What is the initial treatment for Chorea (involuntary, abrupt movements) postpartum?

Initial Treatment for Postpartum Chorea

Dopamine antagonists are the first-line treatment for postpartum chorea, with haloperidol being the most effective initial therapy for controlling involuntary movements while underlying causes are investigated.

Understanding Postpartum Chorea

Chorea is characterized by irregular, flowing, non-stereotyped and random involuntary movements that often possess a writhing quality. When occurring in the postpartum period, it requires prompt evaluation and management due to potential serious underlying causes.

Clinical Presentation

• Involuntary, jerky movements that move randomly from one body part to another
• Movements worsen with stress and anxiety, subside during sleep
• May be asymmetric (hemichorea) or generalized
• Often incorporated into purposeful activity to disguise it
• Associated with motor impersistence (varying grip strength, inability to sustain eye closure)

Diagnostic Approach

Before initiating treatment, a thorough evaluation should be conducted to identify the underlying cause:

1. Brain imaging: MRI of the brain without IV contrast is the optimal initial imaging modality 1

   • CT may be useful to exclude cerebrovascular disease but has limited soft-tissue characterization
   • Focus on evaluating basal ganglia structures (particularly caudate and putamen)

2. Laboratory testing:

   • Antiphospholipid antibodies (chorea has been associated with antiphospholipid antibodies/APS) 1
   • Autoimmune markers (particularly for SLE)
   • Metabolic panel to exclude other causes

Treatment Algorithm

First-line Treatment

1. Dopamine antagonists 1
   • Haloperidol is typically the first choice for symptomatic control
   • Atypical antipsychotics may also be effective with potentially fewer side effects

Second-line/Adjunctive Treatment

Based on underlying etiology:

1. If associated with antiphospholipid antibodies/APS:

   • Add antiplatelet and/or anticoagulation therapy 1
   • Particularly important when other antiphospholipid/APS-related manifestations are present

2. If associated with autoimmune disease (e.g., SLE):

   • Add glucocorticoids in combination with immunosuppressive agents 1
   • Options include azathioprine or cyclophosphamide

3. For refractory cases:

   • Consider VMAT2 inhibitors (tetrabenazine, deutetrabenazine, valbenazine) 2
   • Anti-epileptics or anti-glutamatergic agents may be considered

Prognosis and Monitoring

Most patients (55-65%) experience a single episode of chorea that subsides within days to a few months 1. Regular follow-up is essential to:

1. Monitor response to treatment
2. Adjust medication dosages as needed
3. Evaluate for resolution or progression of symptoms

Important Considerations

• Duration of therapy: Most postpartum chorea is self-limited, so treatment may be temporary
• Side effect monitoring: Watch for extrapyramidal side effects from dopamine antagonists
• Pregnancy planning: Discuss future pregnancy considerations if chorea was associated with antiphospholipid syndrome

Pitfalls to Avoid

1. Delayed diagnosis: Postpartum chorea may be mistaken for anxiety or normal postpartum restlessness
2. Missing underlying causes: Always investigate for antiphospholipid syndrome, SLE, or other autoimmune conditions
3. Inadequate treatment: Insufficient dosing of dopamine antagonists may lead to poor symptom control
4. Overlooking thrombotic risk: Failure to anticoagulate patients with antiphospholipid antibodies could lead to serious complications

Remember that prompt recognition and treatment of postpartum chorea is essential, as it may represent a serious underlying condition requiring specific management beyond symptomatic control.