109 Severe dysautonomia in NMDAR encephalitis

Abstract

IntroductionAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the more common forms of autoimmune encephalitis, predominantly affecting children and women of the child-bearing age. It is characterised by memory deficit, behavioural disturbance and seizures. Dysautonomia is recognised as a feature but rarely the first symptom of the condition.1 Here we present a case of severe dysautonomia preceding the diagnosis of NMDAR encephalitis.MethodsWe conducted a retrospective review of the admissions to the neurology ward at the Calvary Hospital, Canberra in 2018 to identify patients diagnosed with NMDAR encephalitis.ResultsOne patient was identified from the registry data. A 37-year-old woman presented with a week-long history of symptomatic orthostatic hypotension. Her supine systolic blood pressure was 110mmHg with a 46mmHg postural drop. Over the first week of hospitalisation, she became increasingly disoriented and erratic in behaviour with fluctuating levels of consciousness requiring intensive unit care. Her CSF demonstrated lymphocytic pleocytosis and NMDAR antibodies were detected in both CSF and serum. She was treated with IVIG, IV steroids and subsequently Rituximab. A pelvic teratoma was found and removed. Her symptomatology including dysautonomia improved substantially by the end of her six-week hospital admission. Her modified Rankin Scale was zero at three months.ConclusionAutonomic dysfunction is not a common feature of autoimmune encephalitides. Our case highlighted the possibility that dysautonomia can be the initiating symptom of this disease entity. Physician awareness is important in the early recognition and treatment of this condition.ReferenceTitulaer MJ, McCracken L, Gabilondo I, Armangue T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013;12(2):157–165.

Persistent Central Hypoventilation Following Probable Remission from Anti-N-methyl-D-aspartate Receptor Encephalitis

“…Formes frustes of anti-NMDAR encephalitis have been described, but they usually consist of predominant psychiatric symptoms, seizures, or abnormal movements. 3 Cases of severe dysautonomia preceding or prevailing over neuropsychiatric disturbances in anti-NMDAR encephalitis were described, 5 but syndromes with prominent central hypoventilation were only reported in anti-Hu brainstem encephalitis. 6 In hindsight, our patient's presumed viral meningoencephalitis in 2019 was most likely a paucisymptomatic initial presentation of anti-NMDAR encephalitis.…”