Table 1

Symptomatic management for autoimmune encephalitis

Symptom categoryTherapeutic optionsPrecautions
Psychosis/agitation/mania
  1. Acute immunotherapy with IVMP, IVIg and/or PLEX.

  2. Benzodiazepines (eg, clonazepam, diazepam).

  3. Antipsychotics (eg, quetiapine).

  4. Mood stabilisers (eg, valproic acid).

  5. Establish safety measures as necessary (eg, bed padding, soft restraints, room sitter).

  1. Avoid over-sedation and unnecessary polypharmacy.

  2. Avoid medications that lower seizure threshold in patients with high seizure risk (eg, clozapine, olanzapine).

  3. Avoid medications that prolong QT interval in dysautonomic patients (eg, ziprasidone, haloperidol).

  4. Watch out for worsening of involuntary movements or development of neuroleptic malignant syndrome.

Seizures
  1. Acute immunotherapy with IVMP, IVIg and/or PLEX.

  2. Antiseizure medications (sodium channel blockers like carbamazepine or lacosamide may be preferred in LGI1-antibody encephalitis).

  3. Medically induced coma with midazolam, pentobarbital or propofol is required for NORSE.

  1. Instate early immunotherapy for patients with seizures in the setting of suspected AE. Avoid use of anti-seizure medications alone.

  2. May cautiously attempt weaning antiseizure medications in patients with early seizure freedom and normal brain MRI and EEG.

Movement disorders
  1. Acute immunotherapy with IVMP, IVIg and/or PLEX.

  2. Benzodiazepines (eg, clonazepam, diazepam) for myoclonus, SPS, PERM, catatonia, dystonia, stereotypies and hyperkinesia.

  3. Anticholinergics (eg, trihexyphenidyl, benzatropine) for dystonia.

  4. Muscle relaxants (eg, baclofen, tizanidine) for dystonia and spasticity.

  5. Dopamine blockers (eg, risperidone) or depleters (tetrabenazine) for chorea, athetosis, balism, tics and hyperkinesia.

  6. Dopamine agonists (eg, pramipexole, ropinirole) or carbidopa/levodopa for acquired parkinsonism, rigidity and akinetic mutism.

  1. Avoid over-sedation and unnecessary polypharmacy.

  2. Watch for paradoxical worsening of involuntary movements or development of neuroleptic malignant syndrome.

  3. Practice caution with anticholinergics in patients with dysautonomia.

  4. Practice caution with anticholinergics and dopaminergic medications in patients with psychosis.

Dysautonomia
  1. Acute immunotherapy with IVMP, IVIg and/or PLEX.

  2. ICU monitoring for severe dysautonomia.

  3. Beta-blockers (eg, propranolol), alpha-2 blockers (eg, clonidine), and/or acetylcholine esterase inhibitors (pyridostigmine) for increased sympathetic drive.

  4. Midodrine, fludrocortisone or droxidopa for symptomatic postural hypotension.

  5. Temporary pacing for heart block or severe arrhythmia.

  6. Total parental nutrition for patients with severe gastrointestinal dysmotility.

  7. Anti-muscarinics (eg, oxybutynin) for bladder incontinence.

  1. Watch for exaggerated response to sympatholytic therapies.

  2. Watch for supine hypertension when treating postural hypotension.

  3. Watch for cognitive and cardiac side effects when using antimuscarinics.

Sleep disorders
  1. Acute immunotherapy with IVMP, IVIg and/or PLEX.

  2. Promote sleep hygiene and uninterrupted night-time sleep.

  3. Melatonin to promote the sleep-wake cycle.

  4. Sedating benzodiazepines (eg, temazepam), benzodiazepine receptor agonists (eg, zolpidem) and/or non-benzodiazepine hypnotics (eg, zopiclone) for insomnia.

  5. Wake-promoting agents (eg, modafinil) and/or traditional stimulants (eg, methylphenidate) for excessive daytime sleepiness.

  6. Evaluate residual sleep disorders with polysomnography and treat sleep disordered breathing if present.

  1. Avoid over-sedation and unnecessary polypharmacy.

  2. Practice caution when using stimulants in patients with seizures or hyperkinetic involuntary movements.

  • AE, autoimmune encephalitis; EEG, electroencephalogram; ICU, intensive care unit; IVIg, intravenous immunoglobulins; IVMP, intravenous methyl-prednisolone; LGI1, leucine-rich glioma inactivated-1; NORSE, new onset refractory status-epilepticus; PERM, progressive encephalomyelitis with rigidity and myoclonus; PLEX, plasma exchange; SPS, stiff person syndrome.