Autoimmune encephalitis

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Target exams

Red flags

• A child with rapid onset psychiatric change, seizures, and abnormal movements has autoimmune encephalitis until proven otherwise, not a primary psychiatric diagnosis
• Do not wait for antibody results to start immunotherapy once infection is reasonably excluded; early treatment is the strongest predictor of good outcome
• Hypoventilation and autonomic instability in anti-NMDAR encephalitis can kill and mandate intensive care monitoring
• A post-pubertal girl with anti-NMDAR encephalitis has an ovarian teratoma until imaging proves otherwise, and tumour removal is the most effective single intervention
• Clinical relapse weeks after treated herpes simplex encephalitis is usually post-HSE autoimmune encephalitis, not recurrent infection
• Intracellular onconeural antibody syndromes are often poorly responsive to immunotherapy and demand a tumour search and honest prognosis

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises autoimmune encephalitis as a treatable cause of subacute neuropsychiatric decline and states that anti-NMDAR is the commonest subtype in children
• States the first-line and second-line immunotherapy ladder and the need to start steroids or IVIG early while excluding infection
• Identifies the ovarian teratoma association in anti-NMDAR encephalitis and the need for imaging to find and remove it
• Applies the Graus 2016 diagnostic criteria distinguishing possible, probable, and definite autoimmune encephalitis and the specific anti-NMDAR criteria
• Explains the mechanistic distinction between cell-surface antibodies with receptor internalisation and intracellular onconeural antigens with cytotoxic T-cell injury
• Justifies the timing of second-line rituximab or cyclophosphamide at 10 to 14 days for poor first-line response, drawing on Titulaer and the paediatric consensus
• Graus diagnostic tiers and the six symptom groups of probable anti-NMDAR encephalitis
• First-line methylprednisolone, IVIG, and plasma exchange doses and second-line rituximab and cyclophosphamide doses with maxima
• The extreme delta brush EEG pattern, CSF lymphocytic pleocytosis, and the search for an occult tumour
• Runs the workup and first-line immunotherapy of a child with subacute psychiatric change and abnormal movements while treating seizures and dysautonomia
• Decides on and times second-line therapy and tumour removal and coordinates the intensive care and rehabilitation course
• Counsels the family on the cause, the prolonged recovery, the relapse risk, and the cognitive and school reintegration plan
• Level 1: Recognition that subacute neuropsychiatric decline with seizures or movement disorder can be autoimmune and treatable
• Level 2: Application of the Graus diagnostic criteria, the antibody workup, and the first-line immunotherapy ladder
• Level 3: Coordination of second-line therapy, tumour surveillance, intensive care of dysautonomia, and long-term cognitive rehabilitation
• Anti-NMDAR encephalitis as the commonest paediatric autoimmune encephalitis and its prodrome, psychiatric, neurological, and dysautonomic phases
• The Graus 2016 diagnostic tiers and the distinction between cell-surface and intracellular antigen syndromes
• The first-line and second-line immunotherapy ladder and the Titulaer outcome data on early treatment and second-line therapy
• Structured assessment and first-line management of a child with subacute psychiatric change, seizures, and movement disorder
• Recognition of dysautonomia and hypoventilation needing intensive care, and the tumour search in anti-NMDAR disease
• Communication of the diagnosis, the prolonged recovery, and the relapse surveillance and school reintegration to the family
• Autoimmune encephalitis as a treatable mimic of primary psychiatric and infectious encephalitic illness in children
• First-line steroids, IVIG, and plasma exchange and second-line rituximab and cyclophosphamide with doses and timing
• The ovarian teratoma association, the EEG and CSF findings, and the long-term cognitive and seizure outcomes
• Recognition of subacute neuropsychiatric decline with seizures and movement disorder as possible autoimmune encephalitis
• Stepwise immunotherapy with first-line then second-line agents and tumour removal
• Multidisciplinary care of dysautonomia, prolonged recovery, cognitive rehabilitation, and relapse surveillance
• Canadian diagnostic approach to autoimmune encephalitis using the Graus criteria and antibody testing in CSF and serum
• First-line and second-line immunotherapy with the paediatric consensus doses and the timing of second-line therapy
• Coordination of intensive care, rehabilitation, and long-term follow-up including cognitive and psychiatric sequelae