What is the management of Bickerstaff encephalitis?

Management of Bickerstaff Brainstem Encephalitis

Bickerstaff brainstem encephalitis should be treated with immunotherapy—specifically intravenous immunoglobulin (IVIg), high-dose corticosteroids, or plasma exchange—as patients receiving any form of immunotherapy demonstrate faster symptom resolution compared to supportive care alone. 1

Initial Assessment and Stabilization

• Obtain urgent neurological specialist assessment within 24 hours of presentation, as patients with encephalitis require specialized neurological care to optimize morbidity and mortality outcomes 2

• Assess for airway protection and ventilatory support needs immediately in patients with declining consciousness, as BBE can cause rapid deterioration requiring ICU-level care for management of raised intracranial pressure and cerebral perfusion optimization 2

• Monitor closely for fulminant progression, as rare cases—particularly in pediatric patients—can deteriorate to brain death within days despite optimal management 3

Diagnostic Workup

• Obtain MRI of the brain within 48 hours (preferred over CT), as neuroimaging is essential for diagnosis and patients with neuroimaging changes require longer recovery time 2, 1

• Perform lumbar puncture for CSF analysis with results ideally available within 24-48 hours, as this is critical for confirming diagnosis 2

• Test for anti-GQ1b antibodies, though nearly half of pediatric cases are positive and antibody presence does not correlate with healing time 1

• Consider EEG when distinguishing organic versus psychiatric causes or when subtle seizures are suspected, as EEG is abnormal in >80% of encephalitis cases 2

Immunotherapy Treatment Options

First-line immunotherapy should be initiated promptly, as precocity of treatment directly impacts prognosis 1:

• Intravenous immunoglobulin (IVIg) represents an effective alternative treatment option, with documented rapid clinical recovery in BBE patients 4

• High-dose intravenous methylprednisolone (1 g IV daily for at least 3-5 days) is recommended for immune-mediated encephalitis, including BBE 5, 6

• Plasma exchange should be considered in patients who respond poorly to corticosteroids, though IVIg may be preferred when plasma exchange is difficult or contraindicated 5, 4

Treatment Algorithm

The choice between immunotherapies can follow this approach:

1. Initiate IVIg or high-dose corticosteroids as first-line therapy immediately upon diagnosis 1, 4, 6

2. Add plasma exchange if inadequate response to initial immunotherapy within 3-5 days 5

3. Continue supportive care throughout, including management of complications such as dysarthria, dysphagia, and sensory disturbances 6

Special Considerations and Pitfalls

• Do not delay treatment waiting for anti-GQ1b antibody results, as antibody negativity does not exclude the diagnosis and treatment efficacy is time-dependent 1

• Be vigilant for transient episodes of brainstem dysfunction during recovery, including reflex myoclonus affecting face, neck, and limbs, which may originate from brainstem lesions and typically resolve as BBE improves 6, 7

• Recognize that elderly patients may experience frequent transient brainstem dysfunction episodes during recovery, possibly due to ephaptic transmission, but can still achieve full recovery with aggressive treatment 7

• Monitor for recurrence, particularly in pediatric patients, as second episodes can occur with potentially fulminant courses 3

Discharge and Follow-up Planning

• Arrange comprehensive rehabilitation assessment before discharge, as sequelae may not be immediately apparent and patients commonly experience ongoing complications including cognitive deficits 2

• Ensure definite or suspected diagnosis is established and formulate clear plans for outpatient follow-up, ongoing therapy, and rehabilitation at a discharge meeting 2