Clinical Presentation of Spasticity and Passive Range of Motion Deviations in Dyskinetic Cerebral Palsy in Relation to Dystonia, Choreoathetosis, and Functional Classification Systems.

Objectives: To map the presence, severity, and distribution of spasticity and passive range of motion (pROM) deviations in dyskinetic cerebral palsy (DCP), and to explore their relation with dystonia, choreoathetosis, and functional abilities.Methods: This cross-sectional study included 53 participants with DCP. Spasticity was assessed with the Modified Ashworth Scale, limited- and increased pROM (hypermobility) with a goniometer, dystonia and choreoathetosis with the Dyskinesia Impairment Scale, gross motor and manual abilities with corresponding functional classification systems.Results: Spasticity and limited pROM were correlated with dystonia of the upper limbs (0.41< rs<0.47, <0.001 < p < .002) and lower limbs (0.31< rs<0.41, 0.002 < p < .025), and both functional systems of gross motor (0.32< rs<0.51, <0.001 < p < .018) and fine manual abilities (0.34< rs<0.44, 0.001 < p < .014). Hypermobility is correlated only with choreoathetosis of the lower limbs (0.44, p = .001).Conclusions: Coexisting spasticity and pROM deviations in DCP are functionally limiting and should be addressed accordingly. Hypermobility may lead to an increased luxation risk.

Scapular dyskinesis in myotonic dystrophy type 1: clinical characteristics and genetic investigations.

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.

Defining the spectrum of spasticity-associated involuntary movements.

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.

[Non-epileptic paroxysmal disorders in pre-school children]. / Trastornos paroxísticos no epilépticos en pre-escolares.

Paroxysmal events are commonly encountered in toddlers. These events include a variety of conditions with different manifestations and pathophysiology. For that reason, the diagnosis of these events can be challenging. In some instances, studies such as EEG and polysomnogram may be useful to differentiate between epileptic and non-epileptic events. In the majority of cases, a complete clinical history is enough to make an appropriate diagnosis. In this article, we review some of the most common paroxysmal non-epileptic events affecting toddlers, such as: tics, dyskinesias, sleep related events, etc. We also discuss diagnostic strategies and treatment options.

Trastornos Paroxísticos No Epilépticos En Pre-Escolares / Non-epileptic paroxysmal disorders in pre-school children

Los trastornos paroxísticos no epilépticos son comunes en la población de niños pre-escolares. Estas condiciones incluyen una variedad de eventos cuyas manifestaciones y fisiopatología son muy distintas. Por esa razón, el diagnóstico puede representar un difícil reto. En algunas ocasiones, estudios como el EEG o la polisomnografía pueden ayudar a clarificar el diagnóstico y descartar un trastorno epiléptico. Sin embargo, la historia clínica y el examen físico suelen ser suficientes para llegar al diagnóstico correcto. En este artículo, presentamos información sobre los trastornos paroxísticos no epilépticos más comunes en la población de niños pre-escolares, incluyendo: tics, discinesias, eventos relacionados al sueño, etc. Además, discutimos estrategias para el diagnóstico y opciones de tratamiento.

Paroxysmal events are commonly encountered in toddlers. These events include a variety of conditions with different manifestations and pathophysiology. For that reason, the diagnosis of these events can be challenging. In some instances, studies such as EEG and polysomnogram may be useful to differentiate between epileptic and non-epileptic events. In the majority of cases, a complete clinical history is enough to make an appropriate diagnosis. In this article, we review some of the most common paroxysmal non-epileptic events affecting toddlers, such as: tics, dyskinesias, sleep related events, etc. We also discuss diagnostic strategies and treatment options.

Open-Access Electronic Diary for Motor Fluctuation and Dyskinesia Evaluation in Parkinson Disease: Comparison With Paper Diary.

OBJECTIVE: To determine the utility of an electronic diary for registering motor fluctuations and dyskinesia in Parkinson disease (PD). METHODS: Free, open-access touch screen software suitable for Android 4.4 or higher, with medication alarms, adjustable intervals, and medication dose settings was developed to evaluate ON-OFF periods and dyskinesia. Prospective evaluation included a first phase conducted to make adjustments concerning motor limitations when using the tablet, as well as for proper motor complication identification, and a second phase of 3 days of use at home with a prior diary training session comparing a modified paper version of Core Assessment Program for Surgical Interventional Therapies in PD and the electronic diary. RESULTS: All patients correctly identified ON-OFF periods and dyskinesia. Rater/patient matching ON-OFF fluctuations ranged between 94% and 100% for evaluations of different motor states. Dyskinesia matching percentage was 100% for patients with dyskinesia interfering with activities of daily living and 88% for those who reported no-interference. No significant differences between paper and electronic diaries were identified when reporting ON-OFF motor states or in the number of errors when filling the diaries. CONCLUSIONS: This electronic motor diary proved to be reliable for ON-OFF state and dyskinesia identification and classification. However, no advantage to paper diary has been observed in terms of number of erroneous entries. Based on these results, to improve home motor fluctuations, detection efforts should be directed toward the development of automatic wearable devices rather than digital versions of current available ON-OFF diaries.

Identification, classification and assessment of dyskinesia in children with cerebral palsy: A survey of clinicians.

AIM: The aims of this study were to investigate clinicians' knowledge, and barriers they perceive exist, relating to the identification and measurement of dyskinesia (dystonia/choreoathetosis) in children with cerebral palsy (CP) and to explore educational needs regarding improving identification and assessment of dyskinesia. METHODS: This was a cross-sectional online survey of clinicians working with children with CP. Data analysis was descriptive, with qualitative analysis of unstructured questions. RESULTS: In total, 163 completed surveys from Australian clinicians were analysed. Respondents were allied health (n = 140) followed by medical doctors (n = 18) working mainly in tertiary hospitals and not-for-profit organisations. Hypertonia subtypes and movement disorders seen in children with CP appear to be identified by clinicians, although limited knowledge about dyskinesia and access to training were reported as significant barriers to accurate identification. Despite knowledge of available measurement scales, only a small percentage were used clinically and reported to be only somewhat useful or not useful at all. Barriers identified for use of scales included limited training opportunities and knowledge of scales and lack of confidence in their use. CONCLUSION: A lack of confidence in identifying and measuring movement disorders in children with CP was reported by Australian clinicians. It was identified that a greater understanding of dyskinetic CP and the tools available to identify and measure it would be valuable in clinical practice. The results of this survey will inform the development of a 'Toolbox' to help identify, classify and measure dyskinetic CP and its impact on activity and participation using the framework of the International Classification of Functioning, Disability and Health.

Classification of Involuntary Movements in Dogs: Myoclonus and Myotonia.

Myoclonus is a sudden brief, involuntary muscle jerk. Of all the movement disorders, myoclonus is the most difficult to encapsulate into any simple framework. On the one hand, a classification system is required that is clinically useful to aid in guiding diagnosis and treatment. On the other hand, there is need for a system that organizes current knowledge regarding biological mechanisms to guide scientific research. These 2 needs are distinct, making it challenging to develop a robust classification system suitable for all purposes. We attempt to classify myoclonus as "epileptic" and "nonepileptic" based on its association with epileptic seizures. Myotonia in people may be divided into 2 clinically and molecularly defined forms: (1) nondystrophic myotonias and (2) myotonic dystrophies. The former are a group of skeletal muscle channelopathies characterized by delayed skeletal muscle relaxation. Many distinct clinical phenotypes are recognized in people, the majority relating to mutations in skeletal muscle voltage-gated chloride (CLCN1) and sodium channel (SCN4A) genes. In dogs, myotonia is associated with mutations in CLCN1. The myotonic dystrophies are considered a multisystem clinical syndrome in people encompassing 2 clinically and molecularly defined forms designated myotonic dystrophy types 1 and 2. No mutation has been linked to veterinary muscular dystrophies. We detail veterinary examples of myotonia and attempt classification according to guidelines used in humans. This more precise categorization of myoclonus and myotonia aims to promote the search for molecular markers contributing to the phenotypic spectrum of disease. Our work aimed to assist recognition for these 2 enigmatic conditions.

Functional outcomes in children and young people with dyskinetic cerebral palsy.

AIM: This cross-sectional study aimed to map the functional profile of individuals with dyskinetic cerebral palsy (CP), to determine interrelationships between the functional classification systems, and to investigate the relationship of functional abilities with dystonia and choreoathetosis severity. METHODS: Fifty-five children (<15y) and young people (15-22y) (30 males, 25 females; mean age 14y 6mo, standard deviation 4y 1mo) with dyskinetic CP were assessed using the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), Communication Function Classification System (CFCS), Eating and Drinking Ability Classification System (EDACS), and Viking Speech Scale (VSS), as well as the Dyskinesia Impairment Scale. RESULTS: Over 50 per cent of the participants exhibited the highest limitation levels in GMFCS, MACS, and VSS. Better functional abilities were seen in EDACS and CFCS. Moderate to excellent interrelationship was found among the classification scales. All scales had significant correlation (rs =0.65 - 0.81) with dystonia severity except for CFCS in the young people group. Finally, only MACS (rs =0.40) and EDACS (rs =0.55) in the young people group demonstrated significant correlation with choreoathetosis severity. INTERPRETATION: The need for inclusion of speech, eating, and drinking in the functional assessment of dyskinetic CP is highlighted. The study further supports the strategy of managing dystonia in particular at a younger age followed by choreoathetosis in a later stage.

Classification of involuntary movements in dogs: Tremors and twitches.

This review focuses on important new findings in the field of involuntary movements (IM) in dogs and illustrates the importance of developing a clear classification tool for diagnosing tremor and twitches. Developments over the last decade have changed our understanding of IM and highlight several caveats in the current tremor classification. Given the ambiguous association between tremor phenomenology and tremor aetiology, a more cautious definition of tremors based on clinical assessment is required. An algorithm for the characterisation of tremors is presented herein. The classification of tremors is based on the distinction between tremors that occur at rest and tremors that are action-related; tremors associated with action are divided into postural or kinetic. Controversial issues are outlined and thus reflect the open questions that are yet to be answered from an evidence base of peer-reviewed published literature. Peripheral nerve hyper-excitability (PNH; cramps and twitches) may manifest as fasciculations, myokymia, neuromyotonia, cramps, tetany and tetanus. It is anticipated that as we learn more about the aetiology and pathogenesis of IMs, future revisions to the classification will be needed. It is therefore the intent of this work to stimulate discussions and thus contribute to the development of IM research.

Generalized Models for the Classification of Abnormal Movements in Daily Life and its Applicability to Epilepsy Convulsion Recognition.

The identification and the modeling of epilepsy convulsions during everyday life using wearable devices would enhance patient anamnesis and monitoring. The psychology of the epilepsy patient penalizes the use of user-driven modeling, which means that the probability of identifying convulsions is driven through generalized models. Focusing on clonic convulsions, this pre-clinical study proposes a method for generating a type of model that can evaluate the generalization capabilities. A realistic experimentation with healthy participants is performed, each with a single 3D accelerometer placed on the most affected wrist. Unlike similar studies reported in the literature, this proposal makes use of [Formula: see text] cross-validation scheme, in order to evaluate the generalization capabilities of the models. Event-based error measurements are proposed instead of classification-error measurements, to evaluate the generalization capabilities of the model, and Fuzzy Systems are proposed as the generalization modeling technique. Using this method, the experimentation compares the most common solutions in the literature, such as Support Vector Machines, [Formula: see text]-Nearest Neighbors, Decision Trees and Fuzzy Systems. The event-based error measurement system records the results, penalizing those models that raise false alarms. The results showed the good generalization capabilities of Fuzzy Systems.

[Intercultural adaptation of the AIMS in German language: A scale for abnormal involuntary movements]. / Interkulturelle Adaption der AIMS in deutscher Sprache : Eine Skala für abnorme unwillkürliche Bewegungen.

BACKGROUND: Dyskinesias are abnormal involuntary movements and occur across many movement disorders. In Parkinson's disease dyskinesias can be troublesome and are a determinant of the quality of life throughout the course of the disease. Assessment and rating of dyskinesias is thus important for clinical assessment of patients, as well as for academic studies and clinical trials. The abnormal involuntary movement scale (AIMS) is an English language standardised, reliable and validated scale to evaluate dyskinesias. In this article we present a linguistically validated German version of AIMS. METHODS: The intercultural adaptation of the German translation was performed following an internationally accepted procedure. Firstly, two neurologists independently translated the original into German. Taking both versions into account, a consensus version was agreed on by both translators and was tested on 10 patients. This preliminary German version was then independently translated back into the original language by two different neurologists, and again, a consensus version was agreed on. All translators then compared this English version to the original. Subsequently, the German version was linguistically modified until it resulted in a final German version, which was agreed on by all translators, deemed linguistically acceptable, and the translation back into English was considered to be as unambiguous as possible. This final German version of AIMS was applied to 50 patients in two different hospitals for diagnostic purposes and tested for feasibility and comprehension. RESULTS: In this paper, we present an intercultural adaptation of a linguistically validated German version of AIMS.

Automatic assessment of medication states of patients with Parkinson's disease using wearable sensors.

Motor fluctuations are a major focus of clinical managements in patients with mid-stage and advance Parkinson's disease (PD). In this paper, we develop a new patient-specific algorithm that can classify those fluctuations during a variety of activities. We extract a set of temporal and spectral features from the ambulatory signals and then introduce a semi-supervised classification algorithm based on K-means and self-organizing tree map clustering methods. Two different types of cluster labeling are introduced: hard and fuzzy labeling. The developed algorithm is evaluated on a dataset from triaxial gyroscope sensors for 12 PD patients. The average result of using K-means and fuzzy labeling on the trunk and the more affected leg sensors' readings was 75.96%, 70.57%, and 86.93% for accuracy, sensitivity, and specificity, respectively. The accuracy for individual patients varied from 99.95% to 42.53%, which was correlated with dyskinesia severity and the improvement of the PD symptoms with medication.

Comprehensive classification test of scapular dyskinesis: A reliability study.

BACKGROUND: Assessment of scapular dyskinesis (SD) is of clinical interest, as SD is believed to be related to shoulder pathology. However, no clinical assessment with sufficient reliability to identify SD and provide treatment strategies is available. OBJECTIVES: The purpose of this study was to investigate the reliability of the comprehensive SD classification method. DESIGN: Cross-sectional reliability study. METHOD: Sixty subjects with unilateral shoulder pain were evaluated by two independent physiotherapists with a visual-based palpation method. SD was classified as single abnormal scapular pattern [inferior angle (pattern I), medial border (pattern II), superior border of scapula prominence or abnormal scapulohumeral rhythm (pattern III)], a mixture of the above abnormal scapular patterns, or normal pattern (pattern IV). The assessment of SD was evaluated as subjects performed bilateral arm raising/lowering movements with a weighted load in the scapular plane. Percentage of agreement and kappa coefficients were calculated to determine reliability. RESULTS: Agreement between the 2 independent physiotherapists was 83% (50/60, 6 subjects as pattern III and 44 subjects as pattern IV) in the raising phase and 68% (41/60, 5 subjects as pattern I, 12 subjects as pattern II, 12 subjects as pattern IV, 12 subjects as mixed patterns I and II) in the lowering phase. The kappa coefficients were 0.49-0.64. CONCLUSIONS: We concluded that the visual-based palpation classification method for SD had moderate to substantial inter-rater reliability. The appearance of different types of SD was more pronounced in the lowering phase than in the raising phase of arm movements.

[Meige's syndrome or segmental craniocervical dystonia: terminology, history and contemporary view].

The term "Meige's syndrome" or "Breughel's syndrome" is used by specialists for the description of blepharospasm with the involuntary movements in the lower part of the face and/or masseter muscle. These eponyms "Meige's syndrome" and "Breughel's syndrome" make some mess in the terminology. The term "segmental craniocervical dystonia" joins different blepharospam-plus phenotypes and reflects contemporary perceptions about its genetic and pathophysiological community. Botulinotherapy is in fact the only way of symptomathic treatment of craniocervical dystonias.

Can clinical observation differentiate individuals with and without scapular dyskinesis?

BACKGROUND: Altered scapular rotation and position have been named scapular dyskinesis. Visual dynamic assessment could be applied to classify this alteration based on the clinical observation of the winging of the inferior medial scapular border (Type I) or of the prominence of the entire medial border (Type II), or by the excessive superior translation of the scapula (Type III). OBJECTIVE: The aim of this study was to determine if there were differences in scapular rotations (Type I and II) and position (Type III) between a group of subjects with scapular dyskinesis, diagnosed by the clinical observation of an expert physical therapist, using a group of healthy individuals (Type IV). METHOD: Twenty-six asymptomatic subjects volunteered for this study. After a fatigue protocol for the periscapular muscles, the dynamic scapular dyskinesis tests were conducted to visually classify each scapula into one of the four categories (Type IV dyskinesis-free). The kinematic variables studied were the differences between the maximum rotational dysfunctions and the minimum value that represented both normal function and a small dysfunctional movement. RESULTS: Only scapular anterior tilt was significantly greater in the type I dyskinesis group (clinical observation of the posterior projection of the inferior angle of the scapula) when compared to the scapular dyskinesis-free group (p=0.037 scapular and p=0.001 sagittal plane). CONCLUSIONS: Clinical observation was considered appropriate only in the diagnoses of dyskinesis type I. Considering the lower prevalence and sample sizes for types II and III, further studies are necessary to validate the clinical observation as a tool to diagnose scapular dyskinesis.

Can clinical observation differentiate individuals with and without scapular dyskinesis? / O exame clínico de observação da discinese escapular é capaz de diferenciar portadores da disfunção dos normais?

Background: Altered scapular rotation and position have been named scapular dyskinesis. Visual dynamic assessment could be applied to classify this alteration based on the clinical observation of the winging of the inferior medial scapular border (Type I) or of the prominence of the entire medial border (Type II), or by the excessive superior translation of the scapula (Type III). Objective: The aim of this study was to determine if there were differences in scapular rotations (Type I and II) and position (Type III) between a group of subjects with scapular dyskinesis, diagnosed by the clinical observation of an expert physical therapist, using a group of healthy individuals (Type IV). Method: Twenty-six asymptomatic subjects volunteered for this study. After a fatigue protocol for the periscapular muscles, the dynamic scapular dyskinesis tests were conducted to visually classify each scapula into one of the four categories (Type IV dyskinesis-free). The kinematic variables studied were the differences between the maximum rotational dysfunctions and the minimum value that represented both normal function and a small dysfunctional movement. Results: Only scapular anterior tilt was significantly greater in the type I dyskinesis group (clinical observation of the posterior projection of the inferior angle of the scapula) when compared to the scapular dyskinesis-free group (p=0.037 scapular and p=0.001 sagittal plane). Conclusions: Clinical observation was considered appropriate only in the diagnoses of dyskinesis type I. Considering the lower prevalence and sample sizes for types II and III, further studies are necessary to validate the clinical observation as a tool to diagnose scapular dyskinesis. .

Contextualização: A movimentação ou posição alterada da escápula é definida como discinese escapular. O exame visual dinâmico pode ser utilizado para classificá-la de acordo com o julgamento clínico de projeção posterior excessiva da borda inferior medial (tipo I), da borda medial (tipo II) ou ainda translação excessiva no sentido cranial (tipo III). Objetivo: Determinar se há diferenças nas rotações escapulares (tipo I e II) e posição (tipo III) entre grupos de discinese e normais (tipo IV), os quais foram diagnosticados visualmente por um fisioterapeuta experiente. Método: Vinte e seis participantes assintomáticos foram voluntários neste estudo. Após um protocolo de fadiga periescapular, a avaliação dinâmica da discinese foi conduzida para classificar visualmente cada uma das escápulas em uma das quatro categorias (tipo IV - sem discinese). As variáveis cinemáticas estudadas foram a diferença entre o valor máximo indicativo da disfunção e o mínimo valor correspondente ao padrão normal esperado para o movimento ou o mínimo do próprio movimento disfuncional. Resultados: Apenas a inclinação anterior da escápula foi significantemente maior no grupo de discinese tipo I (observação visual de projeção posterior do ângulo inferior da escápula) quando comparada com o grupo sem discinese (p=0,037 plano escapular e p=0,001 plano sagital). Conclusões: A avaliação visual foi considerada apropriada apenas para o diagnóstico da discinese do tipo I. Considerando a baixa prevalência e o tamanho amostral dos tipos II e III, mais estudos são necessários para validar completamente a observação clínica como método adequado para o diagnóstico ...

[Overview of involuntary movements].

This issue is a kind introduction for the next three speakers who will give lectures about three considerably pathophysiology-known involuntary movements: tremor, myoclonus and dystonia.What's involuntary movement? This question relies on what is a voluntary movement. Some movements may include voluntary components and involuntary components. It usually excludes normal reflex movements, but they are sometimes included in the involuntary when much enhanced and cause some problems for the patients.Classification of involuntary movements. Clinical classification is based on clinical features, and it is, therefore, the same as how we explain some unknown movements to others. The rhythmicity, rapidness, pattern, stereotypy, part of the body involved, trigger factors are main features we use.How to record them. Video recording is the best method to record and show them to others. EEG, evoked potentials, polygraphs and some other physiological studies may help you to analyze their pathophysiology.