The International Classification of Functioning (ICF) to evaluate deep brain stimulation neuromodulation in childhood dystonia-hyperkinesia informs future clinical & research priorities in a multidisciplinary model of care.

The multidisciplinary team (MDT) approach illustrates how motor classification systems, assessments and outcome measures currently available have been applied to a national cohort of children and young people with dystonia and other hyperkinetic movement disorders (HMD) particularly with a focus on dyskinetic cerebral palsy (CP). The paper is divided in 3 sections. Firstly, we describe the service model adopted by the Complex Motor Disorders Service (CMDS) at Evelina London Children's Hospital and King's College Hospital (ELCH-KCH) for deep brain stimulation. We describe lessons learnt from available dystonia studies and discuss/propose ways to measure DBS and other dystonia-related intervention outcomes. We aim to report on current available functional outcome measures as well as some impairment-based assessments that can encourage and generate discussion among movement disorders specialists of different backgrounds regarding choice of the most important areas to be measured after DBS and other interventions for dystonia management. Finally, some recommendations for multi-centre collaboration in regards to functional clinical outcomes and research methodologies for dystonia-related interventions are proposed.

[Clinical and neurophysiological heterogeneity of attention deficit hyperactivity disorder]. / Klinicheskaya i neirofiziologicheskaya geterogennost' sindroma defitsita vnimaniya s giperaktivnost'yu.

AIM: To determine clinical/neurophysiological characteristics of different forms of attention deficit hyperactivity disorder (ADHD) and the efficacy of treatment with cerebrolysin. MATERIAL AND METHODS: Sixty children, aged 9 to 12 years, with ADHD were examined using clinical and electroencephalographic methods. Idiopathic and residual-organic forms were compared. RESULTS AND CONCLUSION: The study shows significantly higher levels of impulsivity and hyperactivity in children with residual-organic form of the disease. There were significant differences in the amplitude component of engaging in action (P3 Go) and the amplitude of the action suppression component (P3 NOGO) in patients with different forms of ADHD. The high clinical efficacy (improvement in 70.0% of patients with idiopathic form of ADHD and 86.7% of patients with residual-organic form of the disease) was found.

Burke-Fahn-Marsden dystonia severity, Gross Motor, Manual Ability, and Communication Function Classification scales in childhood hyperkinetic movement disorders including cerebral palsy: a 'Rosetta Stone' study.

AIM: Hyperkinetic movement disorders (HMDs) can be assessed using impairment-based scales or functional classifications. The Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFM-M) evaluates dystonia impairment, but may not reflect functional ability. The Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS) are widely used in the literature on cerebral palsy to classify functional ability, but not in childhood movement disorders. We explore the concordance of these three functional scales in a large sample of paediatric HMDs and the impact of dystonia severity on these scales. METHOD: Children with HMDs (n=161; median age 10y 3mo, range 2y 6mo-21y) were assessed using the BFM-M, GMFCS, MACS, and CFCS from 2007 to 2013. This cross-sectional study contrasts the information provided by these scales. RESULTS: All four scales were strongly associated (all Spearman's rank correlation coefficient rs >0.72, p<0.001), with worse dystonia severity implying worse function. Secondary dystonias had worse dystonia and less function than primary dystonias (p<0.001). A longer proportion of life lived with dystonia is associated with more severe dystonia (rs =0.42, p<0.001). INTERPRETATION: The BFM-M is strongly linked with the GMFCS, MACS, and CFCS, irrespective of aetiology. Each scale offers interrelated but complementary information and is applicable to all aetiologies. Movement disorders including cerebral palsy can be effectively evaluated using these scales.

Paroxysmal sympathetic hyperactivity after acquired brain injury: consensus on conceptual definition, nomenclature, and diagnostic criteria.

A syndrome of paroxysmal, episodic sympathetic hyperactivity after acquired brain injury has been recognized for almost 60 years. This project sought to simplify the confused nomenclature for the condition (>31 eponyms) and simplify the nine overlapping sets of diagnostic criteria. A consensus-developed questionnaire based on a systematic review of the literature was circulated to a widely representative, international expert group utilizing a Delphi approach. Diagnostic criteria were dropped if group consensus failed to agree on their relative importance, with a goal of reaching a Cronbach α of 0.8 (suitable for research purposes). The resulting criteria were combined into an assessment measure for clinical and research settings. The consensus group recommend that the term "paroxysmal sympathetic hyperactivity" replace previous terms to describe the "syndrome, recognised in a subgroup of survivors of severe acquired brain injury, of simultaneous, paroxysmal transient increases in sympathetic [elevated heart rate, blood pressure, respiratory rate, temperature, sweating] and motor [posturing] activity." An 11 point probabilistic diagnostic scale was developed with reference to published criteria, yielding an acceptable Cronbach α of 0.8. These 11 items were proceduralized and combined with a symptom severity index to produce a diagnostic tool for use with adults (the paroxysmal sympathetic hyperactivity assessment measure [PSH-AM]). Development of a pediatric version of the scale and further research into the validity of the PSH-AM is recommended. The consensus position builds on previous literature to establish diagnostic definitions and criteria, an important move to standardize research and management of this condition.

[Deep brain stimulation for hyperkinetic movement disorders]. / Tiefe Hirnstimulation bei hyperkinetischen Bewegungsstörungen.

The term hyperkinetic movement disorder encompasses dystonia, tremor, chorea, myoclon and tics. These symptoms are all caused by dysfunctional neural networks including the basal ganglia loop and can be accompanied by other neurological or psychiatric symptoms. Deep brain stimulation (DBS) is an important extension of therapeutic options for this group of patients in whom drug therapy is limited. Permanent electrodes are implanted in various subcortical brain areas in order to achieve an improvement in motor symptoms by high frequency stimulation. Already established indications include primary generalized or segmental dystonia and essential tremor but an increasingly better understanding of systemic pathophysiology has allowed DBS to be explored as a treatment for other disorders of the hyperkinetic spectrum. This article provides an overview of common hyperkinetic movement disorders from the viewpoint of recent advances in neurostimulation therapy.

Evaluation of hyperactivity, attention deficit, and impulsivity before and after adenoidectomy/adenotonsillectomy surgery.

OBJECTIVE: The objective of this study was to determine the severity of attention deficit, hyperactivity, and impulsivity symptoms in patients with obstructive airway problems scheduled to undergo adenoidectomy or adenotonsillectomy operation. The effects of the surgical treatment on these symptoms will also be investigated in a case-control design. METHODS: This prospective study included 63 patients (29 girls, 34 boys) who were operated on at the Department of Otorhinolaryngology, Dicle University Medical School, between January 2010 and May 2011 because of obstructive symptoms caused by adenoid or adenotonsillar hypertrophy. The age range of the patients was between 4 and 13 years. The control group consisted of 33 (17 girls, 16 boys) healthy children. RESULTS: Among the patients, 15 children underwent adenoidectomy because of adenoid hypertrophy; 13 patients had adenotonsillectomy because of adenotonsillar hypertrophy, and the remaining patients underwent adenotonsillectomy related with chronic or recurrent tonsillitis with adenoid hypertrophy. Based on the preoperative data, statistically significant difference was observed between the patient and control groups. The patients' attention deficit, hyperactivity, and impulsivity symptoms preoperatively and postoperatively have shown statistically significant differences. CONCLUSIONS: Attention deficit, hyperactivity, and impulsivity symptoms were common among the children who show signs of airway obstruction due to adenotonsillar hypertrophy. Adenoidectomy or adenotonsillectomy operations were both observed to be associated with improvement in these symptoms.

Drug-induced hyperkinetic movement disorders by nonneuroleptic agents.

Hyperkinetic movement disorders are characterized by excess movement, and include chorea, akathesia, asterixis, dystonia, tremor, myoclonus, and tics. A wide variety of pharmacologic agents may induce or exacerbate these disorders. Neuroleptic-induced tardive dyskinesia and levodopa-induced hyperkinesia are the most common causes of medication-induced chorea. However, several nonneuroleptic agents, including antidepressants and antiepileptic medications, may also worsen hyperkinetic movement disorders. Over-the-counter medications, such as analgesics and antiheartburn medications, have also occasionally been implicated as causing hyperkinetic movement disorders. Most information regarding drug-induced hyperkinetic disorders comes from case reports and anecdotes, rather than controlled clinical trials. Further research with larger controlled trials needs to verify many of these findings.

Does attention-deficit/hyperactivity disorder have a dimensional latent structure? A taxometric analysis.

An understanding of the latent structure of attention-deficit/hyperactivity disorder (ADHD) is essential for developing causal models of this disorder. Although some researchers have presumed that ADHD is dimensional and others have assumed that it is taxonic, there has been relatively little research directly examining the latent structure of ADHD. The authors conducted a set of taxometric analyses using data from the NICHD Study of Early Child Care and Youth Development (ns between 667 and 1,078). The results revealed a dimensional latent structure across a variety of different analyses and sets of indicators for inattention, hyperactivity/impulsivity, and ADHD. Furthermore, analyses of correlations with associated features indicated that dimensional models demonstrated stronger validity coefficients with these criterion measures than dichotomous models. These findings jibe with recent research on the genetic basis of ADHD and with contemporary models of ADHD.

Definition and classification of hyperkinetic movements in childhood.

Hyperkinetic movements are unwanted or excess movements that are frequently seen in children with neurologic disorders. They are an important clinical finding with significant implications for diagnosis and treatment. However, the lack of agreement on standard terminology and definitions interferes with clinical treatment and research. We describe definitions of dystonia, chorea, athetosis, myoclonus, tremor, tics, and stereotypies that arose from a consensus meeting in June 2008 of specialists from different clinical and basic science fields. Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. Chorea is an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments. Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture. Myoclonus is a sequence of repeated, often nonrhythmic, brief shock-like jerks due to sudden involuntary contraction or relaxation of one or more muscles. Tremor is a rhythmic back-and-forth or oscillating involuntary movement about a joint axis. Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement. Stereotypies are repetitive, simple movements that can be voluntarily suppressed. We provide recommended techniques for clinical examination and suggestions for differentiating between the different types of hyperkinetic movements, noting that there may be overlap between conditions. These definitions and the diagnostic recommendations are intended to be reliable and useful for clinical practice, communication between clinicians and researchers, and for the design of quantitative tests that will guide and assess the outcome of future clinical trials.

Treatment of hyperkinetic movement disorders.

Parkinson's disease, the most common hypokinetic movement disorder, has received much attention from the clinical and scientific community, but there has been a relative paucity of comprehensive reviews of hyperkinetic disorders, even though they are equally or even more disabling. Hyperkinetic movement disorders include tremors, dystonia, chorea, tics, myoclonus, stereotypies, restless legs syndrome, and various other disorders with abnormal involuntary movements. Substantial progress has been made in the understanding of the role of the basal ganglia in the pathophysiology of these hyperkinesia disorders and in motor control, muscle tone, posture, and cognitive processes. Although therapies that target pathogenesis are still lacking, effective management of hyperkinetic movement disorders demands that physicians are knowledgeable about current and novel pharmacological and surgical approaches. In addition to tetrabenazine, a monoamine-depleting drug, new formulations of botulinum toxin are being increasingly used in the treatment of these movement disorders. Finally, success with surgical approaches, particularly deep brain stimulation in patients with Parkinson's disease who have levodopa-induced dyskinesias, has been extended to the treatment of many hyperkinetic movement disorders.

A classification system for delirium subtyping with the use of a commercial mobility monitor.

The usefulness of motor subtypes of delirium is unclear due to inconsistency in subtyping methods and a lack of validation with objective measures of activity. The activity of 40 patients was measured over 24h with a commercial accelerometer-based activity monitor. Accelerometry data from patients with DSM-IV delirium that were readily divided into hyperactive, hypoactive and mixed motor subtypes, were used to create classification trees that were subsequently applied to the remaining cohort to define motoric subtypes. The classification trees used the periods of sitting/lying, standing, stepping and number of postural transitions as measured by the activity monitor as determining factors from which to classify the delirious cohort. The use of a classification system shows how delirium subtypes can be categorised in relation to overall activity and postural changes, which was one of the most discriminating measures examined. The classification system was also implemented to successfully define other patient motoric subtypes. Motor subtypes of delirium defined by observed ward behaviour differ in electronically measured activity levels.

A typology of behavior problems in preschool children.

The purpose of this study was to produce a typology of behavior problems in preschool children. Distinct subtypes were identified through the use of cluster analytic techniques on data from the Behavior Assessment System for Children (BASC)-Parent Rating Scales. Analyses were based on archival data collected on a sample of 268 children, aged 2 to 5 years, who were referred to determine their suitability for a day treatment program. Five distinct and reliable subtypes of preschool children's behavior problems emerged. Ratings of adaptive and social skills based on the BASC Adaptive Scales and measures from the Parenting Stress Index served as external variables to assess the distinctiveness of the derived subtypes. The findings provide evidence for the reliability and validity of the subtypes identified in this study that are consistent with the findings of previous subtyping investigations focusing on school-aged children.

Behavior and orofacial characteristics of children with attention-deficit hyperactivity disorder during a dental visit.

ADHD is a neuropsychological disorder, affecting attention, impulsiveness and activeness. The study included 36 children with ADHD, 47 without, and two silent observers. A dental form, SNAP-IV and ADHDT symptom checklists were used. Statistically significant differences were observed in hospitalization histories, oral habits, tongue characteristics, and facial biotype. Differences in orofacial characteristics and behavior between the groups were confirmed.

[Detection of fasciculations and other types of muscular hyperkinesias with ultrasound]. / Sonographische Darstellung von Faszikulationen und anderen Formen muskulärer Hyperkinesen.

Muscular hyperkinesias can be visualized by means of ultrasound (US): Ultrasonographic detection of fasciculations has been best evaluated. However, so-called ripplings, myoclonias and choreatic hyperkinesias as well as tremores can also be visualized by US. Muscular US has proven as a precise imaging technique for the detection of fasciculations. Fasciculations are brief, localized muscle twitches, usually lasting for 0.2 - 0.5 seconds. Some myoklonias may present similar in US, others present with a longer duration. In addition, muscle US allows a very easy and reliable detection of tremor frequency. This article reviews the present ultrasonographic facilities in the detection of muscular hyperkinesias and gives future perspectives.

6: Movement disorders II: the hyperkinetic disorders.

Involuntary movements or hyperkinesias are classified into syndromes of chorea, ballism, tremor, dystonia, myoclonus and tics. The hyperkinesias are caused by disturbances in the circuitry connecting the cerebral cortex, thalamus, basal ganglia and cerebellum. Drugs are a common cause of movement disorders. The aim of management is to characterise the movement disorder, identify and treat the cause or institute symptomatic treatment. The genetic basis of many movement disorders is increasingly recognised. Where there are potential implications for family members, accurate diagnosis and counselling are particularly important.

Hipercinesia un equivalente epiléptico: beneficio del tratamiento integral / Hiperkinesy a epileptic equivalent: integral of treatment benefit

De un grupo de doscientos setenta y ocho pacientes hiperactivos en el INAPSI, durante los años 1988 a 1992, se tomó una muestra de setenta y cuatro. El tratamiento farmacológico utilizado fue la Difenilhidantoína o la Carbamazepina, por considerar la hiperactividad, trastornos caracteriales (irritabilidad, impulsividad, baja tolerancia a las frustraciones), sueño intranquilo, bruxismo y sonambulismo, un "equivalente epiléptico". Se obtuvo un buen resultado. Desde 1965 el doctor A. Pimentel utiliza estos dos medicamentos y posteriormente el Clonazepán en pacientes adultos con hiperactividad, trastornos caracteriales, del sueño, algunos cuadros depresivos y otros, con resultados igualmente satisfactorios, pareciendo apoyar nuestras observaciones en niños. El tratamiento multidisciplinario consistió en ofrecer simultáneamente el control farmacológico, educación y orientación familiar, psicoterapia grupal, terapia educativa y otras terapias especiales según requerimientos del paciente

Genetic and environmental influences on the covariation between hyperactivity and conduct disturbance in juvenile twins.

Structural equation models were applied to the maternal ratings of 265 MZ and 163 DZ male-male, 347 MZ and 160 DZ female-female, and 262 male-female twin pairs, aged 8-16 years, who participated in the Virginia Twin Study of Adolescent Behavioral Development (VTSABD). Substantial additive genetic influences and contrast effects were found for hyperactivity, and additive genetic and shared environmental effects or positive comparison effects (particularly for the girls) for oppositional/ conduct disturbance. Bivariate model fitting showed that the covariation between hyperactivity and oppositional/conduct problems in both younger and older boys and girls is almost entirely attributable to genetic factors. However, whereas in the younger males and females the same set of genes explain all the variation in hyperactivity and conduct disturbance, in the older cohort at least some of the genetic effects are behavior- and gender-specific.