Treatment of Hyperglycemic Hemichorea
Immediate Priority: Aggressive Glycemic Control
The cornerstone of treatment for hyperglycemic hemichorea is aggressive normalization of blood glucose, which alone resolves symptoms in the majority of cases. 1, 2, 3, 4
Initial Insulin Therapy
- Start basal-bolus insulin immediately for patients presenting with severe hyperglycemia (typically glucose >300-500 mg/dL and HbA1c >10-15%) 5, 6
- Begin with 0.3-0.5 units/kg/day total daily insulin dose, divided as 50% basal insulin (glargine or detemir) and 50% prandial insulin (rapid-acting analog) 5, 7
- Titrate basal insulin aggressively by 4 units every 3 days until fasting glucose reaches 80-130 mg/dL 6, 7
- Target fasting glucose of 80-130 mg/dL and avoid hypoglycemia, as hypoglycemic episodes can paradoxically trigger or worsen hemichorea 8
Glycemic Monitoring
- Check fasting blood glucose daily during titration and monitor for hypoglycemia vigilantly 6
- Measure HbA1c every 3 months during intensive management to assess long-term control 6
- Avoid both severe hyperglycemia AND hypoglycemia, as the case literature demonstrates hemichorea can occur with either extreme 8, 4
Symptomatic Treatment When Glucose Control Alone Is Insufficient
While most patients improve with glycemic normalization alone, some require adjunctive pharmacotherapy for persistent choreiform movements 1, 2, 3.
First-Line Symptomatic Agents
- Dopamine receptor antagonists (such as risperidone or haloperidol) are the primary symptomatic treatment when movements persist despite glucose normalization 1, 8, 2
- Start risperidone at low doses (0.5-1 mg daily) and titrate based on symptom response 2
- Benzodiazepines (such as clonazepam or diazepam) can be used as adjunctive therapy, though they are less effective than dopamine antagonists 1, 2
Duration of Symptomatic Treatment
- Continue dopamine receptor antagonists until choreiform movements completely resolve, which may take weeks to months 8, 2
- Do not discontinue dopamine antagonists prematurely, as the case literature shows relapse can occur even after initial symptom resolution 8
- Attempt gradual taper only after sustained glycemic control (HbA1c <8%) and complete symptom resolution for at least 1-2 months 8
Foundation Therapy: Metformin Optimization
- Continue or initiate metformin at maximum tolerated dose (up to 2000-2500 mg daily) unless contraindicated, as this reduces insulin requirements and provides complementary glucose-lowering effects 9, 6
- Metformin should be maintained even when intensifying insulin therapy 9, 6
Expected Clinical Course and Prognosis
- Choreiform movements typically improve within days to weeks of achieving glycemic control, with complete resolution in most cases 1, 2, 4
- The patient in one case report showed complete disappearance of chorea by the fourth day of insulin therapy with glucose normalization 4
- MRI findings (T1 hyperintensity in basal ganglia) may persist for months after clinical resolution 1, 3
Critical Diagnostic Considerations
- Confirm non-ketotic hyperglycemia by checking serum or urine ketones (should be negative or minimally elevated) 1, 3
- Obtain brain MRI to identify characteristic T1 hyperintensity in the contralateral basal ganglia (typically putamen and caudate), though absence of MRI findings does not exclude the diagnosis 8, 3
- Consider CT scan if MRI unavailable, which may show hyperdensity in basal ganglia 4
Common Pitfalls to Avoid
- Never delay insulin initiation while attempting oral agent optimization in patients with severe hyperglycemia and neurological symptoms 5, 6
- Do not use sliding scale insulin as monotherapy—implement scheduled basal-bolus regimen immediately 6
- Avoid overly aggressive glucose correction that causes hypoglycemia, as this can trigger or worsen hemichorea 8
- Do not discontinue dopamine antagonists prematurely if used, as relapse can occur even with good glycemic control 8
- Do not assume MRI abnormalities are required for diagnosis—hemichorea can occur without basal ganglia T1 hyperintensity 8