Variety Of Hyperkinetic Movement Disorders Research Articles

Vascular Hemiballismus Due to Extraluysian Lesions

Hemiballismus represents a rare variety of hyperkinetic movement disorders that can complicate early-stage stroke. It is classically seen in contralateral Luysian lesions. We present the case of a 63-year-old hypertensive patient consulted for the sudden onset of involuntary movements. The neurological examination revealed right hemiballismus. Brain CT scan showed lacunar images of the internal capsule and left subthalamic muscles. The integrity of the subthalamic nucleus (STN) was confirmed by brain magnetic resonance image. The etiological assessment concluded that it was paroxysmal atrial fibrillation. The evolution was favorable after treatment with haloperidol. Hemiballismus represents less than 7% of movement disorders. It is usually linked to a vascular lesion of the contralateral STN. Extraluysian involvement remains rare.

A Retrospective Longitudinal Study in a Cohort of Children With Dyskinetic Cerebral Palsy Treated With Tetrabenazine.

Tetrabenazine has been studied with a variety of hyperkinetic movement disorders, but there is limited and empirical literature on the potential efficacy of tetrabenazine in children with dyskinetic cerebral palsy (DCP). The purpose of this study was to evaluate the efficacy of tetrabenazine in a sample of children with DCP using the Movement Disorders—Childhood Rating Scale 4–18 Revised (MD-CRS 4–18 R). The study is a multicenter retrospective longitudinal study in which the participants were selected from the databases of each Center involved, according to detailed inclusion criteria. The study was performed on 23 children and adolescents (19 male and 4 females; mean age 8.28 years, SD 3.59) with DCP having been evaluated before starting the treatment (baseline), after 6 and 12 months of treatment and in a sub-cohort after >2 years follow-up. A linear mixed model was used to evaluate the effects of the different timings on each MD-CRS 4–18 R Index (Index I, Index II, and Global Index) adding age and type of movement disorder as random effect. A significant clinical improvement related to a reduction of MD-CRS 4-18 R Indexes was detected between the baseline and after 6 and 12 months of treatment. Findings support the efficacy of tetrabenazine in children with DCP through a standardized outcome measure (MD-CRS 4–18 R) and confirm the use of this scale as a suitable tool to detect changes in further randomized clinical trials.

Hyperkinetic movement disorders in congenital disorders of glycosylation.

Congenital disorders of glycosylation (CDG) represent an increasing number of rare inherited metabolic diseases associated with abnormal glycan metabolism and disease onset in infancy or early childhood. Most CDG are multisystemic diseases mainly affecting the central nervous system. The aim of the current study was to investigate hyperkinetic movement disorders in patients affected by CDG and to characterize phenomenology based on CDG subtypes. Subjects were identified from a cohort of patients with CDG who were referred to the University Hospital of Catania, Italy. Patients were evaluated by neurologists with expertise in movement disorders and videotaped using a standardized protocol. A variety of hyperkinetic movement disorders was detected in eight unrelated CDG patients. Involuntary movements were generally observed early in childhood, maintaining a clinical stability over time. Distribution ranged from a generalized, especially in younger subjects, to a segmental/multifocal involvement. In patients with phosphomannomutase 2 CDG, the principal movement disorders included dystonia and choreo-athetosis. In patients affected by other CDG types, the movement disorders ranged from pure generalized chorea to mixed movement disorders including dystonia and complex stereotypies. Hyperkinetic movement disorder is a key clinical feature in patients with CDG. CDG should be considered in the differential diagnosis of childhood-onset dyskinesia, especially when associated with ataxia, developmental delay, intellectual disability, autism or seizure disorder.

Safety and Efficacy of Tetrabenazine and Use of Concomitant Medications During Long-Term, Open-Label Treatment of Chorea Associated with Huntington's and Other Diseases.

Although tetrabenazine, a drug that depletes presynaptic dopamine by inhibiting vesicular monoamine transporter 2 (VMAT2), was approved by the U.S. Food and Drug Administration in 2008 for the treatment of chorea associated with Huntington's disease (HD), there is a paucity of data on its long-term efficacy and safety. Approximately 2,000 patients with a variety of hyperkinetic movement disorders had been treated with open-label tetrabenazine at the Movement Disorders Clinic, Baylor College of Medicine, since 1979. Tetrabenazine was usually started at 12.5 mg/day, and the dosage was gradually increased (up to 300 mg/day). Responses were rated by the investigator 1-5, with 1 = marked chorea reduction, excellent improvement in function; 2 = moderate chorea reduction, very good improvement in function; 3 = fair chorea improvement, only mild improvement in function; 4 = poor or no response for chorea and function; and 5 = worsening chorea, some functional deterioration. Efficacy and safety were analyzed retrospectively. By 2004, 98 HD chorea patients had received tetrabenazine for a mean of 3.1 years (range ≤1-11.4 years). Of those with valid ratings, 75% had either marked or very good responses (rating 1 or 2) at their optimal dosages. The most common adverse events occurring in ≥5% of the patients were somnolence (39%), insomnia (33%), depression (31%), accidental injury (26%), and dysphagia (19%). Efficacy and safety were comparable to results for non-HD chorea patients. Tetrabenazine treatment was associated with long-term improvement in chorea. Adverse event rates were comparable to those reported from controlled trials.

Movement disorder in a patient with stroke

The T2-weighted MRI and the diffusion weighted image (DWI) MRI show high signal intensity in the left putamen, and there was a corresponding isointense lesion on the apparent diffusion coefficient (ADC) map. These features are consistent with an ischaemic stroke. The MRI brain was performed four days after symptom onset. A variety of hyperkinetic movement disorders can occur (uncommonly) after strokes in the basal ganglia, or elsewhere in the brain. 1–3 In three relatively large series, the incidence of poststroke chorea/ballism was reported to range from 0.4% to 1.3%. 1–3 Hemichorea and hemiballism are believed to form part of a clinical spectrum, with shared pathophysiologic mechanisms; a frequent observation is for ballism to go through a phase of chorea prior to resolution. 4,5

Comparison of Weight Gain in Treatments for Tourette Syndrome: Tetrabenazine Versus Neuroleptic Drugs

Weight gain occurs with most neuroleptic drugs used to treat tics. Tetrabenazine, a vesicular monoamine transporter type 2 inhibitor, inhibits dopamine release. It is used to treat a variety of hyperkinetic movement disorders, including tics. Weight gain over time was compared in a group of pediatric tic patients taking only tetrabenazine with an age-matched group of tic patients taking only neuroleptic drugs. Age in the tetrabenazine group (n = 36, 32 males) at initial therapy was 13.4 (SD 3.3) years. They were monitored for 25.2 (SD 12.3) months. The average dose of tetrabenazine (all visits) was 54.4 (SD 26.6) mg/d. In the neuroleptic group (n = 41, 33 males), the age at therapy onset was 12.3 +/- 4.0 years. They were monitored for 18.9 (SD 14.8) months. Weight increase was 0.8 lb/mo in the tetrabenazine group compared with 1.7 lb/mo in the neuroleptic group (P = .006). Most patients who switched from a neuroleptic drug to tetrabenazine subsequently lost weight. Although the study was not designed to compare efficacy, this tended to be greater with tetrabenazine than with neuroleptic drugs.

Tetrabenazine for hyperglycemic‐induced hemichorea–hemiballismus

We reported a 74-year-old woman with new-onset diabetes mellitus who presented with the sudden onset of mild left hemiparesis and marked left hemichorea-hemiballismus. Brain CT scan and MRI showed T1W, T2W, and DWI lesions in the right putamen and caudate, which have been previously reported in cases of hyperglycemic-induced hemichorea-hemiballismus (HIHH). The patient dramatically responded to tetrabenazine within a day. Subsequent dose reductions lead to a reemergence of symptoms. Tetrabenazine improves a variety of hyperkinetic movement disorders but, to our knowledge, its use has never been reported for HIHH.

Tetrabenazine Treatment in Movement Disorders

Tetrabenazine (TBZ) is a catecholamine depletor used for the treatment of a variety of movement disorders. The purpose of this study was to assess the efficacy of TBZ in a retrospective chart review in 3 tertiary care movement disorders centers over long-term treatment. Of 150 patients to whom TBZ was prescribed, 118 were followed up and assessed using the Clinical Global Impression of Change (CGIC), (-3 to +3), a composite grade from a patient and caregiver scale over variable periods. The patients had a variety of hyperkinetic movement disorders including dystonia (generalized and focal: axial, Meige syndrome, torticollis, blepharospasm, bruxism), Huntington disease (HD) or other choreas, tardive dyskinesia (TD) or akathisia, and Tourette syndrome. Mean patient age was 48.8 +/- 18.7 years; 48 were men (40.7%) with a mean disease duration of 93 months. The mean follow-up time was 22 months and the mean TBZ dose was 76.2 +/- 22.5 mg/d (median 75 mg, range 25-175 mg/d). The mean CGIC score was +1 (mild improvement). The group of patients who scored +3 on the CGIC (very good improvement) represented 18.6% (n = 22) of all patients. They had HD or other types of chorea 7.6% (n = 9), facial dystonia/dyskinesia (n = 7, 5.9%), 1 with TD, 2 with trunk dystonia, 2 with Tourette syndrome, and 1 with tardive akathisia. This group had the longest treatment duration and received a mean TBZ dose of 70.5 mg/d (median 75 mg/d) for a mean of 25.4 +/- 21.3 months. The report concludes that TBZ is a moderately effective treatment of a large variety of hyperkinetic movement disorders, with excellent effects in a subgroup with chorea and facial dystonia/dyskinesias.

Long-term effects of tetrabenazine in hyperkinetic movement disorders.

Over the past 15 years we have treated 526 patients with severe hyperkinetic movement disorders with tetrabenazine (TBZ), a monoamine-depleting and a dopamine-receptor-blocking drug. We report here the results in 400 patients with adequate follow-up. The response was rated on a scale of 1 to 5 (1 = marked improvement, 4 = no response, 5 = worsening) and was assessed initially and at the last clinic visit. The average duration of TBZ treatment was 28.9 months (+/- 31.1; range, 0.25 to 180 months). The global response rating of 1 (marked improvement) was recorded in 89.2% of 93 patients with tardive stereotypy, 83.3% of 12 with myoclonus, 82.8% of 29 with Huntington's disease, 80.5% of 82 with tardive dystonia, 79.3% of 29 with other movement disorders, 62.9% of 108 with idiopathic dystonia, and in 57.4% of 47 with Tourette's syndrome. The most common side effects included drowsiness (36.5%), parkinsonism (28.5%), depression (15.0%), insomnia (11.0%), nervousness or anxiety (10.3%), and akathisia (9.5%). The side effects were controlled with reduction in the dosage. TBZ is an effective and safe drug for the treatment of a variety of hyperkinetic movement disorders. In contrast to typical neuroleptics, TBZ has not been demonstrated to cause tardive dyskinesia.

Botulinum A exotoxin for glabellar folds: A double-blind, placebo-controlled study with an electromyographic injection technique

Background: Botulinum A exotoxin (BTX) has been used successfully to treat a variety of hyperkinetic movement disorders. BTX is also capable of reducing hyperkinetic facial lines including prominent glabellar frown lines. Objective: The purposes of this study were to (1) confirm the efficacy of BTX in a double-blind, placebo-controlled investigation; (2) evaluate the use of an electromyogram attached to the injection needle to confirm intramuscular corrugator placement of the BTX; and (3) determine the optimum direction injection technique. Methods: Length and depth of glabellar frown lines were measured before treatment and 4 and 12 weeks after injection of 10 units of BTX or saline solution. Results: Patients treated with BTX had a highly significant reduction in depth and length of glabellar frown lines compared with control subjects. Conclusion: BTX appears to be effective and safe for reduction of glabellar frown lines.