Clinical presentation and treatment of autoimmune encephalitis: Single center cohort study of 47 cases

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Conference Proceeding

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Objective: In this retrospective cohort study, we aim to identify the distinctive clinical, laboratory and radiological presentation of autoimmune encephalitis and the response to treatment.

Background: Autoimmune encephalitis are severe inflammatory disorders of central nervous system and diagnosis in clinical practice remains challenging despite recent advances in identification of new syndromes and autoantibodies. The diagnosis relies on the clinical manifestations, finding biomarkers and therapy response. However, this can be problematic given that antibody testing results are not readily available and negative testing does not exclude the diagnosis.

Design/Methods: The study setting was a southeastern Michigan large health system. Electronic Medical Record was queried for diagnosis of autoimmune/paraneoplastic conditions using ICD 10 diagnostic code. Neurological symptoms, sociodemographic, laboratory, radiographic, treatment and outcomes data using modified Rankin scale (mRS) were collected. Two sample t-tests and Fisher's exact tests were used. SAS version 9.4 was used for data analyses.

Results: Of 47, 64% were female, 51% were white. The mean age at diagnosis was 56.9 years. The most common presentation was cognitive impairment (34%), followed by psychosis (30%). 69% had antibody to cell surface and 33% had normal MRI. 68% of 41 patients with mRS data had a mRS ≤ 2 and 32% had a mRS >2. Patients with mRS ≤ 2 were younger (p=0.046), less likely to have abnormal protein (p=0.031) or elevated IgG index (p=0.09), but higher rate of elevated WBC in CSF (p=0.097). PLEX treatment was less likely to be required for patients with mRS ≤ 2 (p=0.069). Steroid treatment was used in 62%, PLEX 28%, IVIG 19%. 23% remained on long term immunosuppression.

Conclusions: Autoimmune encephalitis is increasingly recognized as a heterogeneous group presenting with acute/subacute symptoms, but a diagnosis test is still yet to be found. Early recognition is important for this potentially treatable disorders as it may improve the prognosis.




15 Suppl 1

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