Autoimmune Encephalitis

AUTOIMMUNE ENCEPHALITIS -- CLINICAL REFERENCE A comprehensive, evidence-based clinical reference app for the diagnosis and management of autoimmune encephalitis (AIE), developed by Dr Atif Elnil MBBS MRCP, Neurology St George's Hospital, London UK. Based on Graus et al. (Lancet Neurology 2016) diagnostic criteria, Abboud et al. (JNNP 2021) best practice recommendations, and Titulaer et al. (Lancet Neurology 2013) treatment evidence. DESIGNED FOR: Neurologists, stroke physicians, psychiatrists, intensivists, emergency physicians, and specialist trainees managing patients with suspected or confirmed autoimmune encephalitis. KEY SECTIONS: DIAGNOSTIC APPROACH -- Graus 2016 possible and probable AIE criteria -- Interactive CASE (Clinical Assessment Scale for Autoimmune Encephalitis) calculator with tap-to-score interface -- Red flag recognition: faciobrachial dystonic seizures, orofacial dyskinesias, FBDS, NORSE and FIRES -- Step-by-step diagnostic pathway ANTIBODY SYNDROMES (7 tap-to-expand cards) -- Anti-NMDAR: clinical stages, orofacial dyskinesias, autonomic instability, teratoma association -- Anti-LGI1: faciobrachial dystonic seizures, hyponatraemia, SIADH, thymoma -- Anti-CASPR2: Morvan syndrome, neuromyotonia, neuropathic pain -- Anti-AMPAR: high relapse rate, tumour associations, long-term immunosuppression -- Anti-GABA-B: refractory seizures, SCLC association -- Anti-DPPX: diarrhoea prodrome, agitation, hyperekplexia -- Onconeural panel: Hu, Yo, Ri, Ma2, CV2, amphiphysin with full clinical profiles IMMUNOTHERAPY PROTOCOLS (5 tabs) -- First line: IV methylprednisolone 1g x5 days plus IVIG 2g/kg with dosing details -- Second line: Rituximab (375mg/m2 weekly x4 or 1g x2) and cyclophosphamide protocols -- Third line: Bortezomib, daratumumab, tocilizumab for refractory disease -- Maintenance: Mycophenolate, azathioprine, rituximab maintenance schedules -- Symptomatic: Seizure management, psychiatric symptoms, ICU management INVESTIGATIONS -- Urgent blood panel with autoimmune and onconeural antibody testing -- LP protocol with HSV empirical treatment reminder -- MRI sequences and pattern recognition -- EEG indications and continuous monitoring guidance TUMOUR SCREENING -- Antibody-specific tumour associations (NMDAR/teratoma, GABA-B/SCLC, Hu/SCLC, Yo/ovary) -- Step-by-step screening protocol -- FDG-PET/CT guidance for occult tumours -- Teratoma resection urgency guidance in NMDAR EEG PATTERNS -- Extreme delta brush: pathognomonic NMDAR pattern with full description -- Focal temporal slowing in limbic encephalitis -- FBDS ictal correlates in LGI1 -- Differential EEG patterns for CJD, metabolic encephalopathy DIFFERENTIAL DIAGNOSIS -- HSV encephalitis: first exclusion with aciclovir reminder -- Infectious mimics: HHV-6, VZV, TB, neurosyphilis -- Metabolic: Wernicke, hepatic, uraemic encephalopathy -- Prion disease: CJD differentiation -- Structural: CNS vasculitis, lymphoma, CVST, anti-MOG MONITORING AND RELAPSE -- Relapse rates by antibody (AMPAR 70%+, NMDAR 12-25%, LGI1 15-35%) -- Follow-up schedule: 1 month, 3 months, 6 months, annual -- Prognostic factors for good and poor outcome -- DVLA notification guidance REFERENCES (20+ citations) All content derived from: -- Graus F et al. Lancet Neurology 2016 -- Abboud H et al. JNNP 2021 -- Titulaer MJ et al. Lancet Neurology 2013 -- Dalmau J et al. Lancet Neurology 2008 -- Irani SR et al. Annals of Neurology 2011 -- Schmitt SE et al. Annals of Neurology 2012 -- And 15+ additional peer-reviewed publications FOR HEALTHCARE PROFESSIONALS ONLY This app is an educational reference tool for qualified healthcare professionals. It does not replace clinical assessment or specialist review. Autoimmune encephalitis management should always involve a neurologist with specialist expertise.