Paeds Vivas · neurology-neurodisability-and-neuromuscular
Autoimmune encephalitis: Viva
Branching clinical structured oral on paediatric autoimmune encephalitis covering the Graus diagnostic criteria, the anti-NMDAR presentation and its age-related variations, the pathophysiology of cell-surface versus intracellular antigen syndromes, the first-line and second-line immunotherapy ladder with doses and timing, the ovarian teratoma association, the post-herpes trigger, and the Titulaer outcome data.
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Branch 1: Making the diagnosis
A strong candidate recognises this as a probable case of anti-NMDA receptor encephalitis and applies the Graus 2016 criteria. The diagnosis turns on the tempo and the pattern rather than on a single test. She has a rapid onset over days of psychiatric symptoms together with at least four of the six major symptom groups of probable anti-NMDAR disease: abnormal behaviour, speech dysfunction, seizures, movement disorder, decreased consciousness, and autonomic dysfunction or hypoventilation. She has an inflammatory CSF with lymphocytic pleocytosis and oligoclonal bands, and her mimics, particularly infectious encephalitis, are being excluded. A normal MRI is fully compatible with the diagnosis. The candidate sends a paired CSF and serum neural antibody panel, with CSF as the more sensitive sample, but does not wait for the result to treat. [1]
References5ShowHide
- [1]Graus F, Titulaer MJ, Balu R, et al A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol, 2016.PMID 26906964
- [2]Titulaer MJ, McCracken L, Gabilondo I, et al Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol, 2013.PMID 23290630
- [3]Dalmau J, Gleichman AJ, Hughes EG, et al Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol, 2008.PMID 18851928
- [7]Nosadini M, Thomas T, Eyre M, et al. International Consensus Recommendations for the Treatment of Pediatric NMDAR Antibody Encephalitis. Neurol Neuroimmunol Neuroinflamm, 2021.PMID 34301820
- [11]Armangue T, Leypoldt F, Málaga I, et al. Herpes simplex virus encephalitis is a trigger of brain autoimmunity. Ann Neurol, 2014.PMID 24318406