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Paeds Casesneurology-neurodisability-and-neuromuscular

Paeds Cases · neurology-neurodisability-and-neuromuscular

Autoimmune encephalitis: Case

Clinical case of a 13-year-old girl with anti-NMDA receptor encephalitis, covering the staged presentation from psychiatric change to seizures and movement disorder, the diagnostic workup with paired CSF and serum antibodies, the first-line immunotherapy ladder, the ovarian teratoma search and removal, the escalation to second-line therapy at 10 to 14 days, the management of dysautonomia and hypoventilation in intensive care, and the prolonged cognitive and school reintegration.

paediatric emergency long case3 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A previously well 13-year-old girl is brought to the emergency department after 10 days of agitation, insomnia, and auditory hallucinations. Over the last 48 hours she has had two generalised tonic-clonic seizures, repetitive grimacing and chewing movements, and episodes of shallow slow breathing. Her temperature is 37.4 degrees Celsius, her capillary glucose is 5.0 millimoles per litre, and her weight is 48 kilograms. She is obtunded. MRI brain is normal. CSF shows 45 lymphocytes per microlitre, protein 0.55 g per litre, and oligoclonal bands. Her serum antibody panel is pending.

Summary and immediate impression

This 13-year-old girl presents a textbook case of anti-NMDA receptor encephalitis. The tempo is subacute over days. The psychiatric phase of agitation, insomnia, and auditory hallucinations preceded the neurological phase of seizures, orofacial dyskinesia, and hypoventilation. The CSF is inflammatory with lymphocytic pleocytosis and oligoclonal bands, and the MRI is normal, which is fully compatible with the diagnosis. The antibody result is pending and must not delay treatment. [1]

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References5ShowHide
  1. [1]Graus F, Titulaer MJ, Balu R, et al A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol, 2016.PMID 26906964
  2. [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. [5]Abboud H, Probasco JC, Irani S, et al Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry, 2021.PMID 33649022
  4. [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
  5. [11]Armangue T, Leypoldt F, Málaga I, et al. Herpes simplex virus encephalitis is a trigger of brain autoimmunity. Ann Neurol, 2014.PMID 24318406
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