Autoimmune Encephalitis Treatment Guidelines

Diagnostic Workup Prior to Treatment

Evaluate the likelihood of autoimmune encephalitis based on clinical presentation, including behavioral changes, seizures, and autonomic dysfunction, and perform brain MRI and EEG to identify focal or multifocal brain abnormalities, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry 1, 2
Conduct lumbar puncture to confirm inflammatory etiology and exclude infectious causes, and test for neuronal autoantibodies in both cerebrospinal fluid and serum, as suggested by the Journal of Neurology, Neurosurgery and Psychiatry 1, 2
Consider brain FDG-PET when clinical suspicion is high but other studies are uninformative, and perform cancer screening with contrast-enhanced CT of chest, abdomen, and pelvis in relevant cases, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry 1, 2

Start high-dose corticosteroids (intravenous methylprednisolone 1-2 mg/kg/day or pulse dosing at 1g daily for 3-5 days) once infection is ruled out, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry 1, 2, 3
Consider IVIG (0.4 g/kg/day for 5 days) as first-line therapy instead of steroids in patients with contraindications to steroids, agitated or combative patients, or patients with bleeding disorders, as suggested by the Journal of Neurology, Neurosurgery and Psychiatry and Praxis Medical Insights 1, 2, 3, 4
Consider PLEX (5-10 sessions performed every other day) as first-line therapy in patients with severe hyponatremia, high thromboembolic risk, or associated brain or spinal demyelination, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry and Praxis Medical Insights 1, 2, 3, 4

If no clinical, radiological, or electrophysiological improvement is observed after initial treatment with corticosteroids, add IVIG or PLEX, as suggested by the Journal of Neurology, Neurosurgery and Psychiatry and Praxis Medical Insights 1, 2, 4
For severe initial presentations, consider combination therapy with steroids plus IVIG or steroids plus PLEX from the beginning, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry and Praxis Medical Insights 1, 2, 4

Rituximab is the preferred second-line agent for antibody-mediated autoimmune encephalitis, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry and Praxis Medical Insights 1, 2, 3
Consider cyclophosphamide for cell-mediated autoimmunity, as suggested by the Journal of Neurology, Neurosurgery and Psychiatry and Praxis Medical Insights 1, 2, 3

Manage seizures with appropriate antiepileptic medications, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry 1, 2
Monitor and treat blood pressure and heart rate fluctuations in patients with dysautonomia, and consider temporary pacemaker for severe dysrhythmia, as suggested by the Journal of Neurology, Neurosurgery and Psychiatry 2
Implement fluid restriction for hyponatremia related to inappropriate antidiuretic hormone secretion, and monitor intracranial pressure in cases with massive inflammation and brain edema, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry 2

Delaying immunotherapy while waiting for antibody test results can worsen outcomes; treatment should be initiated promptly once infection is ruled out, as recommended by the Journal of Neurology, Neurosurgery and Psychiatry 1, 2
Failure to screen for underlying malignancy may miss paraneoplastic causes of autoimmune encephalitis, as suggested by the Journal of Neurology, Neurosurgery and Psychiatry 1, 2
For immune checkpoint inhibitor-related autoimmune encephalitis, the checkpoint inhibitor should be permanently discontinued, as recommended by Praxis Medical Insights 3