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.

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.

Physical therapy for a child with sudden-onset choreoathetosis: a case report.

PURPOSE: This case report describes the physical therapy examination, intervention, and outcomes for a 5-year-old girl who developed choreoathetosis following mitral valve repair. CASE DESCRIPTION: This child was admitted to an inpatient short-term rehabilitation program with marked choreoathetosis and dependence for all functional mobility. She received physical therapy twice a day for 5 weeks. Physical therapy intervention included therapeutic exercise emphasizing stabilization and closed chain exercises, aquatic therapy, and functional training to improve gross motor skills and mobility. Tests and measures included the Selective Control Assessment of the Lower Extremity, 66-item Gross Motor Function Measure, and Pediatric Evaluation of Disability Inventory. OUTCOMES: At discharge, this child demonstrated improvements in her Selective Control Assessment of the Lower Extremity, Gross Motor Function Measure, and Pediatric Evaluation of Disability Inventory scores. She was independent in all functional mobility tasks. SUMMARY: This case study describes physical therapy tests and measures, intervention, and positive outcomes for a child with sudden-onset choreoathetosis.

Movement disorders after status epilepticus and other brain injuries.

A retrospective medical record review was conducted of 173 consecutive children hospitalized for acquired brain injuries on a specialized pediatric rehabilitation service. The chart review identified children who developed movement disorders with acquired brain injuries: 8 with status epilepticus, 2 with trauma, and 1 with anoxia. Movement disorders were observed more frequently following status epilepticus (8 of 12) than following other causes of acquired brain injury (3 of 161; P = .0001). Four additional children had severe neurologic deficits following status epilepticus but did not develop movement disorders. The 11 patients who developed movement disorders had choreiform movements predominantly. Even though status epilepticus is a clinical phenomenon resulting from a variety of etiologies, the features of movement disorders in these children were strikingly similar. The pathophysiology of this complication is unknown.

[Appraisal of work capacity and rehabilitation in infantile cerebral palsy]. / Voprosy ékspertizy trudosposobnosti i reabilitatsii pri detskom tserebral'nom paraliche.

In clinical conditions 189 patients with infantile cerebral paralysis were examined, and ways of their medical, family, social, and working rehabilitation were analyzed. The medical rehabilitation appeared to be the most effective in patients with pareses of the extremities and speech disorders. The prognosis as regards the working capacity was the most favourable in patients with mild and mode;ate pareses (hemi-, para-, and fetrapareses) and unfavourable in patients with severe hyperkinesias, pronounced oligophrenia, and combined pathology (hyperkinesias + tetrapareses; tetrapareses + debility and/or speech disorders). While specifying the disability groups attention was paid to the degree of the function impairment (mild, moderate, grave) and to the working prognosis. Patients with pronounced tetrapareses and contractures in all the joints, grave hyperkinesias in all the four extremities, and imbecility were classed with disability group I: those with pronounced para-, hemi-, and tetrapareses, extensive hyperkinesias, combination of the motor disorders with debility were placed into disability group II. While classing patients with disability group III the character and degree of the function impairments, the patients profession, contraindicated factors of the work, and compensatory potentialities were taken into account. The studies have shown that the existing scepticism with regard to the working capacity prognosis in patients with infantile cerebral paralysis is due to insufficient elucidation of their working potentialities in literature.