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.

Inferior olivary hypertrophy is associated with a lower functional state after pontine hemorrhage.

BACKGROUND: Inferior olivary hypertrophy (IOH) may develop after pontine hemorrhage and may become a pacemaker for symptomatic palatal tremor (SPT). However, there is no information available that elucidates how IOH may affect the functional outcome. The purpose of this study was to investigate how frequently IOH was associated with clinical manifestations of involuntary movements, including ocular myoclonus (OM) and SPT, and whether IOH influenced the functional outcome after pontine hemorrhage. METHODS: In 20 consecutive patients undergoing inpatient multidisciplinary rehabilitation after pontine hemorrhage, the location of lesions (tegmental vs. ventral) and the presence of IOH were examined by magnetic resonance imaging, and the functional outcome was assessed by means of Fugl-Meyer scale for neurological impairment and Functional Independence Measure for disability on admission and discharge. RESULTS: In 10 patients, IOH was detected, and the tegmentum was involved in 7 of the 10 patients. OM or SPT was present in 7 of these 10 patients. In the remaining 10 patients, IOH was not detected, and the tegmentum was involved only in 2 of these 10 patients. None of them had OM or SPT. The presence of IOH was associated with a lower functional status on admission and discharge. CONCLUSION: After pontine hemorrhage, IOH may be associated with tegmental lesions with OM or SPT and may impose a detrimental effect on the functional outcome.

Myoclonus of the scapula after acute long thoracic nerve lesion: a case report.

We describe a patient who presented myoclonus in the left scapula 3 months after a traumatic lesion of the left long thoracic nerve. Myoclonic activity was recorded as pseudorhythmic electromyographic bursts repeated at a frequency of 2 to 4 Hz, each lasting between 100 and 200 msec, in the left serratus-dorsalis muscle region, trapezius, and deltoid muscles. A combination of peripheral and central mechanisms may have induced the myoclonus in this case.

Post-anoxic myoclonus: a case presentation and review of management in the rehabilitation setting.

Post-anoxic myoclonus is a rare movement disorder manifested by diffuse action-triggered jerking movements that may result in significant disability. The incidence of this disorder is not clearly established, but over 122 cases have so far been reported in the literature. The pathogenesis is not entirely known, although it has been hypothesized that particular susceptibility of the Purkinje cells of cerebellum to anoxic injury may play a key role. A case is presented of an independently living 60-year-old woman admitted to the rehabilitation unit with diffuse myoclonus after sustaining a cardio-pulmonary arrest. She presented with severe jerking movements in all extremities with startle to noise and exacerbations upon attempting any purposeful actions. The patient's myoclonus was controlled by a variety of anti-epileptic medications. The patient initially responded to a combination of divalproex sodium and zonisamide, but relapsed in several weeks, requiring addition of levetriacetam and clonazepam. At a 1-year follow-up she demonstrated a significant improvement in ambulation and self-care activities. This case illustrates that, although patients with post-anoxic myoclonus cannot be fully cured, their quality of life can be significantly improved by rehabilitation interventions.

Tizanidine for the treatment of intention myoclonus: a case series.

OBJECTIVE: To evaluate the effectiveness of tizanidine in treating intention myoclonus. DESIGN: Case series. SETTING: Inpatient rehabilitation center. PARTICIPANTS: Three subjects whose activities of daily living were impaired due to intention myoclonus related to mitochondrial encephalomyopathy, stroke, and multiple sclerosis (MS). INTERVENTION: Tizanidine. MAIN OUTCOME MEASURES: Reduction in intention myoclonus and change in score on the FIM instrument. RESULTS: The patient with mitochondrial encephalomyopathy had left upper- and lower-extremity intention myoclonus; the patient with stroke had left upper intention myoclonus; and the patient with MS had right upper- and left lower-intention myoclonus. In the patient with mitochondrial encephalomyopathy, the FIM score increased from 90 to 103 points over 2 days of tizanidine. The stroke patient's FIM score improved only from 74 to 79 after 4 weeks of tizanidine. The patient with MS improved from 83 to 101 after 6 days of tizanidine. All 3 patients had almost full resolution of the intention myoclonus. All continued on tizanidine except the patient with stroke, who had minimal gains and a low systolic blood pressure. None of the patients experienced significant sedation or hypotension. CONCLUSIONS: Tizanidine may be a safe and effective option for treating intention myoclonus that occurs in a variety of neurologic conditions.

Action myoclonus following acute cerebral anoxia.

Action myoclonus secondary to posthypoxic encephalopathy is being seen increasingly with improved resuscitation techniques. A case report describes 5 specific physical and occupational therapeutic techniques for achieving independence in ambulation, transfers and self-care: (1) analysis and segmentation of complex motions into small steps; (2) controlled progression of training; (3) voluntary cessation of abnormal activity (pacing); (4) progressive densensitization to external stimuli; and (5) quantification of progress. Literature review suggests that posthypoxic action myoclonus is secondary to a loss of inhibitory synapses in the brainstem reticular formation due to low serotonin levels. The proposed therapeutic effect of clonazepam, the drug used in this patient, is decreased serotinin degredation. L-5-hydroxytryptamine, an investigative drug, is also therapeutic, for it stimulates increased serotonin production.

Myoclonic dystonia.

Myoclonic dystonia is a rare disorder that occurs in an hereditary and a sporadic form. The autosomal-dominantly inherited form is responsive to alcohol but not to other drugs. The sporadic form has been relatively resistant to drug treatment. We report a young man with myoclonic dystonia who displayed only little response to alcohol but improved significantly with a combination of sodium valproate for myoclonus and trihexiphenidyl hydrochloride for dystonia. His rehabilitation, however, was confounded by public authorities who thought the patient's appearance was indicative of drug use.