were observed when contrasting patients with TS with patients with functional tics only. Simple phonic tics showed the greatest diagnostic accuracy for TS, compared with STD, but marked overlap in the types of tics and comorbidities was observed between patients with TS and STD. Patients with TS were more likely males, had a younger age at onset, phonic tics and motor tics affecting predominantly the head and neck area, and had a greater complexity and severity of tics than those with STD. When these features are absent a consideration should be given to the possibility of a tic disorder other than TS., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Background and Objective: The hyperdense middle cerebral artery sign (HMCAS) is observed in a proportion of patients with acute ischemic stroke (AIS). This sign reflects the presence of an intravascular thrombus rich in red blood cells. Several studies have demonstrated that HMCAS increases the risk of poor outcomes in AIS patients treated with IV thrombolysis or no reperfusion therapy; however, whether HMCAS predicts a poor outcome in patients treated with endovascular thrombectomy (EVT) is less clear. We aimed to evaluate the functional outcome by the modified Rankin scale (mRS) at 90 days and technical challenges in patients with HMCAS undergoing EVT., Methods: We studied 143 consecutive AIS patients with middle cerebral artery M1 segment or internal carotid artery + M1 occlusions who underwent EVT., Results: There were 73 patients (51%) with HMCAS. Patients with HMCAS had a higher frequency of cardioembolic stroke ( p  = 0.038); otherwise, no other baseline difference was observed. No differences in functional outcomes (mRS) at 90  days ( p  = 0.698), unfavorable outcomes (mRS > 2) ( p  = 0.929), frequency of symptomatic intracranial hemorrhage ( p  = 0.924), and mortality (mRS-6) ( p  = 0.736) were observed between patients with and without HMCAS. In patients with HMCAS, EVT procedures were 9  min longer, requiring a higher number of passes ( p  = 0.073); however, optimal recanalization scores (modified thrombolysis in cerebral infarction: 2b-3) were equally achieved by both groups., Conclusion: Patients with HMCAS treated with EVT do not have a worse outcome at 3  months compared with no-HMCAS patients. Patients with HMCAS required a greater number of thrombus passes and longer procedure times., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Chen, Diana, Mofatteh, Zhou, Chen, Huang, Wu, Yang, Zeng, Zhang, Ouyang, Nguyen, Yang, Baizabal-Carvallo and Liao.)

Background: Tourette syndrome (TS) is the most common cause of chronic tics. Patients with TS frequently manifest motor tics involving the eyes and face but oromandibular (OM) tics have been rarely studied., Materials and Methods: We reviewed the medical records and video-recordings of 155 consecutive patients with TS in our movement disorders clinic. In addition, we studied 35 patients with classic tardive dyskinesia (TD) and compared their clinical and demographic features with those with TS., Results: We identified 41 patients with OM tics (26.5%). Although patients with OM tics had a greater overall tic severity and higher frequency of.complex motor and phonic tics, in the bivariate analysis, only comorbid dystonic tics (P = 0.001), greater number of affected body parts (P = 0.012) and more frequent eye-rolling tics (P = 0.059) were included in the final regression model after controlling for other variables. When compared with patients with OM tics, patients with classic TD had more frequently masticatory movements (sensitivity, 0.86; specificity, 0.95), continuous tongue movements (sensitivity, 0.71; specificity, 1.0) and continuous OM movements (sensitivity, 0.4; specificity, 1.0)., Conclusions: OM tics are common and often troublesome or even disabling symptoms in patients with TS. They may be difficult to differentiate from TD, but the latter is typically manifested by continuous orolingual and masticatory movements., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Objective: Tourette syndrome (TS) is a neurodevelopmental disorder characterized by the presence of motor and phonic tics. Blocking phenomena, characterized by arrests in motor activity causing interruptions in movements or speech, have also been described in patients with TS. In this study, we aimed to characterize the frequency and features of blocking tics in patients with TS., Methods: We studied a cohort of 201 patients with TS evaluated at our movement disorders clinic., Results: We identified 12 (6%) patients with blocking phenomena. Phonic tic intrusion causing speech arrest was the most common (n = 8, 4%), followed by sustained isometric muscle contractions arresting body movements (n = 4, 2%). The following variables were statistically related to blocking phenomena: shoulder tics, leg tics, copropraxia, dystonic tics, simple phonic tics, and number of phonic tics per patient (all p < 0.050). In the multivariate regression, the presence of dystonic tics (p = 0.014) and a higher number of phonic tics (p = 0.022) were associated with blocking phenomena., Conclusion: Blocking phenomena are present in approximately 6% of patients with TS, and the presence of dystonic tics and a higher frequency and number of phonic tics increase the risk for these phenomena.

Background: Secondary dystonia has been associated with diverse etiologies. Dystonia associated with brain tumors has not been well characterized., Objectives: To characterize dystonia and relationship with parenchymal brain tumors., Methods: We present six patients (1.03%) with dystonia related to parenchymal brain tumors, among 580 screened cases., Results: Contralateral hemidystonia was observed in four cases, followed by focal limb (n = 1) and cervical dystonia (n = 1). Dystonia presented during the phase of tumor growth in four cases, and following tumor treatment in two, one case had re-emergent dystonia. Tumors were low-grade (WHO I or II) and located in the basal ganglia (n = 3), cortical areas (n = 2), thalamus (n = 1) and cerebral peduncle (n = 1)., Conclusions: Secondary dystonia may be caused by brain tumors in diverse locations including basal ganglia, cortex and thalamus. It may be the presenting symptom of brain tumor or follow surgical resection combined with ancillary therapy., Competing Interests: Declaration of Competing Interest None for all authors., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Encephalitis has been recognized in patients with autoimmunity related to the 65-kDa isoform of glutamic acid decarboxylase (GAD65) antibodies; however, patients with meningoencephalitis associated with those antibodies have been rarely identified in the medical literature. We aimed to define the frequency, clinical features, response to therapy, and functional outcomes of patients with meningoencephalitis associated with GAD antibodies., Methods: We retrospectively studied consecutive patients attending a tertiary care center for evaluation of an autoimmune neurological disorder from January 2018 to June 2022. The modified Rankin Scale (mRS) was used to assess the functional outcome at the last follow-up., Results: We evaluated 482 patients with confirmed autoimmune encephalitis during the study period. Four among the 25 patients with encephalitis related to GAD65 antibodies were identified. One patient was excluded owing to the coexistence of NMDAR antibodies. Three male patients aged 36, 24, and 16 years had an acute ( n = 1) or subacute ( n = 2) onset of confusion, psychosis, cognitive symptoms, seizures, or tremor. No patient had fever or clinical signs of meningeal irritation. Mild pleocytosis (<100 leukocytes/106) was identified in two patients, whereas one patient had normal CSF. Following immunotherapy with corticosteroids ( n = 3) or intravenous immunoglobulin ( n = 1), significant improvement was observed in all three cases, achieving a good outcome (mRS 1) in all cases., Conclusion: Meningoencephalitis is an uncommon presentation of GAD65 autoimmunity. Patients present with signs of encephalitis but with meningeal enhancement and have good outcomes., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Kuang, Baizabal-Carvallo, Mofatteh, Xie, Pan, Ye, Zhou, Yang, Wang, Chen and Li.)

Background: Stroke is one of the leading causes of mortality across the world. However, there is a paucity of information regarding mortality rates and associated risk factors in patients with acute ischemic stroke (AIS) undergoing endovascular thrombectomy (EVT). In this study, we aimed to clarify these issues and analyzed previous publications related to mortality in patients treated with EVT., Methods: We analyzed the survival of 245 consecutive patients treated with mechanical thrombectomy for AIS for which mortality information was obtained. Early mortality was defined as death occurring during hospitalization after EVT or within 7 days following hospital discharge from the stroke event., Results: Early mortality occurred in 22.8% of cases in this cohort. Recanalization status (modified thrombolysis in cerebral infarction, mTICI) ( p = 0.002), National Institute of Health Stroke Scale Score (NIHSS) score 24-h after EVT ( p < 0.001) and symptomatic intracerebral hemorrhage (sICH) ( p < 0.001) were independently associated with early mortality. Age, sex, cardiovascular risk factors, NIHSS score pre-treatment, Alberta Stroke Program Early CT Score (ASPECTS), stroke subtype, site of arterial occlusion and timing form onset to recanalization did not have an independent influence on survival. Non-survivors had a shorter hospitalization ( p < 0.001) but higher costs related to their hospitalization and outpatient care., Conclusion: The recanalization status, NIHSS score 24-h after EVT and sICH were predictors of early mortality in AIS patients treated with EVT., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Chen, Zhou, Yang, Mofatteh, Hu, Wei, Lai, Zeng, Yang, Yu, Chen, Sun, Wei, Nguyen, Baizabal-Carvallo and Liao.)

Background: Lower limb weakness is a long-recognized symptom in patients with Parkinson's disease (PD), described by James Parkinson in his seminal report on 'paralysis agitans'. However, little is known on the frequency, clinical correlations, and association with objective decrease in muscle strength in such patients., Objective: The objective of this study was to assess the frequency of objective and perceived lower limb weakness in patients with PD., Methods: We studied 90 consecutive patients with PD and 52 age-matched controls. We recorded clinical and demographic variables, as well as perceived weakness and allied abnormal lower limb sensations, including 'heavy legs', 'fatigued legs', and 'pain'. Symptoms consistent with restless legs syndrome were not considered. Lower limb strength was determined in both legs by means of the Medical Research Council scale, dynamometric (leg flexion) and weighting machine (leg pressure) measures., Results: Weakness and allied abnormal lower limb sensations were reported in 69% of patients with PD and 21% of healthy controls. Patients with PD had decreased leg pressure compared with healthy controls ( p  = 0.002). Among patients with PD, an association between perceived leg weakness (and allied sensations) and gait freezing ( p  = 0.001) was observed in the multivariate regression analysis; however, these variables only explained 30.4% of the variance. Moreover, PD patients with and without abnormal lower limb sensations had similar muscle strength by objective measurements., Conclusion: Perceived lower limb weakness and allied abnormal sensations are common in patients with PD. However, there is a dissociation between perceived weakness and objective muscle strength in the lower limbs. These abnormal sensations were mostly related to gait freezing but a causal association is questionable., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s), 2022.)

Background: Dystonic tics differ from clonic tics by their slower and more sustained nature. Dystonic tics are often present in patients with Tourette syndrome (TS) and other tic-disorders. However, their phenomenology and impact on overall impairment have not been extensively studied., Materials and Methods: We assessed clinical history and tic duration in video-recordings from patients with TS evaluated at our movement disorders clinic. Dystonic tics were defined as those lasting ≥ 1000 ms (ms)., Results: Of the total of 201 patients with TS, there were 156 with video-recordings suitable for tic duration analysis, of their tics, 57 (36.5%) of whom had dystonic motor tics, including 9 (5.7%) with dystonic phonic tics. Dystonic motor tics had a duration range between 1033 and 15,000 ms and dystonic phonic tics between 1132 and 17,766 ms. Patients with dystonic tics were older 24.4 vs. 16.5 years (P = 0.005) and had an older age at onset 12.9 vs. 7.2 years (P < 0.001), than patients without dystonic tics. The bivariate analysis showed an association between the presence of dystonic tics, greater tic severity and wider body distribution. The multivariate regression analysis showed a statistical association with older age at evaluation (P = 0.001), greater tic severity on video-recordings (P = 0.001) and co-occurrence with complex motor tics (P = 0.020). The presence of dystonic tics increased the risk for being considered for deep brain stimulation therapy, odds ratio: 15.7 (P = 0.002)., Conclusion: Dystonic tics, observed in about a third of patients with TS, are associated with increased severity of TS., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Background: Oculogyric tics are identified in a substantial proportion of patients with Tourette syndrome (TS) and related tic-disorders. Nevertheless, studies assessing its frequency and clinical correlations are lacking., Materials and Methods: We reviewed video-recordings and clinical history of 201 patients with TS and chronic motor/phonic tic disorder, diagnosed according to DSM-5., Results: Oculogyric tics presented in 22.4% of our patients. Transient upward gaze was the most common phenomenology in patients with simple oculogyric tics; whereas eye-closure followed by upward and lateral deviations was the most commonly observed phenomenon in patients with combined oculogyric tics. Oculogyric tics have a median duration of 799 ms (range 299-34,500 ms). Patients with oculogyric tics were younger (P = 0.023) and had a higher frequency of cranial tics (P = 0.037) compared to those without oculogyric tics. No differences in tic severity, frequency of attention-deficit/hyperactivity disorder, obsessive-compulsive disorder or use of dopamine receptor antagonists were observed in patients with and without oculogyric tics., Conclusions: Oculogyric tics are a common phenomenology in chronic tic disorders. They are more common in younger patients with TS and are markers of other cranial tics, but not of TS severity or comorbid neuropsychiatric syndromes., (© 2022. The Author(s) under exclusive licence to Belgian Neurological Society.)

Introduction: Tourette syndrome (TS) is characterized by the presence of motor and phonic tics, as well as a variety of behavioral co-morbidities. Self-injurious behavior (SIB) is one of the most serious manifestations of TS, but its pathophysiology is poorly understood., Methods: Consecutive patients with TS studied in a tertiary care center., Results: We identified a total of 34 patients (16.9%) with SIB from a cohort of 201 patients with TS. Most of these patients (n = 23, 11.4%) experienced self-directed damage; while others had outward-directed (n = 7, 3.5%) or tic-related SIB (n = 4, 2%). Compared to other patients with TS, those who manifested SIB (self- and outward-directed damage) were more likely to have tics involving shoulder (P = 0.046), trunk (P = 0.006), and arm (P = 0.017); as well as dystonic tics (P = 0.016); complex motor tics (P < 0.001), copropraxia (P = 0.045), complex phonic tics (P = 0.003), higher number of phonic tics (P = 0.001), verbalizations (P = 0.001), coprolalia (P = 0.006) and obsessive compulsive disorder (OCD) (P < 0.001) as determined by bivariate analysis. In the multivariate analysis only complex motor tics (P = 0.006), obsessive-compulsive behavior (P = 0.025) and greater severity of tics (P = 0.002) showed a statistically significant association with SIB. Patients with SIB had a greater probability of being selected for deep brain stimulation (DBS) therapy by the treating clinician (P = 0.01)., Conclusions: SIB is observed in about 17% of patients with TS. The presence of complex motor tics, OCD and greater severity of tics was related to the presence of SIB., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

Tremor is the most common movement disorder and there are numerous causes of tremor. In many individuals, tremor can be due to drugs. The most common drugs associated with tremor include amiodarone, selective serotonin (and norepinephrine) reuptake inhibitors (SSRIs/SNRIs), amitriptyline, lithium, valproate, β-adrenoceptor agonists, dopamine receptor antagonists, VMAT2 inhibitors, or drugs of abuse: ethanol, cocaine, etc. Drug-induced tremor usually resembles essential or parkinsonian tremor, depending on the offending drug; however, features such as unilateral, task-specific, position-dependent tremor or sudden onset, distractibility, entrainment and arrest with contralateral movements suggest etiologies such as dystonic or functional (psychogenic) tremor. Risk factors for drug-induced tremor include polypharmacy, male gender, older age, high doses and immediate-release preparations or reaching toxic levels of the offending drugs. Drug-induced tremor usually resolves once the offending medication is discontinued, however, persistent tremor may be observed in some cases (tardive tremor). In this manuscript, we discuss the most common causes of drug-induced tremor. This article is part of the Special Issue "Tremor" edited by Daniel D. Truong, Mark Hallett, and Aasef Shaikh., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: Tourette syndrome (TS) is a neurodevelopmental disorder characterized by the presence of motor and phonic tics. It is at least three times more common in males compared with females; however, the clinical phenomenology between sexes has not been fully examined. We aimed to contrast the clinical features between males and females with TS and chronic tic disorder., Methods: We studied 201 consecutive patients fulfilling the diagnostic criteria for TS, persistent (or chronic) motor and vocal tic disorder and provisional tic disorder that were considered within the TS spectrum disorder. We performed blinded evaluations of video-recordings and retrospectively reviewed the clinical charts of all patients., Results: Age ranges between 4 and 65 years. Males represented 77.6% of patients in the cohort. Overall, no differences were observed in the frequency, distribution and complexity of tics between sexes, except for a higher frequency of attention-deficit/hyperactivity disorder (ADHD) (P = .003) among males. Patients younger than 18-years old, in addition to a higher frequency of ADHD (P = .026), males had a statistically higher frequency of complex motor tics (P = .049) and earlier age at onset (P = .072) than females in the multivariate regression analysis. However, these differences were lost in patients older than 18 years, due to increased complexity of tics in females with aging., Conclusions: A sexual dimorphism was observed between patients with TS mainly before age of 18 years, suggesting an earlier onset of some types of tics and ADHD in males compared to females.

Parkinson's disease is neurodegenerative disorder with an initial robust response to levodopa. As the disease progresses, patients frequently develop dyskinesia and motor fluctuations, which are sometimes resistant to pharmacological therapy. In recent years, abnormalities in gut microbiota have been identified in these patients with a possible role in motor manifestations. Dysbiosis may reduce levodopa absorption leading to delayed "On" or "no-On" states. Among 84 consecutive patients with PD, we selected 14 with levodopa-induced dyskinesia and motor fluctuations with a Movement Disorders Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) part IV ≥ 8 points following a trial of pharmacological adjustment 2-3 months prior to study enrollment or adjustments in deep brain stimulation therapy. Patients received treatment with sodium phosphate enema followed by oral rifaximin and polyethylene glycol for 7 and 10 days, respectively. Evaluations between 14 to 21 days after starting treatment showed improvement in MDS-UPDRS-IV ( P = 0.001), including duration ( P = 0.001) and severity of dyskinesia ( P = 0.003); duration of medication "Off"-state ( P = 0.004); functional impact of motor fluctuations ( P = 0.047) and complexity of motor fluctuations ( P = 0.031); no statistical improvement was observed in "Off" dystonia ( P = 0.109) and total motor scores ( P = 0.430). Marked to moderate improvement in dyskinesia was observed in 57% of cases with blinded evaluation of videos. About 80% of patients perceived moderate to robust improvement at follow-up. A therapeutic strategy aimed at decontamination of intestines showed benefit in motor fluctuations and dyskinesia. Further studies should confirm and clarify the mechanism of improvement observed in these patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Baizabal-Carvallo, Alonso-Juarez and Fekete.)

Background: Tics are the hallmark of Tourette syndrome (TS). However, TS patients may have a particular vulnerability to develop other movement disorders (MDs), such as dystonia, chorea, stereotypy, and other hyperkinetic disorders that may be wrongly attributed to tics., Materials and Methods: We studied a cohort of 201 patients with motor and phonic tics associated with TS to determine if they have additional, co-existent, MDs., Results: There were 67 (33.3%) patients with comorbid non-tic MDs. Phenomenology-wise, piano-playing movements resembling chorea or myoclonus, were the most common non-tic movement, observed in 11% of cases, followed by stereotypies (8.0%), tremor, dystonia and parkinsonism, 5.0% each. Drug-induced was the most common etiology (6.0%), followed by functional movement disorders (5.0%) and tardive phenomena (5.0%). No clear etiology was identified in most patients. Piano-playing movements, were associated with a younger age at onset (P = 0.004) and younger age at presentation (P < 0.001). Patients with drug-induced movements and tardive phenomena had a lower frequency of craniofacial tics. FMDs, and idiopathic MDS showed no specific associations with TS. Tic severity was not a predictor of any co-existent MD., Conclusion: About a third of patients with TS present with comorbid MDs which should be differentiated and distinguished from tics as their etiopathogenesis and treatment may be different., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Competing Interests: None

Tremor and parkinsonism are recognized side effects of valproate; however, the relationship between rest tremor and other signs of parkinsonism has not been addressed in patients taking valproate. We studied a cohort of 125 consecutive patients treated with valproate due to epilepsy or migraine, evaluated with the Fahn-Tolosa-Marin Tremor Rating Scale (FTM-TRS). A total of 14 (11.2%) patients had rest tremor (bilateral n = 10, unilateral n = 4). Patients with rest tremor had significant higher scores in the FTM-TRS (P < 0.001), but only one was diagnosed with parkinsonism. Patients may have valproate-induced parkinsonism or exacerbated motor features of Parkinson's disease by valproate. The frequency of parkinsonism was 1.6% in this cohort and of 3% in the pooled data of 717 patients from previous reports. Rest tremor is observed in 11.2% of patients treated with valproate and is related to the burden of valproate-induced tremor, rather than the presence of parkinsonism.

Competing Interests: None

Background: Parkinson's disease (PD) is a neurodegenerative disorder characterized by motor impairment. Freezing of gait, impaired mobility and falls are common problems in these patients. We aimed to evaluate the effect of a novel therapy for these patients., Methods: We studied patients with moderate to severe freezing of gait who underwent antigravity treadmill training twice a week for 4 consecutive weeks with 50% reduction of body weight., Results: We enrolled 26 consecutive patients with PD, 19 completed the study. There were 10 males; mean age at evaluation was 72.7 ± 10.1 years. Compared to baseline, patients showed improvement in the Freezing of Gait Questionnaire ( p = 0.001); and a mean reduction of 7 s in the Timed Up & Go (TUG) test ( p = 0.004). Moderate or significant improvement in gait was reported by 84% of patients., Conclusions: Antigravity treadmill training improved freezing of gait and mobility in patients with PD.

Chorea is defined by the presence of abnormal, involuntary, continuous, random movements that results from a number of autoimmune, hereditary, vascular, metabolic, drug-induced and functional (psychogenic) causes. Chorea may present at all stages of life, from newborns to elderly individuals. While Huntington disease is the main suspicion in adults presenting with chorea, once a drug-induced or parkinsonian dyskinesia have been ruled out; Huntington disease exceptionally presents with chorea in children. Sydenham chorea is considered the most common cause of acute childhood-onset chorea, but its prevalence has decreased in Western countries. However, in younger children other etiologies such as dyskinetic cerebral palsy, anti-NMDAR receptor encephalitis, other autoimmune conditions, or mutations in NKX2-1, ADCY-5, FOXG1, GNAO1, GPR88, SLC2A1, SQSTM1, ATP8A2, or SYT-1 should be considered. In this manuscript, we review the main causes, diagnosis and management of chorea in children.

Parkinson's disease (PD) is the second most common neurodegenerative disorder. Despite its high frequency the etiology is still unclear; several lines of evidence show that an inflammatory process is implicated in the pathogenesis of this disorder; where activation of brain microglia plays a central role in the damage of dopaminergic neurons of the substantia nigra. Such inflammation has been attributed to the toxic effect of aggregated α-synuclein; however, evidence also implicates an altered gut microbiota (dysbiosis) through the systemic release of endotoxins such as lipopolysaccharide and other metabolic products. This exposure may be enhanced by increased permeability of the intestinal ("leaky gut") and the blood brain barrier; enhancing the entrance of microbiota-produced substances into the central nervous system. In this manuscript, we explore the evidence from clinical and basic science implicating microglia activation by gut dysbiosis and how this phenomenon may impact in the symptomatology and progression of PD., (Copyright © 2020 IBRO. Published by Elsevier Ltd. All rights reserved.)

Background: Functional gait disorders (FGDs) are relatively common in patients presenting for evaluation of a functional movement disorder (FMD). The diagnosis and classification of FGDs is complex because patients may have a primary FGD or a FMD interfering with gait., Methods: We performed a detailed evaluation of clinical information and video recordings of gait in patients diagnosed with FMDs., Results: We studied a total of 153 patients with FMDs, 68% females, with a mean age at onset of 36.4 years. A primary FGD was observed in 39.2% of patients; among these patients, 13 (8.5%) had an isolated FGD (a gait disorder without other FMDs). FMDs presented in 34% of patients with otherwise normal gait. Tremor was the most common FMD appearing during gait, but dystonia was the most common FMD interfering with gait. Patients with FGD had a higher frequency of slow-hesitant gait, astasia-abasia, bouncing, wide-based gait and scissoring compared with patients with FMDs occurring during gait. Bouncing gait with knee buckling was more frequently observed in patients with isolated FGD (P = 0.017). Patients with FGDs had a trend for higher frequency of wheelchair dependency (P = 0.073) than those with FMDs interfering with gait., Conclusions: Abnormal gait may be observed as a primary FGD or in patients with other FMDs appearing during gait; both conditions are common and may cause disability.

Background: Functional neurological disorders are generally more common in females than males, but the reason for this gender difference is not well understood., Objectives: In this study, we aim to compare the clinical and demographic features of functional movement disorders (FMDs) between males and females., Methods: We examined clinical data and video-recordings of patients with FMDs evaluated at the Baylor College of Medicine Movement Disorders Clinic., Results: Of the 196 patients with FMDs, males represented only 30% (n = 59) of the entire cohort. Men had an older age at onset: 40.5 versus 34.1 years ( P = 0.026) and an older age at evaluation: 43.8 versus 38.1 years ( P = 0.041) compared to women. Functional dystonia was more frequently observed in women: 47.5 versus 20.3% ( P < 0.001), but there was a trend for higher frequency of functional gait disorder in men: 44 versus 30% ( P = 0.056). Females were particularly over-represented (73.7%) in children and adolescents; but the genders were equally represented in patients aged ≥50 years., Conclusions: Female patients are over-represented in FMDs, except in individuals aged ≥50 years. Compared to female patients, males with FMDs present later in life and are less likely to have functional dystonia., Competing Interests: The authors report no sources of funding and no conflicts of interest., (© 2019 International Parkinson and Movement Disorder Society.)

Background: Hand deformities have been recognized since the 19th century as part of the postural abnormalities observed in Parkinson's disease (PD). However, their pathogenesis and clinical correlations are poorly understood., Methods: We evaluated 104 hands of 52 consecutive patients with PD by high-resolution photographs taken from the radial aspect of each hand; the degree of flexion of the 2nd metacarpophalangeal (MCP) joint was measured by software. The presence of classical striatal hand deformity (CSHD) was also evaluated, defined as MCP flexion, proximal interphalangeal joint extension, and distal interphalangeal joint flexion., Results: Patients with PD had a mean age of 63.3 ± 12.7 years, and 29 (56%) were male. The degree of MCP joint flexion in both hands showed moderate correlation with the MDS-UPDRS-III motor score (r = 0.518, P < 0.001), mainly related to ipsilateral rigidity and ipsilateral bradykinesia scores, and fair correlation with the Hoehn-Yahr stage. A CSHD only correlated with a younger age at onset of PD (P = 0.049). These hand deformities were not markers of dyskinesia, levodopa equivalent dose, or cognitive dysfunction., Conclusions: Metacarpophalangeal joint flexion is the most common hand deformity in PD and correlates with rigidity and bradykinesia. A CSHD was only related to a younger age at onset., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Functional movement disorders (FMDs), known over time as "hysteria", "dissociative", "conversion", "somatoform", "non-organic" and "psychogenic" disorders, are characterized by having a voluntary quality, being modifiable by attention and distraction but perceived by the patient as involuntary. Although a high prevalence of depression and anxiety is observed in these patients, a definitive role of psychiatric disorders in FMDs has not been proven, and many patients do not endorse such manifestations. Stressful events, social influences and minor trauma may precede the onset of FMDs, but their pathogenic mechanisms are unclear. Patients with FMDs have several abnormalities in their neurobiology including strengthened connectivity between the limbic and motor networks. Additionally, there is altered top-down regulation of motor activities and increased activation of areas implicated in self-awareness, self-monitoring, and active motor inhibition such as the cingulate and insular cortex. Decreased activation of the supplementary motor area (SMA) and pre-SMA, implicated in motor control and preparation, is another finding. The sense of agency defined as the feeling of controlling external events through one's own action also seems to be impaired in individuals with FMDs. Correlating with this is a loss of intentional binding, a subjective time compression between intentional action and its sensory consequences. Organic and functional dystonia may be difficult to differentiate since they share diverse neurophysiological features including decreased cortical inhibition, and similar local field potentials in the globus pallidus and thalamus; although increased cortical plasticity is observed only in patients with organic dystonia. Advances in the pathogenesis and pathophysiology of FMDs may be helpful to understand the nature of these disorders and plan further treatment strategies., (Copyright © 2019 Elsevier Inc. All rights reserved.)

A number of neurological syndromes have been described in patients with positive serum antibodies (Abs) against the enzyme glutamic acid decarboxylase (GAD), the rate limiting step in the synthesis of GABA (γ-aminobutyric acid). These disorders include: classical stiff-person syndrome and variants, cerebellar ataxia, limbic and extra-limbic encephalitis, nystagmus/oculomotor dysfunction, drug-resistant epilepsy, paraneoplastic stiff-person syndrome and progressive encephalopathy with rigidity and myoclonus (PERM), the latter two are mainly related to amphiphysin and the glycine receptor Abs respectively; but patients may also have positive GAD-Abs. Although observations are consistent with an autoimmune response in these patients and there is evidence of GABAergic dysfunction in some cases; the pathogenic role of GAD-Abs in the nervous system has not been clarified and it is a matter of debate. The diagnosis of these syndromes is based on clinical grounds plus the presence of GAD-Abs in serum and CSF with demonstration of intrathecal secretion. Although some presentations may be negative for GAD-Abs, such as stiff-person syndrome; positive GAD-Abs are required for the diagnosis in patients with cerebellar ataxia, encephalitis, and epilepsy. Immunotherapy is required for most patients. Intravenous immunoglobulins, oral or IV steroids and plasma exchange are considered the first line options, aimed to induce remission, but chronic immunosuppression is usually required. Symptomatic therapy should also be provided, aimed to control muscle spasms, seizures, delirium, etc. Prognosis varies among patients; but it is considered intermediate between that of patients with neurological syndromes associated with neural Abs against membrane antigens and those with onconeural Abs., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Hand deformities are well-known abnormalities observed in patients with Parkinson's disease (PD). We determined the frequency and diagnostic accuracy of hand deformities in PD. We studied 44 consecutive patients with PD, 44 age- and gender-matched normal controls and 22 patients with essential tremor (ET). By means of photographs taken in both hands of all participants, the degree of metacarpophalangeal (MCP) joint flexion was quantified by software and by blinded evaluations using a semiquantitative scale from the radial aspect, we grouped hands into four grades. The presence of classical striatal hand deformity (CSHD), defined as MCP joint flexion, proximal interphalangeal joint extension and distal interphalangeal joint flexion was also evaluated. Patients with PD had a higher frequency of MCP joint flexion and CSHD compared to normal controls and patients with ET. Mean MCP joint flexion was higher in both hands in patients with PD: 20.8° vs. normal controls (3.3°-3.9°) and patients with ET (2.8°-6.3°), P = 0.001. Concordance between evaluators for MCP joint flexion was fair: κ = 0.34 (P < 0.001), but poor for CSHD: κ = 0.142-0.235 (P < 0.05). A right hand MCP joint flexion of 12.5° and left hand of 10.5°, showed similar sensitivity (0.70) and specificity (between 0.75 and 0.80) than any degree of MCP joint flexion for the diagnosis of PD. CSHD had a sensitivity (0.60-0.80) and specificity (0.78-0.98) for the diagnosis of PD. Hand deformities are commonly observed in patients with PD, they may aid in the diagnosis of PD when compared to normal controls and patients with ET.

Background: Tremor frequently occurs in patients taking valproate and may resemble that observed in patients with essential tremor (ET). We aimed to compare the distribution, severity, and functional impact of both types of tremor., Methods: Among 118 consecutive individuals taking valproate, we identified 28 patients with valproate-induced tremor severe enough to require pharmacological treatment; through evaluations using the Clinical Rating Scale for Tremor (CRST), they were compared with 29 consecutive patients with ET., Results: Patients with valproate-induced tremor were significantly younger than those with ET: 35.6 ± 15.4 vs 58.8 ± 20.5 years (P < .001), with a shorter evolution time of tremor (P < .001). Total CRST scores did not differ between groups (P = .164), neither in subscores of Parts A (P = .321), B (P = .094), and C (P = .386). Patients with valproate-induced tremor had a higher proportion of postural tongue tremor compared with ET patients: 22 (79%) vs 15 (52%) (P = .034). A frank tremor axis in the Archimedes spirals was observed in a higher proportion of patients with ET compared to valproate-induced tremor: 31% vs 3.6% (P = .006)., Conclusions: Patients with valproate-induced tremor requiring pharmacological treatment have similar distribution, CRST scores, and functional impact than those with ET., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Several neurological disorders have been described in patients with autoimmunity associated with GAD antibodies. Among these disorders, nystagmus and oculomotor dysfunction are increasingly recognized, although they have been rarely reported isolated or as the main manifestation of anti-GAD autoimmunity. Moreover, therapeutic approaches for such patients are unclear. Here we present a 44-year-old man with disabling oscillopsia secondary to downbeat nystagmus, abnormal saccades, ocular pursuit and optokinetic nystagmus, as well as mild gait ataxia and cerebellar atrophy associated with high serum GAD antibodies with intrathecal secretion of such antibodies. The patient did not have clinical benefit with plasma exchange, but had a robust symptomatic improvement with cyclophosphamide. We discuss the possible pathogenic role of GAD antibodies in nystagmus and the role of immunotherapy in these patients., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens. Anti-NMDA receptor encephalitis is one of the most common and may present with a variety of MDs, including: chorea, stereotypies, dystonia and myorhythmia. The recognition of other abnormal motor phenomena such as "faciobrachial dystonic seizures" and neuromyotonia, observed in patients with LGI1 and Caspr-2 antibodies, is important because they may herald the onset of overt limbic encephalitis. Autoimmunity directed against the intracellular enzyme glutamic acid decarboxylase usually presents with MDs, most commonly stiff-person syndrome or cerebellar ataxia. Chorea may be observed in rheumatologic disorders such as systemic lupus erythematosus or antiphospholipid syndrome. Disorders with uncertain autoimmune mechanisms such as Hashimoto's encephalitis and idiopathic opsoclonus-myoclonus syndrome commonly present with tremor, myoclonus and ataxia. A rapid diagnosis of an autoimmune disorder, which typically presents with subacute onset, is critical as early therapeutic intervention improves long-term prognosis and may be life-saving. Treatment usually involves some form of immunotherapy and symptomatic therapy of the abnormal movements with dopamine depleters, dopamine receptor antagonists, or GABAergic drugs. Detection and removal of an underlying tumor is essential for optimal outcome., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Tremor is a known side-effect of anticonvulsants, particularly of valproate. However, there is a dearth of information regarding detailed clinical features and functional impact of valproate-induced tremor., Methods: We studied a cohort of patients treated with anticonvulsants for neurological disorders, through blinded evaluations using the Clinical Rating Scale for Tremor (CRST); we compared the frequency, severity and functional impact of drug-induced tremor between patients treated with valproate and those treated with other anticonvulsants., Results: From a cohort of 218 consecutive patients, 171 were fully evaluated; 118 patients were taking valproate alone or combined with other anticonvulsants and 53 patients were taking other anticonvulsants. Mean age (±SD) at evaluation of the cohort was 32 ± 13 years, females represented 55.6% of cases. Tremor was more frequently observed in patients taking valproate particularly postural upper limb tremor: 49% vs. 15% (right-side) (P < 0.001) and 48.3% vs. 13.2% (left-side), (P < 0.001); had a higher total CRST score: 12.14 vs. 3.06 (P < 0.001), and required more frequently treatment for drug-induced tremor: 23.7% vs. 5.6% (P=0.005) compared with patients taking other anticonvulsants. Among 118 patients taking valproate, women had a higher total CRST score compared with men: 14.54 ± 14.9 vs. 9.56 ± 9.55 (P=0.034). A weak correlation between the total CRST score, dose per Kg of valproate and serum levels of valproate were observed., Conclusions: Tremor is frequently observed in patients taking valproate and is severe enough to require treatment in about 24% of cases., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Several neurological syndromes have been recognized associated to GAD antibodies. Among those disorders, cerebellar ataxia (CA) is one of the most common, along with stiff-person syndrome. Patients with GAD associated CA present with a progressive pancerebellar syndrome, with a subacute or chronic evolution, along with other neurological manifestations such as stiffness, oculomotor dysfunction, epilepsy, and cognitive dysfunction. These symptoms may be preceded by the so-called "brainstem attacks", where manifestations consistent with transient dysfunction of the brainstem may be observed. These patients frequently have extra-neurologic autoimmune manifestations such as diabetes mellitus type 1, polyendocrine autoimmune syndrome, pernicious anemia, vitiligo, etc. A proportion of patients may present with an underlying neoplasia, but the course is less aggressive than in those patients with classical paraneoplastic CA with onconeural antibodies. The diagnosis is based on the present of high serum and CSF titers of GAD antibodies, with intrathecal production of such antibodies. Treatment is aimed to decrease the immunological response with intravenous immunoglobulin, steroids, rituximab and oral immunosuppressive drugs. A subacute presentation and rapid initiation of immunotherapy seem to be the predictors of a favorable clinical response.

Functional (psychogenic) movement disorders (FMDs) may present with a broad spectrum of phenomenology including stereotypic movements. We aimed to characterize the phenomenology of functional stereotypies and compare these features with those observed in 65 patients with tardive dyskinesia (TD). From a cohort of 184 patients with FMDs, we identified 19 (10.3%) with functional stereotypies (FS). There were 15 women and 4 men, with a mean age at onset of 38.6 ± 17.4 years. Among the patients with FS, there were 9 (47%) with orolingual dyskinesia/stereotypy, 9 (47%) with limb stereotypies, 6 (32%) with trunk stereotypies, and 2 (11%) with respiratory dyskinesia as part of orofacial-laryngeal-trunk stereotypy. These patients showed signs commonly seen in FMDs such as sudden onset (84%), prominent distractibility (58%), and periods of unexplained improvement (84%) that were not reported in patients with TD. Besides a much lower frequency of exposure to potential offending drugs, patients with FS differed from those with classic TD by a younger age at onset, lack of self-biting, uncommon chewing movements, more frequent lingual movements without mouth dyskinesia, and associated functional tremor and abnormal speech. Lack of self-biting showed the highest sensitivity (1.0) and abnormal speech showed the highest specificity (0.9) for the diagnosis of functional orolingual dyskinesia. FS represent part of the clinical spectrum of FMDs. Clinical and demographic features are helpful in distinguishing patients with FS from those with TD.