What is the best approach to manage a patient presenting with hemiballismus?

Management Approach to Hemiballismus

Hemiballismus requires immediate identification and treatment of the underlying cause—most commonly stroke or nonketotic hyperglycemia—followed by symptomatic control with dopamine receptor blockers, with the reassuring knowledge that most cases resolve spontaneously within weeks to months. 1, 2

Initial Diagnostic Evaluation

Identify the Underlying Cause

• Check blood glucose immediately at presentation, as nonketotic hyperglycemia is now the second most common cause of hemiballismus after stroke, particularly in elderly diabetic patients 2, 3
• Obtain urgent brain imaging (MRI preferred, CT if unavailable) to identify vascular lesions (most commonly involving basal ganglia structures, not just the subthalamic nucleus), hemorrhage, or structural abnormalities 1, 2
• Screen for HIV infection in appropriate patient populations, as HIV-related complications are an increasingly recognized cause 1
• Consider additional causes including encephalitis, cerebral toxoplasmosis (especially in AIDS patients), multiple sclerosis, brain tumors (primary or metastatic), and other metabolic disturbances 2

Key Clinical Features to Document

• Laterality and amplitude of involuntary movements—hemiballismus characteristically involves violent, coarse, wide-amplitude flinging movements of the ipsilateral arm and leg 1, 4
• Associated neurological deficits including hemiparesis, sensory loss, diplopia, or cranial nerve abnormalities that may indicate brainstem or other CNS involvement 5, 6
• Timing and progression—acute onset suggests vascular etiology, while subacute progression may indicate tumor, infection, or metabolic causes 1, 2

Treatment Algorithm

Address the Underlying Cause First

• For hyperglycemia: Correct blood glucose levels promptly, as this alone may resolve the movement disorder 2, 3
• For stroke: Implement standard acute stroke protocols and secondary prevention measures 1
• For infectious/inflammatory causes: Initiate appropriate antimicrobial or immunomodulatory therapy 2

Symptomatic Pharmacologic Management

First-line therapy:

• Dopamine D2 receptor blockers are highly effective for symptomatic control 1, 4, 2
• In elderly patients, start with sulpiride due to its favorable side effect profile compared to haloperidol or chlorpromazine 4
• Haloperidol or chlorpromazine are well-established alternatives with proven efficacy 1, 4

Alternative agents:

• Olanzapine has demonstrated significant efficacy when traditional neuroleptics fail, with documented reduction in movement frequency from baseline 7
• Tetrabenazine is an effective option for patients requiring symptomatic treatment 1

Surgical Intervention

• Reserve pallidotomy for the minority of patients with severe, persistent, disabling hemiballismus that fails medical management 1
• Surgical treatment targets the internal segment of the globus pallidus, where abnormal neuronal firing patterns are implicated in the pathogenesis 1

Prognosis and Natural History

Expected Clinical Course

• Most cases have a favorable prognosis with spontaneous improvement or complete resolution, contrary to older literature describing high mortality 1, 2
• Vascular subthalamic lesions typically resolve within days to weeks, though some historical reports documented death from exhaustion within 4-6 weeks in untreated severe cases 4
• Lesions outside the subthalamic nucleus tend to persist longer and require more intensive treatment 4

Monitoring and Follow-up

• Reassess frequently during the acute phase to monitor for spontaneous improvement and medication response 1, 2
• Adjust pharmacologic therapy based on symptom control and side effect profile, particularly in elderly patients 4
• Consider neurology referral for persistent symptoms beyond several weeks or when surgical intervention may be warranted 1

Critical Pitfalls to Avoid

• Do not assume subthalamic nucleus involvement—most patients actually have lesions in other basal ganglia structures 1
• Do not overlook hyperglycemia—always check glucose levels, as this treatable cause is increasingly common and may present with hemiballismus as the sole manifestation 2, 3
• Do not use excessive doses of neuroleptics in elderly patients—start with sulpiride or lower doses of other agents to minimize side effects 4
• Do not rush to surgical intervention—given the generally favorable natural history, reserve surgery only for severe, persistent cases refractory to medical management 1