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Background: Congenital Zika syndrome causes a spectrum of neurological symptoms with varying effects on function that require different therapeutic strategies. To date, this spectrum of effects and its clinical implications have not been completely described. We describe the neurological examination findings in toddlers and preschoolers, including predominant symptom complexes and comorbidities., Methods: This study is a case-series neurological evaluation of 75 children with congenital Zika syndrome in Campina Grande, Brazil. The study is part of a cohort of children with congenital Zika syndrome that started in 2015 and is still ongoing. Children with Zika virus infection detected during pregnancy (mothers exhibited rash and were followed and diagnosed by fetal ultrasound abnormalities or RT-PCR) or through microcephaly screening after birth, using Intergrowth 21 guidelines, were selected by laboratory and radiological criteria. Children were examined during a 10-day period in September, 2018, and underwent neurological interview, examination, and assessment of functional outcomes and comorbidities. Children were divided in groups of predominant corticospinal or neuromuscular clinical signs and the associations between these groups and clinical comorbidities were assessed., Findings: All of the children recruited to the study from Nov 29, 2015 to Nov 30, 2017 had imaging correlates of congenital Zika syndrome. Children were assigned to groups depending on the signs exhibited, either corticospinal or neuromuscular, with or without dyskinetic signs. 75 children completed the evaluation, 38 (51%) girls and 37 (49%) boys. Median age was 33 months (range 26-40 months; IQR 29-34). Microcephaly was present at birth in 56 (75%) children, and 19 (25%) children were born with normal head circumference, 15 of whom later developed microcephaly. Neurological examination grouped four children as having isolated dyskinetic signs, 48 children were assigned to the corticospinal group and 23 into the neuromuscular group. Dyskinetic findings were present in 30 (40%) children, either alone (four [5%]) or combined with corticospinal (19 [40%] of 48) or neuromuscular (seven [30%] of 23) findings. Comorbidities were highly prevalent, and the neuromuscular group had worse functional outcomes, evaluated by gross motor function (p=0·026), manual abilities (p=0·0013), and communication function (p<0·0005) classification scales, than the corticospinal group, whereas pneumonia (p<0·0005) and urinary tract infections (p<0·0005) were more frequent in the corticospinal group. Cortical hyperexcitability was supported by several clinical correlates, such as early onset epilepsy, persistence of primitive reflexes, and dystonia., Interpretation: We describe distinct neurological profiles in the congenital Zika syndrome spectrum, with functional outcomes tending to correlate with these groups. The clinical division of children based on the disease signs proposed here is supported by the literature on central and peripheral nervous system pathology in congenital Zika syndrome. The high prevalence of dyskinetic symptoms merits special attention., Funding: Brazilian National Council for Scientific and Technological Development and by the Coordination for the Improvement of Higher Education Personnel., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Conventional Structural Magnetic Resonance Imaging (sMRI) is a mainstay in Cerebral Palsy (CP) diagnosis., Aims: A systematic literature review was performed with the aim to investigate the relationship between structural brain lesions identified by sMRI and motor outcomes in children with CP., Methods: Fifty-eight studies were included. The results were analysed in terms of population characteristics, sMRI (classified according to Krägeloh-Mann & Horber, 2007), gross and fine motor function and their interrelation., Outcomes: White matter lesions were the most common brain lesion types and were present in 57.8 % of all children with uCP, in 67.0 % of all children with bCP and in 33 % of the group of mixed subtypes. Grey matter lesions were most frequently registered in children with dyskinesia (n = 42.2 %). No structural anomalies visualized by sMRI were reported in 5.7 % of all cases. In all lesion types, an equal distribution over the different gross motor function classification system categories was present. The included studies did not report sufficient information about fine motor function to relate these results to structural imaging., Conclusions and Implications: The relationship between brain structure and motor outcome needs to be further elucidated in a representative cohort of children with CP, using a more standardized MRI classification system., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Alterations in glenohumeral and scapulothoracic kinematics have been theorized to contribute to rotator cuff pathology by impacting the magnitude of the subacromial space., Objective: The purpose of this review is to summarize what is currently known about the relationship between shoulder kinematics and subacromial proximities., Conclusions: A variety of methods have been used to quantify subacromial proximities including photographs, MR imaging, ultrasonography, and single- and bi-plane radiographs. Changes in glenohumeral and scapulothoracic kinematics are associated with changes in subacromial proximities. However, the magnitude and direction of a particular motion's impact on subacromial proximities often vary between studies, which likely reflects different methodologies and subject populations. Glenohumeral elevation angle has been consistently found to impact subacromial proximities. Plane of humeral elevation also impacts subacromial proximities but to a lesser degree than the elevation angle. The impact of decreased scapulothoracic upward rotation on subacromial proximities is not absolute, but instead depends on the angle of humerothoracic elevation. The effects of scapular dyskinesis and humeral and scapular axial rotations on subacromial proximities are less clear. Future research is needed to further investigate the relationship between kinematics and subacromial proximities using more homogenous groups, determine the extent to which compression and other factors contribute to rotator cuff pathology, and develop accurate and reliable clinical measures of shoulder motion., (Copyright © 2019 Associação Brasileira de Pesquisa e Pós-Graduação em Fisioterapia. Publicado por Elsevier Editora Ltda. All rights reserved.)

Patients with advanced Parkinson's disease regularly experience unstable motor states. Objective and reliable monitoring of these fluctuations is an unmet need. We used deep learning to classify motion data from a single wrist-worn IMU sensor recording in unscripted environments. For validation purposes, patients were accompanied by a movement disorder expert, and their motor state was passively evaluated every minute. We acquired a dataset of 8,661 minutes of IMU data from 30 patients, with annotations about the motor state (OFF,ON, DYSKINETIC) based on MDS-UPDRS global bradykinesia item and the AIMS upper limb dyskinesia item. Using a 1-minute window size as an input for a convolutional neural network trained on data from a subset of patients, we achieved a three-class balanced accuracy of 0.654 on data from previously unseen subjects. This corresponds to detecting the OFF, ON, or DYSKINETIC motor state at a sensitivity/specificity of 0.64/0.89, 0.67/0.67 and 0.64/0.89, respectively. On average, the model outputs were highly correlated with the annotation on a per subject scale (r = 0.83/0.84; p < 0.0001), and sustained so for the highly resolved time windows of 1 minute (r = 0.64/0.70; p < 0.0001). Thus, we demonstrate the feasibility of long-term motor-state detection in a free-living setting with deep learning using motion data from a single IMU.

Aim: To assess the responsiveness, concurrent validity, and feasibility of the Dyskinesia Impairment Scale (DIS) in non-ambulatory patients with dyskinetic cerebral palsy (CP)., Method: The study is a secondary analysis of data collected in the IDYS trial, a randomized controlled trial on the effects of intrathecal baclofen (ITB). The DIS and Barry-Albright Dystonia Scale (BADS) were conducted at baseline and after 3 months of ITB or placebo treatment. Responsiveness was assessed by comparing the effect sizes and correlation of change after treatment between the DIS and BADS. Concurrent validity was evaluated by assessing the correlations between scales. Feasibility was evaluated for each DIS item by the number of participants who successfully accomplished the item., Results: Thirty-three non-ambulatory patients (9 females, 24 males) with dyskinetic CP (ITB-treated: n=17, mean [SD] age: 14y 1mo [4y 1mo]; placebo-treated: n=16, mean [SD] age: 14y 7mo [4y]) were included in the study. The effect sizes for BADS and DIS were similar in The ITB-treated group (-0.29 and -0.22 respectively). Changes after treatment on the DIS dystonia subscale correlated with changes on the BADS (r=0.64; p<0.001). The DIS dystonia subscale and BADS correlated at baseline and follow-up (r=0.78; p<0.001 and r=0.79; p<0.001). Not all DIS activity items could be performed in this sample of patients., Interpretation: For non-ambulatory patients with dyskinetic CP, the responsiveness of the DIS equalled the responsiveness of BADS. Concurrent validity was adequate. Feasibility for activity items was restricted in patients with severe dyskinetic CP., What This Paper Adds: The Dyskinesia Impairment Scale (DIS) and Barry-Albright Dystonia Scale showed similar responsiveness in non-ambulatory patients with dyskinetic cerebral palsy (CP). No floor or ceiling effect was observed for DIS in non-ambulatory participants. The concurrent validity of DIS was adequate in non-ambulatory participants. Patients with dyskinetic CP in Gross Motor Function Classification System levels IV and V could not perform all DIS activity items., (© 2019 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.)

Scapular dyskinesis is observed in 61% of overhead athletes (Burn et al., 2016). For most of them, it remains asymptomatic. However, scapular dyskinesis is considered a risk factor for shoulder injury by some authors (Clarsen et al., 2014). The aim of this study is to explore the effectiveness of kinesiotaping in modifying scapular kinematics and peri-scapular muscle activity in dyskinetic athletes. The 3-dimensional position and orientation of the scapula as well as the activation of upper trapezius, lower trapezius and serratus anterior were recorded in twenty asymptomatic athletes during shoulder movements (flexion and abduction), in loaded and unloaded conditions and in three circumstances (standard, kinesiotaping 1, kinesiotaping 2). A significant decrease between 9 and 12% in upper trapezius activity was observed with kinesiotaping 1 and 2. Lower trapezius activity was slightly increased with kinesiotaping 1 while it was significantly decreased about 15-20% with kinesiotaping 2. No change was observed in serratus anterior activity, for either kinesiotaping 1 or 2. Considering scapular kinematics, both kinesiotaping 1 and 2 significantly increased posterior tilt and upward rotation. External rotation was decreased with kinesiotaping 2, in comparison to standard condition. Kinesiotaping, and especially taping 1, seems to be an effective method for changing periscapular muscle activity and scapular kinematics., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Objective: This article describes associated impairments in children with cerebral palsy (CP) and its subtypes., Method: Children born between 1990 and 2006 recorded in the Surveillance of Cerebral Palsy in Europe common database were studied. An "impairment index" characterized severity of impairments and their combinations., Results: Amongst the 11,015 children analyzed, 56% ( n  = 5,968) could walk unaided, 54% (4,972) had normal or near-normal intellect (intelligence quotient ≥ 70). Except for ataxic CP, associated impairments were less frequent when walking ability was preserved. The impairment index was low (walking unaided and normal or near-normal intellect) in 30% of cases; 54% ( n  = 1,637) in unilateral spastic, 24% ( n  = 79) in ataxic, 18% ( n  = 913) in bilateral spastic, and 7% ( n  = 50) in dyskinetic CP. Around 40% had a high impairment index (inability to walk and/or severe intellectual impairment ± additional impairments)-highest in dyskinetic (77%, n  = 549) and bilateral spastic CP (54%, n  = 2,680). The impairment index varied little in birth weight and gestational age groups. However, significantly fewer cases in the birth weight group ≤ 1,000 g or gestational age group ≤ 27 weeks had a low impairment index compared to the other birth weight and gestational age groups (23 and 24% vs. between 27 and 32%)., Conclusion: Thirty percent of the children with CP had a low impairment index (they were able to walk unaided and had a normal or near-normal intellect). Severity in CP was strongly associated to subtype, whereas the association was weak with birth weight or gestational age., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim: To assess test-retest reliability of the Dyskinesia Impairment Scale (DIS) in children and young adults with dyskinetic cerebral palsy (CP)., Method: Dystonia and choreoathetosis were assessed in 15 participants with dyskinetic CP (13 males, 2 females; age range 5-22y, mean 14y, SD 4y) using the DIS in two separate sessions over 7 days. Exclusion criteria were changes in muscle relaxant medication within the previous 3 months, orthopaedic or neurosurgical interventions within the previous year, and spinal fusion. Intraclass correlation coefficient, confidence intervals (CI), standard error of measurement, and the minimal detectable difference (MDD) were determined for test-retest reliability., Result: Intraclass correlation coefficients of the DIS, the dystonia subscale of the DIS, and the choreoathetosis subscale of the DIS were 0.98 (95% CI 0.94-0.99), 0.97 (95% CI 0.92-0.99), and 0.96 (95% CI 0.90-0.99). The standard error of measurement and MDD were 2.6% and 7.2%., Interpretation: The DIS is a reliable tool to assess dystonia and choreoathetosis; it remains stable over time in children and young adults with dyskinetic CP. These results add to the current evidence for good clinimetric properties of the DIS., What This Paper Adds: The Dyskinesia Impairment Scale (DIS) shows stability in scoring dystonia and choreoathetosis. The total DIS score and dystonia and choreoathetosis subscales are clinically useful., (© 2019 Mac Keith Press.)

Aim: To review orofacial disabilities and their consequences in children with Moebius syndrome (MBS)., Method: We retrospectively analysed the records of 32 patients (21 males, 11 females) with non-progressive bilateral facial and abducens palsies who had been examined before 6 months of age., Results: All facial muscles were severely involved in 17 patients; in the 15 others, partial movements were found in the lower face. Most patients (n=24) were unable to smile. Patients frequently presented with congenital trismus (n=20) and drooling (n=18). Additional palsies involved cranial nerves IX and X (n=18) and XII (n=25). Sucking was absent or weak in 30 patients; swallowing was impaired in 25. During the first month of life, feeding disorders were graded as severe/moderate in 25. Respiratory complications occurred in 17. Severe feeding disorders were associated with congenital trismus (p=0.01) and with cranial nerve IX and X palsy (p=0.01). Growth failure between 1 and 6 months of age, followed by catch-up growth between 6 and 12 months, was observed in 20 patients. Between 2 and 5 years of age, 25 out of 32 patients attained normal oral diet and 28 out of 29 showed normal growth., Interpretation: Children with MBS frequently require adjusted therapeutic options to prevent failure to thrive. Congenital trismus, cranial nerve IX and X palsy, and laryngeal-tracheal dysfunctions are predictors of severe feeding disorders., What This Paper Adds: Moebius syndrome frequently induces reduced oral intake and early failure to thrive. Normal oral diet and growth parameters are attained at 2 to 5 years of age. Congenital trismus, pharyngeal palsy, and laryngeal disorders predict dysphagia., (© 2019 Mac Keith Press.)

Immune response against neuronal and glial cell surface and cytosolic antigens is an important cause of encephalitis. It may be triggered by activation of the immune system in response to an infection (para-infectious), cancer (paraneoplastic), or due to a patient's tendency toward autoimmunity. Antibodies directed toward neuronal cell surface antigens are directly pathogenic, whereas antibodies with intracellular targets may become pathogenic if the antigen is transiently exposed to the cell surface or via activation of cytotoxic T cells. Immune-mediated encephalitis is well recognized and may require intensive care due to status epilepticus, need for invasive ventilation, or dysautonomia. Patients with immune-mediated encephalitis may become critically ill and display clinically complex and challenging to treat movement disorders in over 80% of the cases (Zhang et al. in Neurocrit Care 29(2):264-272, 2018). Treatment options include immunotherapy and symptomatic agents affecting dopamine or acetylcholine neurotransmission. There has been no prior published guidance for management of these movement disorders for the intensivist. Herein, we discuss the immune-mediated encephalitis most likely to cause critical illness, clinical features and mechanisms of movement disorders and propose a management algorithm.

Objective: Adverse life events have been associated with exacerbating multiple sclerosis (MS) symptoms, but results have been variable, raising the question on the role of other psychological factors. This study examined the role of psychological resilience and vulnerability as mediators between adverse life events on MS symptoms., Methods: Participants with MS (N = 1239) were aged 18 to 81 years (mean [SD] = 45.6 [10.4] years), and 84.5% were female. MS symptoms were measured by the modified Fatigue Severity Scale, modified Fatigue Assessment Scale, Motor Dysfunction Assessment Scale, Paraesthesiae Spell Duration Scale, and the Paraesthesiae Cumulative Duration Scale. Psychological measures included the Connor-Davidson Resilience Scale, Resilience Scale for Adults, Psychological Vulnerability Scale, the vulnerability section of the Defence Style Questionnaire, and the Adverse Life Events Assessment Scale. Regression analyses and structural equation modeling were performed., Results: Adverse life events during the preceding 60 days were associated with fatigue, motor dysfunction, and paresthesia, but with small effect sizes (β from 0.07 to 0.15; p ≤ .014). A structural equation model by which resilience mediated less and vulnerability more MS symptoms after adverse life events during the preceding 60 days showed a statistically significant fit with the data of a moderate to good degree (p < .001; goodness-of-fit statistic = 0.725; root mean square error of approximation = 0.047). Vulnerability played a markedly larger role than did resilience., Conclusion: The results suggest that psychological resilience and vulnerability play mediating roles in the relation between adverse life events and MS symptoms, but other psychological factors also need to be investigated.

Background: In Parkinson's disease (PD) patients, the factors related to weight loss remain unclear., Objective: To investigate determinants of low body mass index (BMI) in PD patients., Methods: We identified factors associated with low BMI in PD patients in a multicenter case-control study. A total of 435 PD patients and 401 controls were included., Results: The mean BMI was significantly lower in PD patients than in controls (22.0±3.4 kg/m2 vs. 25.4±4.3 kg/m2), with an adjusted odds ratio (AOR) of 3.072 (95% CI, 2.103-4.488; p < 0.001) for low BMI (<22 kg/m2) in PD. Compared to the high-BMI PD group (>22 kg/m2), the low-BMI PD group (<22 kg/m2) had more women; a longer disease duration; higher revised Movement Disorder Society Unified PD Rating Scale (MDS-UPDRS) II and IV scores; an increased levodopa equivalent dose (LED); and increased constipation, visual hallucination, dysphagia, dyskinesia and wearing off rates. There were no between-group differences in depression, anhedonia, apathy, sleep problems and daytime sleepiness. Multivariable analysis showed that visual hallucination (AOR, 2.408; 95% CI, 1.074-5.399; p = 0.033) and the MDS-UPDRS IV (AOR, 1.155; 95% CI, 1.058-1.260; p = 0.001) contributed to low BMI after controlling for clinical factors. In a second model, visual hallucination (AOR, 2.481; 95% CI, 1.104-5.576; p = 0.028) and dyskinesia (sum of the MDS-UPDRS 4.3-4.6) (AOR, 1.319; 95% CI, 1.043-1.668; p = 0.021) significantly contributed to low BMI., Conclusion: PD patients were 3 times more likely than healthy controls to have a low BMI. Motor complications, particularly dyskinesia, and visual hallucination were significantly associated with low BMI in PD patients.

Introduction: Functional motor disorders are often delineated according to the dominant motor symptom. In a large cohort, we aimed to find if there were differences in demographics, mode of onset, pain, fatigue, depression and anxiety and levels of physical functioning, quality of life and social adjustment between patients with different dominant motor symptoms., Methods: Baseline data from the Self-Help and Education on the Internet for Functional Motor Disorders Trial was used. Patients were divided into dominant motor symptom groups based on the diagnosis of the referring neurologist. Data on the above topics were collected by means of an online questionnaire and compared between groups using parametric and nonparametric statistics., Results: In 160 patients a dominant motor symptom could be determined, 31 had tremor, 45 myoclonus, 23 dystonia, 30 paresis, 31 gait disorder. No statistical differences between groups were detected for demographics, mode of onset and severity of pain, fatigue, depression and anxiety. Physical functioning was worse in the gait disorder group (median 20, IQR 25) compared to tremor (50 (55), p = 0.002) and myoclonus (50 (52), p = 0.001). Work and social adjustment was less impaired in the myoclonus group (median 20, IQR 18) compared to gait disorder (median 30, IQR18, p < 0.001) and paresis (28, IQR 10, p = 0.001). Self-report showed large overlap in motor symptoms., Conclusion: No differences were detected between groups of functional motor symptoms, regarding demographics, mode of onset, depression, anxiety, pain and fatigue. The large overlap in symptoms contributes to the hypothesis of shared underlying mechanisms of functional motor disorders., Competing Interests: Declaration of competing interest None., (Copyright © 2019. Published by Elsevier Ltd.)

Parkinson's disease (PD) causes both motor and non-motor symptoms, which can partially be reversed by dopamine therapy. These symptoms as well as the effect of dopamine may be explained by distinct alterations in whole-brain architecture. We used functional connectivity (FC) and in particular resting state network (RSN) analysis to identify such whole-brain alterations in a frequency-specific manner. In addition, we hypothesized that standard dopaminergic medication would have a normalizing effect on these whole brain alterations. We recorded resting-state EEGs of 19 PD patients in the medical OFF and ON states, and of 12 healthy age-matched controls. The PD patients were either of akinetic-rigid or mixed subtype. We extracted RSNs with independent component analysis in the source space for five frequency bands. Within the sensorimotor network (SMN) the supplementary motor area (SMA) showed decreased FC in the OFF state compared to healthy controls. This finding was reversed after dopamine administration. Furthermore, in the OFF state no stable SMN beta component could be identified. The default mode network showed alterations due to PD independent of the medication state. The visual network was altered in the OFF state, and reinstated to a pattern similar to healthy controls by medication. In conclusion, PD causes distinct RSN alterations, which are partly reversed after levodopa administration. The changes within the SMN are of particular interest, because they broaden the pathophysiological understanding of PD. Our results identify the SMA as a central network hub affected in PD and a crucial effector of dopamine therapy., (Copyright © 2020 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Recognition of motor signs in the prodromal stage could help identify those at risk of developing Parkinson's disease (PD)., Objective: This study identified motor symptoms and signs in individuals suspected of having PD but who did not have a progressive reduction in the speed and amplitude of finger tapping or other physical signs indicative of bradykinesia., Methods: 146 patients, who had symptoms or signs suggestive of PD, were serially evaluated by a movement disorder specialist, using the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) Part III and video recordings. If the patients 'converted' to PD during follow-up, they were categorized as cases and compared with those who did not meet PD criteria during follow-up (non-cases)., Results: The 82 cases were more likely to have action dystonia or postural/action/rest tremor of a limb (OR 2.8; 95% CI 1.1-7.1; p = 0.02), a reduced blink rate at rest (OR 2.3; 95% CI 1.2-4.6; p = 0.01), anxiety (OR 8.9; 95% CI 2.6-31.1; p < 0.001), depression (OR 7.0; 95% CI 2.9-17.2; p < 0.001), or a frozen shoulder (OR 3.1; 95% CI 1.6-6.2) than the 64 'non-cases'.A reduction of the fast blink rate was common in patients who met the criteria for PD (p < 0.001)., Conclusions: This study emphasizes that motor dysfunction is a component of the clinical prodrome seen in some patients with PD.

The term hemiballism-hemichorea refers to a movement disorder characterized by involuntary movements, often violent, described as uncontrollable jerking, flinging, flailing or kicking, involving proximal muscles of a limb and it is often associated with lesions in the subthalamic nucleus. In this report, we described three cases of hemiballism-hemichorea as the first manifestation of acute ischemic stroke with lesion in the frontoparietal region on brain MRI and no involvement of the subthalamic nucleus. One patient was treated with thrombolysis and recovered within one hour. The other patients recovered within 48 h from symptoms onset. The impairment of the recently described "hyperdirect way", in which the cortical signal reach directly the subthalamic nucleus, may underlie the symptoms. We support, with a clinical point of view, the role of the frontoparietal region in the genesis of the hemiballism-hemichorea. An acute onset of this symptom should lead to think to an acute stroke., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: OFF periods impair quality of life in Parkinson's disease but the nature and degree of this impact is largely unquantified. Optimal treatment relies on assessing the experience and impact of these periods on patients and their carepartners., Objectives: To understand the experience and impact of OFF periods on their lives., Methods: Informed by qualitative interviews we designed questionnaires and surveyed neurologists, people with Parkinson's disease and carepartners., Results: 50 general neurologists, 50 movement disorder neurologists, 442 patients (median disease duration 5 years) and 97 carepartners were included. The most common OFF symptoms reported by patients and carepartners were stiffness, slowness of movement and changes in gait. Non-motor symptoms were less common. A higher proportion of carepartners reported each symptom. A minority of neurologists recognized pain, sweating and anxiety as possible symptoms of OFF periods. The three OFF symptoms most frequently designated as having great impact by people with Parkinson's disease were changes in gait, slowness and stiffness. In contrast, cognitive impairment was most frequently rated as having great impact on carepartners. OFF periods were reported to impact many aspects of the lives of both patients and carepartners., Conclusions: In people with Parkinson's disease of under 10 years duration, motor symptoms of OFF periods predominate in impact, however cognitive impairment has great impact on carepartners. Education is needed for neurologists regarding the non-motor aspects of OFF. The importance of involving carepartners in the assessment regarding OFF periods is supported by the higher frequency of symptom reporting by carepartners, and the significant impact on their lives.

Background/objective: Easily accessible biomarkers are crucial for disease-modifying clinical trials in patients with Parkinson's disease (PD). We investigated integrated plasma and neuroimaging biomarkers correlating with motor and cognitive severity in PD patients., Methods: This cross-sectional study enrolled 170 participants (12 controls and 158 PD patients). Plasma α-synuclein and neurofilament light chain (NfL) level, and global and regional cortical thickness (CTh) on brain MRI were analyzed to predict advanced motor stage (Hoehn & Yahr stage ≥3), and PD dementia (PDD, MMSE score <26)., Results: Plasma α-synuclein and NfL levels were higher in PD patients than controls (both P < 0.0001 for α-synuclein and NfL). Plasma NfL levels were significantly elevated in patients with advanced motor stage (P = 0.008) or PDD; α-synuclein was elevated in the advanced motor stage group. Global CTh was thinner in patients with PDD than controls (2.33±0.19 mm vs 2.43±0.14 mm, P = 0.06). Among PD patients, higher α-synuclein was associated with thinner limbic CTh, whereas higher NfL was associated with thinner temporal CTh and insular CTh. The accuracy of predicting advanced motor stage using age and sex alone (area under the curve [AUC] 0.63) was significantly improved by the addition of plasma α-synuclein and NfL, and temporal and insula CTh (full model, AUC 0.77, P = 0.004). The accuracy of predicting PDD using age and sex alone (AUC 0.82) increased by incorporating plasma α-synuclein and NfL, and temporal and insula CTh as full model (AUC 0.87, P = 0.047)., Conclusions: Integrated plasma and neuroimaging biomarkers reflect both motor and cognitive aspects of PD severity.

Background: Spinocerebellar ataxia type 8 (SCA8) is a rare autosomal dominant neurodegenerative disease caused by CTA/CTG repeat expansion in the ATXN8/ATXN8OS gene., Methods: To analyze the frequency and clinical characteristics of SCA8 patients in mainland China, we combined polymerase chain reaction (PCR) and triplet repeat-primed PCR (TRP-PCR) to detect the CTA/CTG expansion. We studied a cohort of 362 ataxia patients in which the other known causative genes had been previously excluded, from among 1294 index patients. Positive samples were validated by southern blotting., Results: The CTA/CTG expansion was observed in six probands, accounting for approximately 0.46% (6/1294) in all patients, and 1.66% (6/362) in patients without definite molecular diagnosis. Clinically, aside from the typical SCA8 phenotype, some patients carrying the CTA/CTG expansion exhibited the cerebellar form of multisystem atrophy (MSA-C) and ataxia with paroxysmal kinesigenic dyskinesia (PKD)., Conclusion: For the first time, we described the PKD phenotype in association with CTA/CTG expansion, suggesting that CTA/CTG expansion might play a role in the pathogenesis of paroxysmal dyskinesia symptoms.

Objective: To study scapular winging or other forms of scapular dyskinesis (condition of alteration of the normal position and motion of the scapula) in myotonic dystrophy type 1 (DM1), which is generally considered to be a distal myopathy, we performed an observational cohort study., Methods: We performed a prospective cohort study on the clinical features and progression over time of 33 patients with DM1 and pronounced, mostly asymmetric scapular winging or other forms of scapular dyskinesis. We also explored if scapular dyskinesis in DM1 has the same genetic background as in facioscapulohumeral muscular dystrophy type 1 (FSHD1)., Results: The cohort included patients with congenital (n = 3), infantile (n = 6) and adult-onset DM1 (n = 24). Scapular girdle examination showed moderate shoulder girdle weakness (mean MRC 3) and atrophy of trapezius, infraspinatus, and rhomboid major, seemingly similar as in FSHD1. Shoulder abduction and forward flexion were limited (50-70°). In five patients, scapular dyskinesis was the initial disease symptom; in the others it appeared 1-24 years after disease onset. Follow-up data were available in 29 patients (mean 8 years) and showed mild to severe increase of scapular dyskinesis over time. In only three patients, DM1 coexisted with a FSHD mutation. In all other patients, FSHD was genetically excluded. DM2 was genetically excluded in nine patients. The clinical features of the patients with both DM1 and FSHD1 mutations were similar to those with DM1 only., Conclusion: Scapular dyskinesis can be considered to be part of DM1 in a small proportion of patients. In spite of the clinical overlap, FSHD can explain scapular dyskinesis only in a small minority. This study is expected to improve the recognition of shoulder girdle involvement in DM1, which will contribute to the management of these patients.

Background: Hemiballism is a rare hyperkinetic movement disorder characterized by involuntary, high-amplitude, unilateral flailing of upper or lower extremities or both. In the case of hemiballism refractory to pharmaceutical interventions, deep brain stimulation (DBS) is an effective primary neurosurgical treatment. DBS targets for hemiballism include the thalamus, subthalamic nucleus, and globus pallidus internus (GPi)., Case Description: We present a case of a patient who sustained a posterior cerebral artery ischemic stroke that eventually led to uncontrolled hemiballism, which was then successfully treated by unilateral GPi stimulation. We include a video depicting the patient preoperatively, intraoperatively with stimulation off, and intraoperatively with stimulation on. We also review published cases of hemiballism treated by GPi-DBS, which support the claim that GPi-DBS is an effective method for treating hemiballism., Conclusions: Evidence gathered from the literature indicates that GPi-DBS is an effective treatment for hemiballism, especially after neuroleptics have failed. Results from various case studies of GPi-DBS used to treat hemiballism reveal improved motor ability and decreased dyskinesia, although degree of improvement may vary. More studies are required to establish which DBS target requires the least amount of stimulation to treat hemiballism., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: Improvement of quality-of-life (QoL) has been termed as a primary objective in initiating therapy in both Parkinson's disease (PD) and multiple system atrophy Parkinsonian subtype (MSA-P). We aimed to compare the determinants of life quality in drug naïve PD and MSA-P patients., Methods: Eighty-six drug-naïve PD patients and thirty-five drug-naïve MSA-P patients were included to explore the determinants of QoL. Demographic information, motor deficits, and non-motor symptoms were included in the clinical assessment., Results: Both motor and non-motor functions were more severely impaired in the drug-naïve MSA-P patients, with higher PDQ-39 scores indicating poorer QoL. Physical discomfort and stigma were the main affected sub-domains in PD, while mobility and activity of daily life were the main affected ones in MSA-P. BECK depressive scores and UPDRS-III scores were independent variables of PDQ-39 in MSA-P patients. Age, depression, disease stages and non-motor scores were independent variables of PDQ-39 in PD patients., Interpretation: Drug-naïve MSA-P patients suffered from more severe motor and non-motor disability, as well as poorer QoL. Depression and non-motor symptoms were proved to be the most critical determinants for QoL in PD, while motor function was supposed to be the major determinant for MSA-P. When initiating therapy, physicians need to focus more on motor functions in drug-naïve MSA-P patients, but on depression in PD patients.

Introduction : Dyskinesia is a motor complication of Parkinson's disease (PD) characterized by clinical heterogeneity and complex pathogenesis and associated with long-term levodopa therapy. Recent and controversial views on the management of PD patients have suggested that overall dyskinesia rates, and particularly troublesome dyskinesia, may be declining due to more conservative levodopa dosing regimens, widespread availability and early introduction of deep brain stimulation, and use of continuous drug delivery strategies. Nevertheless, anti-dyskinetic agents continue to be evaluated in clinical trials and recent efforts have focused on non-dopaminergic drugs. Areas covered : In this review, the authors discuss the clinical phenomenology and current understanding of dyskinesia in PD with a focus on up-to-date therapeutic strategies to prevent and manage these drug-related involuntary movements. Expert opinion : The way dyskinesia in PD is currently managed should be changed and attention should be focused toward a more personalized medicine rather than a one-fits-all-approach. The correct identification of dyskinesia types and tailored treatments are crucial for a better management of these involuntary movements together with a holistic approach which considers additional influencing factors. The future for dyskinesia treatment is likely to be found in non-dopaminergic approaches, first set into motion by the introduction of amantadine.

Objectives: In National Collegiate Athletic Association Division I swimmers, we examined the differences in thoracic spine rotation in swimmers with and without scapular dyskinesis and the relationship between thoracic spine rotation and shoulder pain/dysfunction according to the Kerlan-Jobe Orthopaedic Clinic (KJOC) score., Design: Cross-sectional., Setting: Laboratory-based., Participants: 34 NCAA Division I swimmers (13 males, 21 females)., Main Outcome Measures: Self-reported upper extremity function and pain assessed with the KJOC questionnaire, thoracic spine range of motion, presence of scapular dyskinesis., Results: Dyskinesis was present in 15 of 34 (44%) subjects. Thoracic rotation averaged 136.7° and KJOC averaged 87.7 with no differences between swimmers with or without dyskinesis. We observed no correlation between KJOC-identified shoulder pain/dysfunction and thoracic rotation., Conclusions: In our cohort of NCAA Division 1 swimmers, no differences were found between swimmers with or without scapular dyskinesis and extent of thoracic rotation. We found no correlation between thoracic rotation and the amount of self-reported pain and dysfunction experienced in the upper extremity. The presence of scapular dyskinesis in nearly half of our subjects indicates that swimmers need to be assessed for this abnormality. If observed, rehabilitation should address the dyskinesis and improve thoracic rotation in an attempt to alleviate further upper extremity pain and dysfunction., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: The possible factors related to functional impairment and limitations in patients with temporomandibular disorders (TMDs) still need to be clarified because recovery of orofacial functions is a goal of their treatment., Objective: To investigate whether chronic TMD patients had any changes in tongue strength, besides the difficulty in chewing and orofacial functional impairment, compared to a control group. Moreover, to examine whether tongue strength, chewing difficulties, and orofacial functions were associated., Methods: Twenty-three patients with chronic TMD according to the Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD) and volunteers without TMD (control group) were compared. Strength measures were obtained using the Iowa Oral Performance Instrument (IOPI) during tongue protrusion (TPS) and swallowing of saliva (SS) tasks. A scale was used to investigate self-reported chewing difficulties, and the orofacial muscles and functions were evaluated using the orofacial myofunctional evaluation with scores protocol (OMES)., Results: Compared to the control group, TMD patients showed reduced TPS and SS, higher difficulty in chewing and worse myofunctional orofacial conditions. Tongue strength was correlated with mastication and swallowing behaviors, as well as with general myofunctional status. Chewing difficulty increased with decreasing tongue strength and with worsening of orofacial muscles and functions., Conclusion: Patients with chronic TMD showed reduced tongue strength and worse masticatory and swallowing functions, and these aspects were interrelated., Clinical Relevance: The present results contribute additional evidence regarding the impairment of orofacial muscles other than jaw elevator muscles in patients with chronic TMD., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Movement disorders including hemichorea-hemiballism as the initial presentation of an acute ischemic stroke are uncommon. Structures outside of the deep subcortical areas such as the subthalamic nucleus or basal ganglia are rarely involved., Case Report: We report a case of a 72-year-old man with vascular risk factors who presented with acute onset right-sided hemichorea-hemiballism. Metabolic-, infectious-, and toxic-related conditions were ruled out, his EEG was without epileptiform changes. An MRI confirmed an acute ischemic stroke in the parieto-occipital region without any subcortical structures involved. Atrial Fibrillation was later discovered during his hospitalization and was treated appropriately., Conclusions: Although rare, strokes outside of the subthalamic nucleus can result in hemichorea-hemiballism., (Published by Elsevier Inc.)

Objective: We assessed differential item functioning (DIF) in the Unified Dyskinesia Rating Scale (UDysRS) to evaluate bias risk from the duration of Parkinson's Disease (PD) and duration of dyskinesia., Background: Assessing DIF is a core validation step for rating scales. If DIF is present for an item, interpretation must consider influences from the tested covariates. DIF can be uniform or non-uniform, depending on the consistency of influence from the given covariate across all levels of dyskinesia., Methods: Using a large UDysRS database (N = 2313), uniform and non-uniform DIF related to the duration of PD and/duration of dyskinesia were tested. Unidimensionality of UDysRS was first confirmed using confirmatory factor analysis. DIF analysis was conducted using two independent latent models. DIF in an item was confirmed if both methods independently identified DIF at a significance level using Bonferroni correction. McFadden pseudo R^2 measured clinical relevancy of DIF magnitude (negligible, moderate, and large) for items identified with DIF, and items with DIF were considered clinically relevant if they exceeded a negligible designation., Results: Most items did not show uniform or non-uniform DIF based on PD and dyskinesia duration in isolation or in combination. For all items where DIF was identified, the magnitude statistic was in the negligible range (McFadden pseudo R^2 < 0.035) and the combined impact of multiple identified DIF items on UDysRS likewise did not exceed the negligible designation., Conclusion: The absence of clinically relevant DIF suggests that the UDysRS can be applied across all patients regardless of their PD- or dyskinesia-duration., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Abnormal involuntary movements (AIM) may occur throughout the course of psychosis. While AIM are thought to indicate striatal abnormalities, the functional and structural correlates of increased AIM remain elusive. Here, we examined the prevalence of AIM in patients with clinical high risk for psychosis (CHR), first episode psychosis (FEP) and clinical controls (CC). Furthermore, we tested the association of AIM with regional cerebral blood flow (rCBF), grey matter volume (GMV), and premorbid IQ., Methods: We conducted a video-based analysis of AIM in patients with CHR (n = 45), FEP (n = 10) and CC (n = 39), recruited in the Early Detection and Intervention Center, Bern. Premorbid intelligence was evaluated using the Peabody Picture Vocabulary test. Additionally, arterial spin labeling MRIs and structural MRIs were acquired in a subgroup of the sample to investigate the association of AIM with rCBF and GMV., Results: Higher total AIM scores were detected in CHR (p = 0.02) and FEP (p = 0.04) as compared to CC. When separated for different muscle groups, lips and perioral movements were significantly increased in CHR patients as compared to CC (p = 0.009). AIM scores correlated positively with rCBF in the premotor cortex, Brodmann area 6 (p < 0.05, FWE corrected). Negative correlations were found between AIM and GMV of the corresponding caudal middle frontal gyrus (p = 0.04, FWE corrected) and premorbid intelligence (p = 0.02)., Conclusions: AIM were more frequent in the psychosis spectrum than in clinical controls. Neuroimaging findings indicate an involvement of cortical motor areas in abnormal motor behavior, instead of pure basal ganglia pathology., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: The early stage of Parkinson's disease (PD) (Hoehn-Yahr (HY) I-II stages) is characterized by a negative pull test, which clinically excludes postural instability. Previous studies with dynamic posturography detected balance disturbances even at the onset of the disease but the age dependency or prediction of dyskinesia with dynamic posturography are not known., Objective/hypothesis: We hypothesized that the postural instability evoked by dynamic posturography was part of the early stage of PD. Furthermore, we studied how we can provoke dyskinesia., Methods: Postural instability with static and dynamic posturography (passing balls with different weights around the body) was studied in 45 patients with PD in their HY I, II stages. They were compared with 35 age-matched healthy controls. Eighteen patients with dyskinesia were involved in the study. Fourteen patients were followed for two years., Results: The pathway and velocity of the movement assessed by static and the dynamic posturography were significantly higher in the group >65 years than that of age-matched healthy controls, while the group ≤65 years showed a significant increment only in the antero-posterior sway during dynamic posturography. The imbalance of patients with dyskinesia was significantly (p < 0.05) provoked by dynamic posturography compared to patients with PD without dyskinesia. The results were independent of age., Conclusion: Postural instability is part of the early symptoms of PD. Non-dopaminergic pathways may be involved in the early stage of PD., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Recent studies have shown how emotional and cognitive factors might combine together to determine the onset and maintenance of functional motor symptoms (FMS). Nevertheless, no studies have assessed whether brain circuits involved in regulation and processing of emotions and attention might be influenced by neuromodulation. The purpose of this study was to evaluate the effect of a single anodic tDCS session over the right posterior parietal cortex in subjects with FMS and in healthy individuals., Materials and Methods: Nine patients and seven healthy subjects underwent two sessions of tDCS (real and sham), in a randomized order. At the end of each session, all participants underwent the heart beat detection task (interoceptive sensitivity) and the Posner paradigm (spatial attention)., Results: After sham stimulation, patients with FMS showed significantly lower interoceptive sensitivity and greater cueing effect for reaction times at the Posner paradigm than healthy controls. There was a significant improvement between the levels of interoceptive sensitivity after real and sham stimulation in the whole group of participants and in the group of patients with FMS., Conclusions: Our study provides first indications for a neuromodulatory effect of a single anodic tDCS session over the right posterior parietal cortex on interoceptive sensitivity in subjects with FMS.

Using bacterial artificial chromosome-double transgenic mice expressing tdTomato in D1 receptor-medium spiny neurons (MSNs) and enhanced green fluorescent protein in D2 receptor-MSNs, we have studied changes in spine density and perisomatic GABAergic boutons density in MSNs of both the D1R and D2R pathways, in an experimental model of parkinsonism (mouse injected with 6-hydroxydopamine in the medial forebrain bundle), both in the parkinsonian and dyskinetic condition induced by L-DOPA treatment. To assess changes in perisomatic GABAergic connectivity onto MSNs, we measured the number of contacts originated from parvalbumin (PV)-containing striatal "fast-spiking" interneurons (FSIs), the major component of a feed-forward inhibition mechanism that regulates spike timing in MSNs, in both cell types as well as the number of vesicular GABA transporter (VGAT) contacts. Furthermore, we determined changes in PV-immunoreactive cell density by PV immunolabeling combined with Wisteria floribunda agglutinin (WFA) labeling to detect FSI in a PV-independent manner. We also explored the differential expression of striatal activity-regulated cytoskeleton-associated protein (Arc) and c-Fos in both types of MSNs as a measure of neuronal activation. Our results confirm previous findings of major structural changes in dendritic spine density after nigrostriatal denervation, which are further modified in the dyskinetic condition. Moreover, the finding of differential modifications in perisomatic GABAergic connectivity and neuronal activation in MSNs suggests an attempt by the system to regain homeostasis after denervation and an imbalance between excitation and inhibition leading to the development of dyskinesia after exposure to L-DOPA.

Background: Spasticity can be associated with several hyperkinetic involuntary movements generally referred to as "spasms" despite different phenomenology and clinical characteristics., Objective: To better characterize the phenomenology and clinical characteristics of spasticity-associated involuntary movements., Methods: We performed a cross-sectional study of a consecutive patient sample from the spasticity clinic. Each patient was interviewed by a movement-disorder neurologist who conducted a standardized movement-disorder survey and a focused exam. Patients with involuntary movements were video-recorded and videos were independently rated by a separate blinded movement-disorder neurologist., Results: Sixty-one patients were included (54% female, mean age 49.7 ± 13.9 years). Of the entire cohort, 11.5% had spinal, 44.3% had cerebral, and 44.3% had mixed-origin spasticity. Fifty-eight patients (95%) reported one or more involuntary movements: 75% tonic spasms (63% extensor, 58% isometric, 41% flexor/adductor), 52% spontaneous clonus, 34% myoclonus, 33% focal dystonia, and 28% action tremor. One third of the involuntary movements were painful. Only 53% of patients reported that their involuntary movements were much or very-much improved with their current anti-spasticity management. Patients treated with intrathecal baclofen therapy were more likely to report much or very-much improvement compared to patients receiving oral and/or botulinum therapy (P = 0.0061 and 0.0069 respectively)., Conclusion: Most spastic patients experience spasticity-associated involuntary movements of variable phenomenology and impact. However, only half of these patients experience significant improvement with the current management strategies. More research is needed to explore better treatment options for spasticity-associated involuntary movements with focus on phenomenology-specific approaches., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Study Objectives: ADCY5 mutations cause early-onset hyperkinetic movement disorders comprising diurnal and nocturnal paroxysmal dyskinesia, and patient-reported sleep fragmentation. We aimed to characterize all movements occurring during sleep and in the transition from sleep to awakening, to ascertain if there is a primary sleep disorder, or if the sleep disturbance is rather a consequence of the dyskinesia., Methods: Using video polysomnography, we evaluated the nocturnal motor events and abnormal movements in 7 patients with ADCY5 -related dyskinesia and compared their sleep measures with those of 14 age- and sex-matched healthy controls., Results: We observed an increased occurrence of abnormal movements during wake periods compared to sleep in patients with ADCY5 -related dyskinesia. While asleep, abnormal movements occurred more frequently during stage N2 and REM sleep, in contrast with stage N3 sleep. Abnormal movements were also more frequent during morning awakenings compared to wake periods before falling asleep. The pattern of the nocturnal abnormal movements mirrored those observed during waking hours. Compared to controls, patients with ADCY5 -related dyskinesia had lower sleep efficiencies due to prolonged awakenings secondary to the abnormal movements, but no other differences in sleep measures. Notably, sleep onset latency was short and devoid of violent abnormal movements., Conclusions: In this series of patients with ADCY5 -related dyskinesia, nocturnal paroxysmal dyskinesia were not associated with drowsiness or delayed sleep onset, but emerged during nighttime awakenings with subsequent delayed sleep, whereas sleep architecture was normal., (Copyright © 2019 American Academy of Sleep Medicine. All rights reserved.)

Rationale: Hemichorea-hemiballism, a rare manifestation of non-ketotic hyperglycemia, characterized by involuntary arrhythmic motions involving one side of the body, results from focal lesions in the contralateral caudate nucleus and putamen. Hyperkinetic disorders can be complications of uncontrolled diabetes mellitus and should not be ignored., Patient Concerns: We present the case of a 39-year-old woman who presented to the emergency department with a 3-day history of left-sided hemichorea-hemiballism. She had type 2 diabetes mellitus with poor control and maintenance of regular hemodialysis., Diagnoses: The patient was diagnosed as hyperglycemia, normal ketone body and hemichorea-hemiballism based on laboratory examination, computed tomography (CT) scan, and brain magnetic resonance image (MRI)., Interventions: Intensive glycemic control via insulin injection was prescribed for correction of hyperglycemia., Outcomes: The unilateral involuntary movements subsided progressively over four weeks. The patient's hemichorea had completely resolved at the three-month follow-up., Lessons: This unusual clinical presentation is often accompanied by severe hyperglycemia. Appropriate blood glycemic control is important. If physicians recognize and provide early treatment for this disease, it is usually treatable and has a good prognosis.

Background: Parkinson's disease (PD) is a progressive neurodegenerative disorder with significant disability. Subjects with advanced PD often suffer from motor complications that may interfere significantly with their daily activities. Levodopa (LD) in combination with a dopa decarboxylase inhibitor such as carbidopa (CD) is considered the gold standard in the treatment of PD. However, long-term treatment with LD often leads to the development of motor complications. Motor complications include motor fluctuations and dyskinesia. Motor fluctuations are states where the subject cycles between periods of "on" state where subjects are in improved mobility and "off" state where subjects are in impaired mobility. Dyskinesia are the involuntary and irregular twisting and/or turning movements., Methods: A Markov transitional probability model is proposed to estimate the likelihood of staying in one state versus transitioning from one state to another., Results: An application of the model to an example from a clinical trial investigating the effect of an extended-release carbidopa-levodopa (CD-LD) product versus an immediate-release CD-LD product is illustrated., Conclusion: A Markov transitional probability model can be used to model the likelihood of staying in one state versus transitional from one state to another. The model can also be used as a basis for multiple imputation of missing data.

Study Design: Crossover repeated-measure design., Introduction: Scapular dyskinesis rehabilitation programs that focus on inhibiting upper trapezius (UT) and activating the lower trapezius (LT) may assist in restoring scapular movements. We hypothesized that taping may be able to normalize scapular movements and associated muscular recruitment., Purpose of the Study: The purpose of this study was to investigate the immediate effects of kinesio taping over trapezius on scapular kinematics and muscular activation in different dyskinesis patterns. We expected that taping can improve scapular kinematics and muscular activation in subjects with dyskinesis., Methods: Fifty-four participants with inferior angle prominence (pattern I), medial border prominence (pattern II), and mixed pattern (pattern I + II) were recruited. Kinesio taping was applied over 3 parts of trapezius muscles, including UT, middle trapezius (MT), and LT. The scapular kinematics and electromyographic data of trapezius and serratus anterior were collected during scapular plane elevation without taping and after each taping application., Results: UT taping decreased UT activity (5%-7%; P = .001-.003) in 72% of participants with pattern II and pattern I + II dyskinesis, with increased posterior tipping (2.2°-2.5°; P = .003) in pattern II dyskinesis. MT taping increased UT activity (3%; P = .003) in 48% of participants with pattern II dyskinesis., Discussion: The taping over the trapezius muscle may help to restore coordinated scapular muscle balance and increased upward rotation of the scapula, especially in pattern II dyskinesis. Although no electromyography or kinematic difference was found with LT taping in each dyskinesis pattern, methods of applying LT taping need to be further investigated., Conclusion: Reduced UT muscle activity and scapular posterior tipping are appropriate when applying taping over UT muscle in patterns II and I + II dyskinesis. Caution should be taken when applying taping over MT and LT muscles in terms of increased UT activity, especially in pattern II dyskinesis., (Copyright © 2017 Hanley & Belfus. Published by Elsevier Inc. All rights reserved.)

Objectives: This study aimed to explore clinical correlates of repetitive speech disorders in patients with Parkinson's disease (PD)., Methods: This study investigated speech function (Assessment of Motor Speech for Dysarthria and Stuttering Severity Instrument-3), motor function (Unified Parkinson's Disease Rating Scale III [UPDRS-III] and UPDRS-IV), cognitive function (Mini-Mental State Examination [MMSE], Montreal Cognitive Assessment [MoCA], Stroop color-word test, verbal fluency, digit span tests, and line orientation), and activities of daily living of 113 PD patients. Comparison between groups (independent t-tests, Mann-Whitney U tests, or χ 2 test) and linear regression analyses were performed to determine clinical correlates of repetitive speech disorders., Results: Totally, 65 patients (57.5%) had repetitive speech disorders. Patients with repetitive speech disorders had significantly worse UPDRS-III (P = .049), MoCA (P = .030), and speech function and higher levodopa equivalent daily dose (LEDD; P = .031) than those without repetitive speech disorders. Males were significantly predominant in patients with repetitive speech disorders (64.6%) compared to those without repetitive speech disorders (18.7%; P < .001). The univariate and subsequent multiple linear regression analyses revealed that the severity of repetitive speech disorders significantly correlated with gender (P < .001), MoCA (P = .006), and speech variables (abnormal rate, P = .007; imprecise consonants, P = .043), independent from disease duration, UPDRS III, and LEDD., Conclusions: PD patients with repetitive speech disorders had worse motor, cognitive, and speech functions than those without repetitive speech disorders. The most influential factor for repetitive speech disorders might be male gender., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Device-assisted therapies (DAT) benefit people with Parkinsons Disease (PwP) but many referrals for DAT are unsuitable or too late, and a screening tool to aid in identifying candidates would be helpful. This study aimed to produce such a screening tool by building a classifier that models specialist identification of suitable DAT candidates. To our knowledge, this is the first objective decision tool for managing DAT referral. Subjects were randomly assigned to either a construction set (n = 112, to train, develop, cross validate, and then evaluate the classifier's performance) or to a test set (n = 60 to test the fully specified classifier), resulting in a sensitivity and specificity of 89% and 86.6%, respectively. The classifier's performance was then assessed in PwP who underwent deep brain stimulation (n = 31), were managed in a non-specialist clinic (n = 81) or in PwP in the first five years from diagnosis (n = 22). The classifier identified 87%, 92%, and 100% of the candidates referred for DAT in each of the above clinical settings, respectively. Furthermore, the classifier score changed appropriately when therapeutic intervention resolved troublesome fluctuations or dyskinesia that would otherwise have required DAT. This study suggests that information from objective measurement could improve timely referral for DAT.

The term "cerebral palsy mimic" is used to describe a number of neurogenetic disorders that may present with motor symptoms in early childhood, resulting in a misdiagnosis of cerebral palsy. Cerebral palsy describes a heterogeneous group of neurodevelopmental disorders characterized by onset in infancy or early childhood of motor symptoms (including hypotonia, spasticity, dystonia, and chorea), often accompanied by developmental delay. The primary etiology of a cerebral palsy syndrome should always be identified if possible. This is particularly important in the case of genetic or metabolic disorders that have specific disease-modifying treatment. In this article, we discuss clinical features that should alert the clinician to the possibility of a cerebral palsy mimic, provide a practical framework for selecting and interpreting neuroimaging, biochemical, and genetic investigations, and highlight selected conditions that may present with predominant spasticity, dystonia/chorea, and ataxia. Making a precise diagnosis of a genetic disorder has important implications for treatment, and for advising the family regarding prognosis and genetic counseling. © 2019 International Parkinson and Movement Disorder Society., (© 2019 International Parkinson and Movement Disorder Society.)

Objective: PANDAS are known as the spectrum of autoimmune pathologies related to a previous or current infection by group A beta-hemolytic streptococcus (SBEGA), dealing with several neuropsychiatric manifestations that mainly affect pediatric age. The main features consist of behavioral disease or movement disease characterized by acute-onset, presenting especially through infant period or adolescence. Specific manifestations, occurring during the progression of the disease, are the presence of otorhinolaryngologic symptoms (ENT) and orofacial movement disorders associated with temporomandibular joint pain., Patients and Methods: We enrolled 130 children (5-15 years) with a clinical diagnosis of PANDAS between 2012 and 2018. Participants were assessed using ENT specific parameters, PSG to examine respiratory disorders and conventional audiological evaluation. Descriptive and comparative statistical analyses were performed with a control group of 51 healthy patients., Results: The prevalence of ENT symptoms associated was significantly detected in 88 patients of 130 in Group A (relative frequency (%) 67.6; p=0.041) and in 51 patients of 130 in the control Group B (relative frequency (%) 39.2; p=0.063). In relation to prevalence of SDB, 54 subjects have presented nocturnal respiratory obstructive symptoms from mild to severe (relative frequency (%) 61.3; p=0.033) vs. 20 patients of Group B (relative frequency (%) 39.2; p=0.055). The obstructive severity average type was correlated to the consensual adenotonsillar development (size 3-4), (relative frequency (%) 45.4; p=0.047). The audiological deficits found were mostly of transmissive type with OME correlated and linked to the presence of occasional episodes of AOM. The four PANDAS patients who presented orobuccal dystonia (relative frequency (%) 4.54; p=0.091) achieved an improvement of the algic symptoms through the exercises of self-rehabilitation., Conclusions: Findings from our study show that respiratory diseases, characterizing a group of patients with pandas, are the direct consequences of the malformed or hypertrophic condition and suggesting in these conditions surgical therapy as an approaching tool.

Background: Walking impairment causes disability and reduced quality of life in patients with multiple sclerosis (MS)., Objective: Characterize the safety and efficacy of ADS-5102 (amantadine) extended release capsules, 274 mg administered once daily at bedtime in patients with MS with walking impairment., Methods: This randomized, double-blind, placebo-controlled, 4-week study was conducted at 14 trial sites in the United States. Study objectives included safety and tolerability of ADS-5102, and efficacy assessments (Timed 25-Foot Walk (T25FW), Timed Up and Go (TUG), 2-Minute Walk Test, and Multiple Sclerosis Walking Scale-12). Fatigue, depression, and cognition also were assessed., Results: A total of 60 patients were randomized (30 to ADS-5102 and 30 to placebo); 59 of whom were treated. The most frequent adverse events (AEs) were dry mouth, constipation, and insomnia. Five ADS-5102 patients and no placebo patients discontinued treatment due to AEs. One patient in the ADS-5102 group experienced a serious AE-suspected serotonin syndrome. A 16.6% placebo-adjusted improvement was seen in the T25FW test ( p < 0.05). A 10% placebo-adjusted improvement in TUG was also observed. No changes in fatigue, depression, or cognition were observed., Conclusion: ADS-5102 was generally well tolerated. These data demonstrate an effect of ADS-5102 on walking speed. Further studies are warranted to confirm these observations.

GNAO1 variants were recently discovered as causes of epileptic encephalopathies and heterogeneous syndromes presenting with movement disorders (MDs), whose phenomenology and clinical course are yet undefined. We herein focused on GNAO1-related MD, providing an analytical review of existing data to outline the main MD phenomenology and management, clinical evolution and genotype-phenotype correlations. Reviewing 41 previously published patients and assessing 5 novel cases, a comprehensive cohort of 46 patients was analyzed, reassuming knowledge about genotypes, phenotypes, disease course and treatment of this condition. GNAO1-related MD consisted of a severe early-onset hyperkinetic syndrome, with prominent chorea, dystonia and orofacial dyskinesia. Symptoms are poorly responsive to medical therapy and fluctuate, with critical and life-threatening exacerbations, such as status dystonicus. The presence of a choreiform MD appears to be predictive of a higher risk of movement disorder emergency. Surgical treatments are sometimes effective, although severe disabilities persist. Differently from the early infantile epileptic encephalopathy phenotype (associated with loss of function variants), no clear correlation between genotype and MD phenotype emerged, although some variants recurred more frequently, mainly affecting exons 6 and 7., (Copyright © 2018 Elsevier Ltd. All rights reserved.)