Treating Dystonia in a Soccer Player Through an Integrated Rehabilitative Approach: A Case Report.

CONTEXT: Sport-related dystonia is a rare form of activity-specific dystonia that can severely impair an athlete's ability to perform. Due to a lack of data on the condition, it is difficult to diagnose and often overlooked, and no gold standard treatment has yet been defined. CASE PRESENTATION: We present a rare and challenging case of sport-related dystonia that affected a 24-year-old male professional soccer player. The patient presented with severe rigidity and dystonia of the right lower-extremity, particularly the ankle and foot. The symptoms set on >1 year prior to the presentation to our outpatient clinic. He began to complain of stiffness and difficulty moving his lower limbs, especially his right leg, initially when playing soccer, but then also when walking normally. On presentation, he was unable to run and walked with difficulty, supporting his body weight only on the outside of his right foot. He also reported a motor trick and reverse motor trick involving the oral musculature in order to move his lower limb more freely. MANAGEMENT AND OUTCOMES: An integrated rehabilitation approach based on postural rehabilitation, neuromuscular rehabilitation, and dental intervention was used to successfully treat this condition. The approach included: (1) postural rehabilitation with the Mézières-Bertelè method to reduce muscular stiffness, (2) neuromuscular re-education with Tai Chi exercises and electromyography-guided biofeedback, and (3) dental intervention and swallowing rehabilitation to limit impaired oral habits (due to the relationship between his impaired lower limb movements and motor tricks of the oral musculature). After 7 months of integrated rehabilitation, the patient returned to professional soccer. CONCLUSIONS: This case report highlights the potential efficacy of an integrative rehabilitation approach for sports dystonia, particularly in cases where traditional treatments may not be effective. Such an approach could be considered a valuable option in the management of this rare, but debilitating, condition in athletes. Further research is needed to assess the effectiveness of this approach in larger populations.

Review of Tone Management for the Primary Care Provider.

Movement disorders in a pediatric population represent a spectrum of secondary functional deficits affecting ease of care, ambulation, and activities of daily living. Cerebral palsy represents the most common form of movement disorder seen in the pediatric population. Several medical and surgical options exist in the treatment of pediatric spasticity and dystonia, which can have profound effects on the functionality of these patients. Given the complex medical and surgical problems in these patients, children are well served by a multidisciplinary team of practitioners, including physical therapists, physical medicine and rehabilitation physicians, and surgeons.

Treatment of Dystonia: Medications, Neurotoxins, Neuromodulation, and Rehabilitation.

Dystonia is a complex disorder with numerous presentations occurring in isolation or in combination with other neurologic symptoms. Its treatment has been significantly improved with the advent of botulinum toxin and deep brain stimulation in recent years, though additional investigation is needed to further refine these interventions. Medications are of critical importance in forms of dopa-responsive dystonia but can be beneficial in other forms of dystonia as well. Many different rehabilitative paradigms have been studied with variable benefit. There is growing interest in noninvasive stimulation as a potential treatment, but with limited long-term benefit shown to date, and additional research is needed. This article reviews existing evidence for treatments from each of these categories. To date, there are many examples of incomplete response to available treatments, and improved therapies are needed.

A therapy-led, multidisciplinary programme for treatment-resistant functional fixed dystonia.

Functional neurological disorder (FND) describes various neurological symptoms that are not explained by an organic aetiology. The condition has a poor prognosis. Despite this, there is sparse research that informs clinical interventions for FND, particularly when treating functional fixed dystonia. Our article has outlined an intervention for a patient with a treatment-resistant functional fixed dystonia that was informed by a biopsychosocial model, which aimed to rehabilitate the patient's functional motor symptoms. This led to favourable outcomes including restoring full range of movement in the patient's right foot and improvements in routine outcome measurement scores. The patient also described the programme as life-changing and was able to re-engage in meaningful and purposeful activities.

Eye Gaze Gaming Intervention in Children with Dyskinetic Cerebral Palsy: A Pilot Study of Task Performance and Its Relation with Dystonia and Choreoathetosis.

OBJECTIVES: To investigate the operational competences screen navigation and dwell function underlying eye gaze performance, and the relation of dystonia and choreoathetosis with eye gaze performance in children with dyskinetic cerebral palsy (DCP). METHODS: During a 5-week intervention, ten participants with DCP played eye gaze video games daily for 30 minutes. Six games were used to assess task performance, fixation count, and eye movement accuracy during four measurements. Dystonia and choreoathetosis were evaluated using the Dyskinesia Impairment Scale. RESULTS: Eye gaze performance improved over time (p = .013). Moderate to strong within-subject correlations were found between eye movement accuracy and task performance, and between eye movement accuracy and fixation count. No significant correlations were found with the movement disorders. CONCLUSIONS: Eye gaze technology shows great potential to be a successful computer interface for children with severe DCP, thereby potentially improving their communication skills, participation levels, and quality of life.

EMG-based vibro-tactile biofeedback training: effective learning accelerator for children and adolescents with dystonia? A pilot crossover trial.

BACKGROUND: This study is aimed at better understanding the role of a wearable and silent ElectroMyoGraphy-based biofeedback on motor learning in children and adolescents with primary and secondary dystonia. METHODS: A crossover study with a wash-out period of at least 1 week was designed; the device provides the patient with a vibration proportional to the activation of an impaired target muscle. The protocol consisted of two 5-day blocks during which subjects were trained and tested on a figure-8 writing task: their performances (at different levels of difficulty) were evaluated in terms of both kinematics and muscular activations on day 1 and day 5, while the other 3 days were purely used as training sessions. The training was performed with and without using the biofeedback device: the week of use was randomized. Data were collected on 14 subjects with primary and secondary (acquired) dystonia (age: 6-19 years). RESULTS: Results comparing kinematic-based and EMG-based outcome measures pre- and post-training showed learning due to practice for both subjects with primary and secondary dystonia. On top of said learning, an improvement in terms of inter-joint coordination and muscular pattern functionality was recorded only for secondary dystonia subjects, when trained with the aid of the EMG-based biofeedback device. CONCLUSIONS: Our results support the hypothesis that children and adolescents with primary dystonia in which there is intact sensory processing do not benefit from feedback augmentation, whereas children with secondary dystonia, in which sensory deficits are often present, exhibit a higher learning capacity when augmented movement-related sensory information is provided. This study represents a fundamental investigation to address the scarcity of noninvasive therapeutic interventions for young subjects with dystonia.

Cognitive approach to rehabilitation in children with hyperkinetic movement disorders post-DBS.

OBJECTIVE: This proof-of-concept feasibility trial examined the potential of the Cognitive Orientation to daily Occupational Performance Approach (CO-OP) to augment deep brain stimulation (DBS) outcomes in childhood-onset hyperkinetic movement disorders (HMD) including dystonia and dyskinetic cerebral palsy. METHODS: This is a single case experimental design using multiple baseline as n-of-1 trial comprising 10 intervention sessions, with replications across participants (n = 10). Treatment focused on 3 participant-selected goals. Transfer was assessed on 2 additional untreated goals. Individuals enrolled were 6-21 years of age and had DBS in situ and sufficient manual ability. Primary outcome was functional performance change on the Performance Quality Rating Scale-Individualized (PQRS-i) measured before, during, and posttreatment, and at 3-month follow-up. Assessors of outcome were blinded to time of assessment, number of intervention session, and treatment allocation. To measure effect size, a nonoverlapping index, Tau-U, was used. Feasibility measures were captured. RESULTS: One participant withdrew before baseline assessment. Effect sizes of at least 0.66 were seen at both posttreatment and follow-up with all participants showing improvements in at least one trained goal in PQRS-i. Six participants improved on all 3 goals and 2 improved on 2 trained goals. Two children showed deterioration in one trained goal each. Transfer to untrained goals was observed in 3 participants for a total of 5 goals. CO-OP was feasible and acceptable to all participants. CONCLUSION: A cognitive-based, task-oriented approach to support performance of personally relevant functional skills enabling participation is acceptable in childhood-onset HMD post-DBS. Further, preliminary efficacy to improve outcomes and proof of concept with CO-OP has been established in this population. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for children with HMD who had undergone DBS, CO-OP improves performance of personally relevant functional skills.

Long-Term Specialized Physical Therapy in Cervical Dystonia: Outcomes of a Randomized Controlled Trial.

OBJECTIVE: To evaluate the effectiveness of a specialized physical therapy (SPT) program on disability in cervical dystonia (CD) compared to regular physical therapy (RPT). DESIGN: A single-blinded randomized controlled trial. SETTING: This study was performed by a physical therapist in a primary health care setting. Measurements were performed at baseline, 6 and 12 months in the botulinum toxin (BoNT) outpatient clinic of the neurology department. PARTICIPANTS: Patients with primary CD and stable on BoNT treatment for 1 year (N=96). MAIN OUTCOME MEASURES: The primary outcome was disability assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Secondary outcomes were pain, anxiety, depression, quality of life (QOL), and health related costs over 12 months. RESULTS: A total of 72 participants (30 men, 42 women) finished the study: 40 received SPT, 32 RPT. No significant between group differences were found after 12 months of treatment (P=.326). Over these 12 months both groups improved significantly (P<.001) on the TWSTRS disability scale compared to baseline (SPT 1.7 points, RPT 1.0 points). Short Form 36 (SF-36) General Health Perceptions (P=.046) and self-perceived improvement (P=.007) showed significantly larger improvements after 12 months in favor of SPT. Total health related costs after 12 months were $1373±556 for SPT compared to $1614±917 for RPT. CONCLUSION: SPT revealed no significant differences compared to RPT after 12 months of treatment on the TWSTRS disability scale. Both groups showed similar improvements compared to baseline. Positive results in the SPT group were higher patient perceived effects and general health perception. Treatment costs were lower in the SPT group. With lower costs and similar effects, the SPT program seems to be the preferred program to treat CD.

Dental appliance therapy in pantothenate kinase-associated neurodegeneration: Case report.

Pantothenate kinase-associated neurodegeneration (PKAN) is a rare condition associated with severe protrusive lingual dystonia, a form of oromandibular dystonia. Dental appliance therapy has been described for oromandibular dystonia however there is a lack of literature regarding its application specifically to PKAN. In this report, the authors describe the use of an appliance in conjunction with botulinum toxin injections for the symptomatic treatment of this condition. A satisfactory outcome is achieved which suggests this technique may be of use to other clinicians.

Protocol for N-of-1 trials proof of concept for rehabilitation of childhood-onset dystonia: Study 1: Protocole des essais de validation à effectif unique pour la réadaptation de la dystonie débutant dans l'enfance : Étude 1.

BACKGROUND: Hyperkinetic movement disorders (HMD) are a heterogeneous group of neurological conditions among which dystonia is the predominant disorder and dyskinetic cerebral palsy the largest secondary dystonia group. Currently, there are no evidence-based, non-medical management options for childhood HMD. The Cognitive Orientation to daily Occupational Performance (CO-OP) Approach is a task-oriented, performance-based intervention that focuses on participation. PURPOSE: This paper reports the protocol for a proof-of-concept study to assess feasibility and preliminary evidence regarding efficacy of CO-OP for HMD following deep brain stimulation (DBS). METHOD: A series of N-of-1 trials with replications will be conducted with children, ages 6 and 21 years with HMD and DBS as indicated by the Manual Ability Classification System. Ten individualized CO-OP sessions, with multiple baselines before, during, and after, will be completed. The primary outcome measures are the Performance Quality Rating Scale and the Assessment of Motor and Process Skills. Outcome data will be plotted over time for each participant and supplemented with graph statistical analysis and effect size estimates. IMPLICATIONS: A written protocol will be developed based on evidence and feedback incorporating any changes to the CO-OP intervention for children and young people with HMD, as per the Medical Research Council's Framework for Complex Interventions.

Protocol for N-of-1 trials with replications across therapists for childhood-onset dystonia rehabilitation: Study 2: Protocole des essais à effectif unique avec répétitions par différents ergothérapeutes pour la réadaptation de la dystonie débutant dans l'enfance : Étude 2.

BACKGROUND: Currently, no evidence-based rehabilitation interventions are available for hyperkinetic movement disorders (HMD), including dyskinetic cerebral palsy (CP). Among these highly heterogeneous disorders, dystonia is the predominant disorder. The Cognitive Orientation to daily Occupational Performance (CO-OP) Approach-a task-oriented, performance-based intervention to enable participation-is currently being evaluated for its potential as an intervention option. PURPOSE: This paper reports the protocol for the second of two studies designed to evaluate the potential of CO-OP to improve functional outcomes for individuals with HMD following deep brain stimulation (DBS). This second study is a systematic replication across multiple treating therapists from multiple centres. METHOD: Systematic replications will be used across centres and treating therapists trained in the CO-OP, using a series of randomized multiple-baseline N-of-1 trials. Participants will be ages 6 to 21 years with HMD and DBS as indicated by the Manual Ability Classification System. Data collection will involve multiple data points collected at baseline, during intervention, and after intervention. The intervention will involve occupation-based goal setting followed by 10 individualized CO-OP sessions. The primary outcome measures are the Performance Quality Rating Scale and the Canadian Occupational Performance Measure. Outcome data will be plotted over time for each participant and supplemented with graph statistical analysis and estimate size effect for N-of-1 trials. IMPLICATIONS: The results of this study will help to inform future training procedures and future clinical trials.

PEDiDBS: The pediatric international deep brain stimulation registry project.

BACKGROUND: Interactive neuromodulation represents the evolving frontier in surgical treatment of a variety of disorders involving the sensory organs and nervous system. Building on the advances of pioneering neurologists and neurosurgeons, progress has been made since the introduction of deep brain stimulation (DBS). Microelectrode recording has greatly aided our understanding of the underlying pathogenesis of movement disorders and the effects of electrical stimulation. The introduction of image - guided systems to provide targeted, controlled neuro-stimulation to specific brain areas is an emerging technique for implantation and may have special appeal for pediatric patients. RATIONALE/AIM: DBS is generally accepted as a treatment for some forms of childhood dystonia. Its potential role in other pediatric movement disorders is less well established. This is important, as most forms of dystonia begin in childhood or adolescence and many are inadequately responsive to current pharmacotherapy and other interventions. Nonetheless, many aspects of deep brain stimulation need clarification. CONCLUSION: This can only be accomplished through an organized platform for data sharing that will allow questions to be asked and hopefully answered, with the ultimate goal of developing evidence based practice based guidelines elucidating the role of DBS in pediatric patients.

Bilateral globus pallidus internus deep brain stimulation for dyskinetic cerebral palsy supports success of cochlear implantation in a 5-year old ex-24 week preterm twin with absent cerebellar hemispheres.

BACKGROUND: Early onset dystonia (dyskinesia) and deafness in childhood pose significant challenges for children and carers and are the cause of multiple disability. It is particularly tragic when the child cannot make use of early cochlear implantation (CI) technology to relieve deafness and improve language and communication, because severe cervical and truncal dystonia brushes off the magnetic amplifier behind the ears. Bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) neuromodulation can reduce dyskinesia, thus supporting CI neuromodulation success. METHODS: We describe the importance of the order of dual neuromodulation surgery for dystonia and deafness. First with bilateral GPi DBS using a rechargeable ACTIVA-RC neurostimulator followed 5 months later by unilateral CI with a Harmony (BTE) Advanced Bionics Hi Res 90 K cochlear device. This double neuromodulation was performed in series in a 12.5 kg 5 year-old ex-24 week gestation-born twin without a cerebellum. RESULTS: Relief of dyskinesia enabled continuous use of the CI amplifier. Language understanding and communication improved. Dystonic storms abated. Tolerance of sitting increased with emergence of manual function. Status dystonicus ensued 10 days after ACTIVA-RC removal for infection-erosion at 3 years and 10 months. He required intensive care and DBS re-implantation 3 weeks later together with 8 months of hospital care. Today he is virtually back to the level of functioning before the DBS removal in 2012 and background medication continues to be slowly weaned. CONCLUSION: This case illustrates that early neuromodulation with DBS for dystonic cerebral palsy followed by CI for deafness is beneficial. Both should be considered early i.e. under the age of five years. The DBS should precede the CI to maximise dystonia reduction and thus benefits from CI. This requires close working between the paediatric DBS and CI services.

A comparative historical and demographic study of the neuromodulation management techniques of deep brain stimulation for dystonia and cochlear implantation for sensorineural deafness in children.

Cochlear implants for sensorineural deafness in children is one of the most successful neuromodulation techniques known to relieve early chronic neurodisability, improving activity and participation. In 2012 there were 324,000 recipients of cochlear implants globally. AIM: To compare cochlear implant (CI) neuromodulation with deep brain stimulation (DBS) for dystonia in childhood and explore relations between age and duration of symptoms at implantation and outcome. METHODS: Comparison of published annual UK CI figures for 1985-2009 with a retrospective cohort of the first 9 years of DBS for dystonia in children at a single-site Functional Neurosurgery unit from 2006 to 14. RESULTS: From 2006 to 14, DBS neuromodulation of childhood dystonia increased by a factor of 3.8 to a total of 126 cases over the first 9 years, similar to the growth in cochlear implants which increased by a factor of 4.1 over a similar period in the 1980s rising to 527 children in 2009. The CI saw a dramatic shift in practice from implantation at >5 years of age at the start of the programme towards earlier implantation by the mid-1990s. Best language results were seen for implantation <5 years of age and duration of cochlear neuromodulation >4 years, hence implantation <1 year of age, indicating that severely deaf, pre-lingual children could benefit from cochlear neuromodulation if implanted early. Similar to initial CI use, the majority of children receiving DBS for dystonia in the first 9 years were 5-15 years of age, when the proportion of life lived with dystonia exceeds 90% thus limiting benefits. CONCLUSION: Early DBS neuromodulation for acquired motor disorders should be explored to maximise the benefits of dystonia reduction in a period of maximal developmental plasticity before the onset of disability. Learning from cochlear implantation, DBS can become an accepted management option in children under the age of 5 years who have a reduced proportion of life lived with dystonia, and not viewed as a last resort reserved for only the most severe cases where benefits may be at their most limited.

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.

Scaled Vibratory Feedback Can Bias Muscle Use in Children With Dystonia During a Redundant, 1-Dimensional Myocontrol Task.

Vibratory feedback can be a useful tool for rehabilitation. We examined its use in children with dystonia to understand how it affects muscle activity in a population that does not respond well to standard rehabilitation. We predicted scaled vibration (ie, vibration that was directly or inversely proportional to muscle activity) would increase use of the vibrated muscle because of task-relevant sensory information, whereas nonscaled vibration would not change muscle use. The study was conducted on 11 subjects with dystonia and 14 controls. Each subject underwent 4 different types of vibration on the more dystonic biceps muscle (or nondominant arm in controls) in a 1-dimensional, bimanual myocontrol task. Our results showed that only scaled vibratory feedback could bias muscle use without changing overall performance in children with dystonia. We believe there may be a role in rehabilitation for scaled vibratory feedback to retrain abnormal muscle patterns.

Informe de evaluación científica en la evidencia disponible: distonía / Scientific evaluation report on the available evidence: dystonia

INTRODUCCIÓN: La distonía es un desorden del movimiento común caracterizado por la contracción muscular sostenida o intermitente que causa movimientos anormales (distónicos), posturas anormales e incluso temblores. Los síntomas frecuentemente comienzan en las extremidades, generalmente en las piernas, para luego avanzar a otras áreas del cuerpo hasta convertirse en un síntoma generalizado en más del 50% de los pacientes. En cambio, cuando el paciente presenta una manifestación tardía, posterior a los 21 años, es más común que empiece por el cuello, brazos o cabeza y tienda a mantenerse de forma focal o segmental. La Estimulación Cerebral Profunda (ECP) actúa eléctricamente en el cerebro mediante un dispositivo, similar a un marcapaso, que genera pulsos eléctricos que son transmitidos a través de electrodos, que son implantados quirúrgicamente en la zona interna del cerebro. Este informe evalúa el uso de este implante para la ECP en pacientes con distonía generalizada severa, que no respondan al tratamiento farmacológico y a las inyecciones de toxina botulínica. TECNOLOGÍAS SANITARIA DE INTERÉS: La estimulación cerebral profunda estimula eléctricamente el cerebro, mediante un dispositivo, similar a un marcapaso, que genera pulsos eléctricos que son transmitidos a través de electrodos, los cuales se implantan quirúrgicamente en la zona interna del "globus pallidus". Este tratamiento no es curativo, del mismo modo que los medicamentos, solo disminuyen los síntomas. EFICACIA DE LOS TRATAMIENTOS: Se encontraron siete revisiones sistemáticas que evaluaban el uso del ECP para pacientes con distonía. En pacientes con distonía primaria severa generalizada con mutación DYT1 de origen desconocido, la evidencia muestra que la ECP en el Globus pallidus internus (GPi) tiene eficacia a largo plazo, pero complicaciones inusuales tardías como la acinesia o la congelación de la marcha. Se observa que un mayor deterioro en la línea de base y una menor edad en la cirugía de ECP estuvieron positivamente relacionados con mejores resultados con GPi-ECP. Todas las revisiones sistemáticas presentan las mismas limitaciones significativas, relacionadas a que la evidencia recopilada son estudios de casos, recopilación de datos multicéntricos, estudios retrospectivos. No hay evidencia disponible sobre estudios clínicos aleatorizados. Entre las desventajas que presenta el ECP se encuentra la necesidad de mantenimiento del dispositivo instalado y la sustitución periódica de la batería. Además, ECP conlleva el riesgo de infección de los materiales implantados, ruptura de los cables de conexión y migración del material. ALTERNATIVAS DISPONIBLES: Tratamiento farmacológico: Muchos agentes orales han sido usados para el tratamiento de la distonía, pero ellos no han sido extensivamente estudiados en ensayos controlados rigurosos. La evidencia disponible sugiere que muchas drogas benefician a una proporción de pacientes con distonía. Inyecciones de toxina botulinum: La toxina botulínica (BoNT) es una potente neurotoxina producida por Clostridium botulinum que causa debilidad muscular regional a través de su acción como una endopeptidasa de zinc que escinde proteínas específicas implicadas en la fusión vesicular. La aplicación de esta toxina reduciría la hiperactividad muscular asociada a esta condición de salud. Cirugía: El tratamiento quirúrgico está reservado para pacientes con distonía grave que no reciben tratamiento con agentes farmacológicos e inyecciones de BoNT. El pilar del tratamiento quirúrgico es la estimulación del microelectrodo profundo del cerebro del segmento interno del globus. RESULTADOS DE LA BÚSQUEDA DE EVIDENCIA: La información presentada fue extraída de 3 revisiones sistemáticas relevantes para la pregunta formulada, las que fueron publicadas entre los años 2006 y 2017, que evaluaron el uso de ECP para pacientes con distonía. En las 3 revisiones sistemáticas utilizadas, se reportaron 2 Ensayos Controlados Aleatorizados (ECAs) que comparaban el uso de ECP contra placebo (sham estimulation) en pacientes con distonía. CONCLUSIÓN: Para dar cumplimiento al artículo 28° del Reglamento que establece el proceso destinado a determinar los diagnósticos y tratamientos de alto costo con Sistema de Protección Financiera, según lo establecido en los artículos 7°y 8° de la ley N°20.850, aprobado por el decreto N°13 del Ministerio de Salud, se concluye que el presente informe de evaluación se considera favorable, de acuerdo a lo establecido en el Título III. de las Evaluaciones Favorables de la Norma Técnica N° 0192 de este mismo ministerio.

Hypokinetic gait changes induced by bilateral pallidal deep brain stimulation for segmental dystonia.

BACKGROUND: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been established as an effective and safe treatment for dystonia. In general, side effects are rare, but there is increasing evidence that GPi DBS in dystonia can induce hypokinetic symptoms like micrographia or freezing of gait. We aimed to evaluate and quantify possible changes of gait following bilateral chronic GPi DBS for dystonia by computerized gait analyses. METHODS: We prospectively performed computerized gait analysis in ten consecutive patients (mean age 57.8+/-14.3 years) with segmental dystonia but without involvement of lower trunk or legs who were treated with bilateral GPi DBS. Using pressure sensitive insoles, several parameters were measured preoperatively (pre-OP) and at a median of 7 months postoperatively. RESULTS: The mean step length significantly decreased from 60.0+/-6.9cm pre-OP to 54.3+/-6.4cm with GPi DBS (p<0.01). Due to only small changes of walking distance and gait velocity, the cadence correspondingly increased from 105.6+/-9.2 steps/min to 111.3+/-11.4 steps/min (p<0.05). More importantly, the variance of several gait parameters significantly decreased postoperatively. CONCLUSIONS: In patients with segmental dystonia, chronic DBS of the posteroventral lateral GPi is associated with only mild hypokinesia of gait, but with a relevant decrease in gait variability. Given other recently reported hypokinetic effects of GPi DBS for dystonia and recent results of electrophysiological coherence studies, these findings support the hypothesis of a general alteration of neuronal activity in striato-pallido-thalamo-cortical motor pathways following chronic stimulation of the posteroventral lateral GPi.

Successful treatment of paroxysmal tonic spasms with topiramate in a patient with neuromyelitis optica.

A 49-year-old woman with neuromyelitis optica (NMO) developed severe quadriplegia and frequent paroxysmal tonic spasms (PTS). Carbamazepine, although initially effective against PTS, caused drug eruption and she was unable to continue. PTS re-emerged after discontinuation of carbamazepine and hindered rehabilitation. Then topiramate was started, and PTS promptly disappeared. The patient became able to resume rehabilitation and her activity of daily life improved significantly. Carbamazepine and topiramate have a common pharmacological action to block voltage-gated sodium channels. The action may have contributed to inhibition of ephaptic transmission in the demyelinating lesions by NMO and eventually improved PTS.

Impact of paroxysmal sympathetic hyperactivity on nutrition management after brain injury: a case series.

OBJECTIVE: To review the nutritional requirements of all new inpatient brain injury admissions presenting with Paroxysmal Sympathetic Hyperactivity (PSH) in the rehabilitation setting. METHODS: RABIU is a 25-bed Regional facility in Northern Ireland which opened in 2006. All records of patients with a single episode non-progressive acquired brain injury admitted to RABIU from 2006 until the present were reviewed for evidence of PSH. Dietetic assessment and management was examined and recorded. RESULTS: Four patients with persisting paroxysmal sympathetic hyperactivity were identified. All patients displayed dystonia and posturing and had clinically important percentage weight loss. All had nutrition and/or hydration requirements markedly above their estimated requirement for slow weight gain, despite adjustment for brain injury. All four had posture-related complications of their dystonia and nutrition. CONCLUSION: Careful monitoring of nutrition, hydration and mineral supplementation is paramount in patients presenting with paroxysmal sympathetic hyperactivity after brain injury. It is argued that morbidity may be reduced by aggressive and expert nutrition management.