Ruth Ann Baird, MD
Joanne M. Wojcieszek, MD
DESCRIPTION
Hemiballismus is a hyperkinetic movement disorder characterized by violent flailing movements involving proximal limbs on one side of the body. Hemiballismus is considered an extreme form of chorea because as ballistic movements subside with time, they have the appearance of classic chorea.
EPIDEMIOLOGY
Incidence
- Uncommon, with an annual incidence of around 1 per 500,000 in the general population.
- Age
- Mean age at presentation >60 years.
- Sex
- Hemiballismus occurs equally in men and women.
Prevalence
- Of 3,084 patients seen at a tertiary care movement disorders clinic, only 21 had hemiballismus (1)[C].
RISK FACTORS
Vascular risk factors, especially hypertension, are most important because stroke is the main cause of hemiballismus.
Genetics
Patients of East Asian origin may be at increased risk for hemiballism due to hyperglycemia.
GENERAL PREVENTION
Because stroke is the most common cause of hemiballismus, prevention would involve treatment of vascular risk factors (i.e. hypertension, diabetes, tobacco use).
PATHOPHYSIOLOGY
Damage to the subthalamic nucleus or surrounding pathways leads to loss of normal subthalamic inhibition, which results in abnormal involuntary movements on the contralateral body. Hemiballismus may also result from pathology within the globus pallidus, thalamus, substantia nigra, putamen, or caudate.
ETIOLOGY
- Hemorrhagic and ischemic strokes account for about two-thirds of all cases of ballismus.
- The second most common cause is hyperglycemia associated with diabetes mellitus.
- Other potential etiologies include head trauma, space-occupying lesions, CNS infections, demyelinating disease, autoimmune diseases (especially systemic lupus erythematosus), medications (levodopa, oral contraceptives, phenytoin, tardive syndromes from neuroleptics), and basal ganglia calcification.
Pregnancy Considerations
There is no specific relationship with pregnancy except that chorea gravidarum occasionally can be severe and unilateral.
Pediatric Considerations
Hemiballismus is rare in children; however, Sydenham's chorea can be unilateral and of such large amplitude to resemble hemiballismus. Hemiballistic limb movements have been reported in pediatric patients with ifosfamide-induced encephalopathy (2)[C].
COMMONLY ASSOCIATED CONDITIONS
- Cerebrovascular disease: Ischemic and hemorrhagic stroke, vascular malformations
- Autoimmune disorders: Systemic lupus erythematosus, antiphospholipid antibody syndrome, Sydenham's chorea, scleroderma
- Metabolic disorders: Diabetes, nonketotic hyperglycemic coma, hypoglycemia
- Infectious diseases:HIV/AIDS, syphilis, tuberculosis, toxoplasmosis, cryptococcosis, influenza A
- Tumors: primary CNS malignancies, metastatic tumors, cystic lesions, abscesses
- Drugs: Levodopa, dopamine agonists, neuroleptics, anticonvulsants (e.g., phenytoin), oral contraceptives, gabapentin, cocaine, amphetamines, CNS stimulants
- Iatrogenic: Subthalamotomy for Parkinson disease, ventriculoperitoneal shunt placement
- Head trauma
HISTORY
- Acute or subacute onset, depending on mechanism of injury
- Movements may be suppressed for brief periods of time
- Interference with normal motor activity and stress makes them worse
- Previously unrecognized diabetes may present with hemiballism
PHYSICAL EXAM
- Large amplitude, proximal usually rotatory throwing or kicking movements
- In half of patients, the leg and arm of the same side are equally affected.
- In two-thirds of patients the face is also involved.
- For unknown reasons, the left hemibody is more commonly affected.
DIAGNOSTIC TESTS AND INTERPRETATION
Lab
Initial Lab Tests
- Directed at determining underlying cause
- CBC, blood glucose, serum osmolality, routine blood chemistries, sedimentation rate, Venereal Disease Research Laboratory antinuclear antibodies, antiphospholipid antibodies, PT, aPTT, pregnancy test, urinalysis
Follow-Up & Special Considerations
- In selected patients: HIV test, anticonvulsant blood levels, throat culture, antistreptolysin antibody titers, anti-dsDNA, Sjögren's syndrome A (SSA) and Sjögren's syndrome B (SSB) antibodies
- HgbA1c may be useful in patients who do not have a prior history of diabetes
Imaging
Initial Approach
Brain MRI or CT +/– contrast should be performed to search for a structural cause of hemiballismus.
Follow-Up & Special Considerations
Brain MRI in acute stages of hemiballism due to nonketotic hyperglycemia may show T1 hyperintensity in the contralateral striatum (3)[C].
Diagnostic Procedures/Other
No special procedures are required for diagnosis.
DIFFERENTIAL DIAGNOSIS
- Tic disorder
- Psychogenic movement disorder
MEDICATION
First Line
Neuroleptics
These drugs are the first-line treatment for ballistic movements because of their proven efficacy. Antagonism of the postsynaptic D2 dopamine receptor seems to be the common feature among agents effective in the treatment of hemiballismus. Chlorpromazine, promethazine, perphenazine, prochlorperazine, haloperidol, pimozide, and tiapride, among other neuroleptics, have been shown to be effective in the treatment of hemiballismus. Clozapine in low doses (50 mg/day) also is useful. Response usually is dramatic and starts within 2 days and almost always within 7 days. If treatment is prolonged or there are side effects, consider using a benzodiazepine, a dopamine-depleting agent (e.g., reserpine, tetrabenazine), or a GABA-ergic agent such as valproate.
- Contraindications
- Neuroleptics should not be used in patients with prior history of hypersensitivity, neuroleptic malignant syndrome, prolonged QT syndrome, or neuroleptic-induced movements.
- Tetrabenazine is indicated for neuroleptic-induced ballistic movements.
- Precautions: The main problem with the use of neuroleptics is the development of extrapyramidal side effects, such as akathisia, drug-induced parkinsonism, neuroleptic malignant syndrome, and tardive dyskinesia. Other side effects include sedation, cardiac conduction abnormalities, weight gain, maculopapular rash, cholestatic jaundice, transient leukopenia, and photosensitivity.
Second Line
- Sedative/hypnotics: A variety of sedative drugs (e.g., barbiturates, chloral hydrate, benzodiazepines) have been used for treatment of hemiballismus. Their efficacy is modest and related to their tendency to induce sleep.
- Catecholamine-depleting agents: Tetrabenazine, reserpine
- GABA-ergic agents: Valproic acid
ADDITIONAL TREATMENT
General Measures
Management requires identification of the cause of hemiballismus, mainly focusing on neuroimaging and identifying and treating risk factors, with special emphasis on vascular risk factors.
Issues for Referral
Patients should follow-up with a neurologist.
Additional Therapies
Botulinum toxin injections may be effective in decreasing amplitude of movements (4)[C].
SURGERY/OTHER PROCEDURES
- Surgery is reserved for patients with refractory hemiballismus. The tendency for movements to improve spontaneously over time should be taken into account before planning an invasive procedure.
- Thalamotomy and pallidotomy have been shown to improve hemiballismus secondary to STN lesions. Deep brain stimulation of the globus pallidus and thalamus has been effective in reducing movements in small numbers of patients (5,6)[C].
IN-PATIENT CONSIDERATIONS
Initial Stabilization
Ensure stable cardiorespiratory status
Admission Criteria
All patients should be admitted for diagnostic evaluation and started on treatment for the ballismus.
IV Fluids
Normal saline should be administered to prevent dehydration.
Nursing
Padding of the limb and bedrails may be necessary to prevent injury.
Discharge Criteria
Discharge criteria and workup depend on the underlying diagnosis.
FOLLOW-UP RECOMMENDATIONS
- Patients should be monitored for medication-induced adverse effects, i.e. drug-induced parkinsonism with neuroleptics, depression with tetrabenazine.
- Patients with stroke as a cause for hemiballismus may require rehabilitation with physical and occupational therapy.
Patient Monitoring
- Patients with stroke should be monitored periodically on an outpatient basis to assess recovery and ongoing treatment of vascular risk factors
- Diabetic patients will need appropriate outpatient blood glucose monitoring
DIET
Varies according to underlying diagnosis
PATIENT EDUCATION
There are no support groups or organizations providing information for patients with hemiballismus. The condition is mentioned briefly at www.wemove.org.
PROGNOSIS
- Spontaneous resolution occurs in majority of cases, usually within 3 months.
- Hemiballismus may evolve into a hemichorea or hemidystonia.
COMPLICATIONS
Severely affected patients may experience medical complications of excessive movement such as dehydration or rhabdomyolysis. Supportive care directed at preventing complications of hospitalization, such as aspiration pneumonia, pulmonary embolism, and urinary tract infection, should be provided.
Stroke is the cause of hemiballismus in a majority of cases.
