Development of the clinical assessment scale in autoimmune encephalitis.

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.

The relationship of dystonia and choreoathetosis with activity, participation and quality of life in children and youth with dyskinetic cerebral palsy.

AIM: To relate dystonia and choreoathetosis with activity, participation and quality of life (QOL) in children and youth with dyskinetic Cerebral Palsy (CP). METHODS: Fifty-four participants with dyskinetic CP (mean age 14y6m, SD 4y2m, range 6-22y) were included. The Dyskinesia Impairment Scale (DIS) was used to evaluate dystonia and choreoathetosis. Activity, participation and quality of life (QOL) were assessed with the Gross Motor Function Measure (GMFM), the Functional Mobility Scale (FMS), the Jebsen-Taylor Hand Function Test (JTT), the ABILHAND-Kids Questionnaire (ABIL-K), the Life Habits Kids (LIFE-H) and the Quality of Life Questionnaire for children with CP (CP-QOL). Spearman's rank correlation coefficient (rs) was used to assess the relationship between the movement disorders and activity, participation and QOL measures. RESULTS: Significant negative correlations were found between dystonia and the activity scales with Spearman's rank correlation coefficient (rs) varying between -0.65 (95% CI = -0.78 to -0.46) and -0.71 (95% CI = -0,82 to -0.55). Correlations were also found with the LIFE-H (rs = -0.43; 95%CI = -0.64 to -0.17) and the CP-QOL (rs = -0.32; 95%CI = -0.56 to -0.03). As far as choreoathetosis is concerned, no or only weak relationships were found with the activity, participation and quality of life scales. INTERPRETATION: This cross-sectional study is the first to examine the relationship of dystonia and choreoathetosis in dyskinetic CP with the level of activity, participation and QOL. The results revealed dystonia has a higher impact on activity, participation and quality of life than choreoathetosis. These findings seem to suggest it is necessary to first focus on dystonia reducing intervention strategies and secondly on choreoathetosis.

Hypertonia Assessment Tool.

The Hypertonia Assessment Tool is a 7-item instrument that discriminates spasticity, dystonia, and rigidity on 3 levels: item scores, subtype, and hypertonia diagnosis for each extremity. We quantified the inter- and intrarater reliability using Kappa statistics, Gwet's first-order agreement coefficient (both with 95% confidence interval), and percentage agreement for all levels. For validity, we compared the Hypertonia Assessment Tool subtype with the clinical diagnosis provided by the physicians. Two physiotherapists tested 45 children with neuromotor disorders. The interrater reliability (n = 45) of the Hypertonia Assessment Tool subtype was moderate to substantial whereas the intrarater reliability (n = 42) was almost perfect. The Hypertonia Assessment Tool showed good agreement in detecting spasticity. On the contrary, there was a higher presence of dystonia of 24% to 25% tested with the Hypertonia Assessment Tool compared to the clinical diagnosis. Even some individual items showed lower agreement between raters; the Hypertonia Assessment Tool subtypes and diagnosis were reliable. Validity of the Hypertonia Assessment Tool to test spasticity is confirmed, whereas, for dystonia and rigidity, further studies are needed.

Fundamentals of Embouchure in Brass Players: Towards a Definition and Clinical Assessment.

Brass players may experience problems producing an optimal sound (or range of sounds) in their instrument. Assessing and treating dysfunctional embouchure requires knowledge of functional embouchure, but peer-reviewed literature on dysfunctional and functional embouchure is scarce. OBJECTIVE: This study aimed to provide a narrative overview of embouchure based on information from different scientific and clinical fields. This should be regarded as a first step in constructing a reliable, valid, and practical multi-item method to assess embouchure for brass players. METHODS: Literature reviews were conducted concerning: 1) the definition of embouchure, 2) physics and acoustics of embouchure, 3) functioning of embouchure-related structures, and 4) instruments to assess embouchure. Also, embouchure experts (clinicians, scientists, and elite wind players) were consulted for information and discussion. RESULTS: A proposal for a new definition of embouchure, an overview of the relevant physics and acoustics, functions of embouchure-related body structures, and the main methods to measure embouchure in brass playing are presented. CONCLUSION: Peer-reviewed information about the fundamentals of dysfunctional embouchure is scarce and sometimes contradictory. A new definition for embouchure is proposed: embouchure is the process needed to adjust the amount, pressure, and direction of the air flow (generated by the breath support) as it travels through the mouth cavity and between the lips, by the position and/or movements of the tongue, teeth, jaws, cheeks, and lips, to produce a tone in a wind instrument. An integrative overview is presented which can serve as a transparent foundation for the present understanding of functional and dysfunctional embouchure and for developing an evidence-based multi-item assessment instrument.

Kinematic and kinetic assessment of upper limb movements in patients with writer's cramp.

BACKGROUND: The assessment and treatment of writer's cramp is complicated due to the variations in the forces and angles of involved joints. Additionally, in some cases compensatory movements for cramp relief further complicates assessment. Currently these variables are subjectively measured with clinical scales and visual assessments. This subjectivity makes it difficult to successfully administer interventions such as Botulinum toxin injection or orthotics resulting in poor efficacy and significant side effects. METHOD: A multi-sensor system was used to record finger and wrist forces along with deviation angles at the wrist, elbow and shoulder while 9 patients with writer's cramp performed a series of standardized tasks on surfaces inclined at different angles. Clinical, kinetic, and kinematic information regarding cramping was collected. RESULTS: First, four tasks appeared to best predict cramp occurrence. Second, unique biomechanical profiles emerged for patients regarding force, angles and cramp severity. Third, cluster analyses using these features showed a clear separation of patients into two severity classes. Finally, a relationship between severity and kinetic-kinematic information suggested that primary cramping versus compensatory movements could be potentially inferred. CONCLUSIONS: The results demonstrate that using a set of standardized tasks and objective measures, individual profiles for arm movements and applied forces associated with writer's cramp can be generated. The clinician can then accurately target the biomechanics specifically, whether it is with injection or other rehabilitative measures, fulfilling an important unmet need in the treatment of writer's cramp.

Perceived Cost and Intrinsic Motor Variability Modulate the Speed-Accuracy Trade-Off.

Fitts' Law describes the speed-accuracy trade-off of human movements, and it is an elegant strategy that compensates for random and uncontrollable noise in the motor system. The control strategy during targeted movements may also take into account the rewards or costs of any outcomes that may occur. The aim of this study was to test the hypothesis that movement time in Fitts' Law emerges not only from the accuracy constraints of the task, but also depends on the perceived cost of error for missing the targets. Subjects were asked to touch targets on an iPad® screen with different costs for missed targets. We manipulated the probability of error by comparing children with dystonia (who are characterized by increased intrinsic motor variability) to typically developing children. The results show a strong effect of the cost of error on the Fitts' Law relationship characterized by an increase in movement time as cost increased. In addition, we observed a greater sensitivity to increased cost for children with dystonia, and this behavior appears to minimize the average cost. The findings support a proposed mathematical model that explains how movement time in a Fitts-like task is related to perceived risk.

Assessment of patients with isolated or combined dystonia: an update on dystonia syndromes.

The clinical evaluation of a patient with dystonia is a stepwise process, beginning with classification of the phenomenology of the movement disorder(s), then formulation of the dystonia syndrome, which, in turn, leads to a targeted etiological differential diagnosis. In recent years, there have been significant advances in our understanding of the etiological basis of dystonia, aided especially by discoveries in imaging and genetics. In this review, we provide an update on the assessment of a patient with dystonia, including the phenomenology of dystonia and highlighting how to integrate clinical, imaging, blood, and neurophysiological investigations in order to formulate a dystonia syndrome. Evolving or emerging dystonia syndromes are reviewed, and potential etiologies of these as well as established dystonia syndromes listed to guide diagnostic testing. © 2013 Movement Disorder Society.

Virtual reality for assessment of patients suffering chronic pain: a case study.

The study of body representation and ownership has been a very active research area in recent years. Synchronous multisensory stimulation has been used for the induction of the illusion of ownership over virtual body parts and even full bodies, and it has provided experimental paradigms for the understanding of the brain processing of body representation. However, the illusion of ownership of a virtual body has rarely been used for patient evaluation and diagnosis. Here we propose a method that exploits ownership of a virtual body in combination with a simple brain computer interface (BCI) and basic physiological measures to complement neurological assessment. A male patient presenting a fixed posture dystonia featuring a permanently closed left fist participated in this case study. The patient saw a virtual body that substituted his own after donning a head-mounted display and thereby entering the virtual reality. The left virtual hand had the same posture as his corresponding real hand. After inducing virtual hand ownership by correlated visuo-tactile stimulation and dynamic reflections in a virtual mirror, the virtual hand would open either automatically or through a cognitive task assessed through a BCI that required him to focus attention on the virtual hand. The results reveal that body ownership induced changes on electromyography and BCI performance in the patient that were different from those in five healthy controls. Overall, the case study shows that the induction of virtual body ownership combined with simple electrophysiological measures could be useful for the diagnosis of patients with neurological conditions.

Anterocollis and camptocormia in parkinsonism: a current assessment.

Postural deformities in parkinsonian syndromes are well recognized, but poorly understood and largely refractory to available therapies. In recent times a number of hypotheses have been proposed to explain the underlying etiology of anterocollis and camptocormia, but currently there is no consensus. As these conditions are more precisely characterized we begin to uncover that this is a heterogeneous and evolving phenomenon. These conditions bring to light the inadequacies of our current tools to study biomechanics of posture, neuromuscular disorders, and dystonic muscular contractions. The development of objective, accurate tools to directly study and measure the severity of these postural disorders will allow for further understanding of the pathophysiology, the development of novel therapeutics, and adequate clinical trial design.

Development of the Hypertonia Assessment Tool (HAT): a discriminative tool for hypertonia in children.

AIM: The aim of this study was to develop a tool to identify paediatric hypertonia subtypes. METHOD: Items generated by experts were subscaled (spasticity, dystonia, rigidity). The tool was administered to 34 children (19 males, 15 females, mean age 8y 2mo, range 2y 5mo-18y 7mo) with hypertonia and cerebral palsy (CP) in Gross Motor Function Classification System (GMFCS) levels: I, n=7; II, n=5; III, n=7 level IV, n=7; and level V, n=8 level. Kuder-Richardson Formula 20 determined internal consistency. To assess reliability, two physicians administered the tool to 25 additional children with CP (15 males, 10 females; mean age 10y 8 mo; GMFCS levels I, n=4; II, n=3; III, n=7; IV, n=4; and V, n=7) on two occasions, 2 weeks apart. To evaluate validity, a third physician diagnosed the hypertonia by neurological examination. RESULTS: The internal consistency of the spasticity items was moderate (alpha = 0.58), and dystonia was high (a=0.79). Item reduction eliminated seven of the 14 original items. The agreement of the spasticity and rigidity subscales was adequate (prevalence-adjusted bias-adjusted kappa [PABAK] ranging from moderate [0.57] to excellent [1.0]) for validity, test-retest reliability, and interrater reliability. For dystonia agreement was lower, with PABAK ranging from fair (0.30) to good (0.65). Eighty-seven per cent had spasticity and 78% had dystonia. INTERPRETATION: The Hypertonia Assessment Tool has good reliability and validity for identifying spasticity and the absence of rigidity, and moderate findings for dystonia.

Risk assessment of isolated aripiprazole exposures and toxicities: a retrospective study.

STUDY OBJECTIVE: To characterize the clinical effects of acute isolated aripiprazole poisonings and to assess the toxic dose of this drug. METHODS: All isolated acute aripiprazole exposures reported to a poison control system from January 2002 through September 2006 were retrospectively reviewed. Patients with incomplete information or those lost to follow-up were excluded. RESULTS: A total of 286 cases were identified. Mean age was 18.9 years (SD 15.7), with a range of 6 months-70 years. Seventy-seven patients (27%) were or=18 years. Doses were known in 255 patients (89%). Symptoms occurred in 158 patients (55%): somnolence 89 (56%), tachycardia 32 (20%, heart rate 102-186), nausea/vomiting 29 (18%), dystonic reactions 21 (13%), tremor 9 (6%), agitation 3 (2%), dizziness 3 (2%), paresthesia 2 (1%), headache 2 (1%), dysphagia 1 (<1%), syncope 1 (<1%), minor facial swelling 1 (<1%), and hypotension 1 (<1%). None of the patients required intubation, and there were no deaths or EKG abnormalities. Median dose for symptomatic and asymptomatic groups were 25 and 15 mg, respectively, for or=18 (p = 0.25). In comparison of drug groups (Group 1, or=91 mg), symptoms were more likely to occur in Group 2 versus Group 1 (OR 2.29, 95% CI 0.79-6.61) in patients or=18 years. CONCLUSIONS: Acute aripiprazole poisonings most commonly result in sedation, sinus tachycardia, nausea/vomiting, or dystonic reactions. Symptoms are more likely with doses above 90 mg, although pediatric patients can be affected at a lower dose.

[Is there are needs treating the adolescents with syndrome of vegetative dysfunction?].

Article presents the pathogenesis aspects and clinical data of adolescents with syndrome of vegetative dysfunction. The authors observe the structure of different clinical forms, reflect on conditions and situation, when adolescents needs a stable complex of etiopathogenetic therapy to control autonomic nervous system dysfunction.

Electrophysiological assessment of the effect of intrathecal baclofen in dystonic children.

OBJECTIVE: To evaluate the effect of intrathecal baclofen in a group of dystonic children using electrophysiological procedures previously validated in spastic children. METHODS: Seven children (aged 2-16 years) with dystonia of various aetiologies (dyskinetic cerebral palsy, pantothenate kinase-associated neurodegeneration and Aicardi-Goutières syndrome) underwent transcranial magnetic stimulation, H-reflex and flexor reflex studies before and after intrathecal injection of baclofen. The Barry-Albright Dystonia Scale (BADS) was used for clinical evaluation of dystonia. RESULTS: Motor-evoked potentials, present in 2 of 5 patients before baclofen, were preserved after injection. Before baclofen, H reflex was present in 6 of 7 patients (mean H(max)/M(max:) 0.45+/-0.21). It was markedly reduced after the injection (mean H(max)/M(max:) 0.09+/-0.11) (P<0.001). Area of flexor reflex significantly decreased after baclofen (P=0.047), while threshold significantly increased (P=0.01). No significant clinical improvement of the BADS scores was observed (P=0.058). CONCLUSIONS: These electrophysiological procedures, previously demonstrated to quantify the action of intrathecal baclofen in spastic adults and children, also appear sensitive in dystonic children. The electrophysiological changes are consistent with primarily spinal sites of action of baclofen. They appear more sensitive than clinical evaluation.

Objective assessment of progression in Huntington's disease: a 3-year follow-up study.

Objective measures to assess progression of Huntington's disease (HD) are desirable. The authors have previously found that patients with HD with higher Unified Huntington's Disease Rating Scale (UHDRS) motor scores exhibited higher variability of isometric grip forces while grasping an object. Therefore, the authors assessed grip force variability during this task in 10 HD patients with a 3-year follow-up. Grip force variability increased in all patients at the follow-up. Thus, grip force variability during grasping might be an objective and quantitative measure to assess motor deficits associated with the progression of HD.