Severe dysautonomia in NMDAR encephalitis [Meeting Abstract]

Abstract

Introduction Anti-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.

Methods We 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.

Results One 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.

Conclusion Autonomic 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.