Abstract Details

Title Use of Clinical Assessment Scale in Autoimmune Encephalitis (CASE) Scores in Autoimmune Encephalitis (AE)

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (11:45 AM-12:45 PM)

Poster/Presentation
Number 8-014

Objective To compare the use of CASE and Modified Rankin score (mRS) scores for description of functional impairment and correlate with treatment responsiveness.

Background Diagnosis of AE is challenging due to a heterogenous clinical spectrum and approach to diagnosis and treatment of seronegative AE is an area of need.

Design/Methods Cases of seropositive and seronegative autoimmune encephalitis, fulfilling Graus et al. (2016) diagnostic criteria, were identified within a 12-month period from two academic medical centers. Retrospective chart review for extraction of pre-defined clinical data was performed. CASE and mRS scores calculated by independent investigators (GM, AA) to permit comparison of inter-rater agreement.

Results 20 cases of AE identified; 6 (30%) seropositive and 14 (70%) seronegative. Mean age 63 years (SD 14.9); 55% Female. 7 cases met criteria for definite limbic AE , 6 for probable seronegative AE, 7 for possible seronegative AE. Most common clinical manifestations were seizures in probable AE (n=5, 45.5%; p=0.04), psychiatric changes in definite limbic AE (n=5, 55.6%; p=0.02). At nadir of initial hospitalization, median CASE scores were 7 (IQR 2), 6.5 (IQR 7) ,12 (IQR=11), median mRS scores were 3 (IQR 1), 3.5 (IQR 2), 4 (IQR 2) for definite limbic, probable and possible AE. 17 patients (85%) received first-line treatment (IV corticosteroids, IVIg) within first two weeks of hospitalization [median 3 days (IQR 9)]. At initial outpatient follow-up median CASE scores were 3.5 (IQR 1), 6 (IQR 5), 2 (IQR 2), and median mRS scores were 3 (IQR 2), 3 (IQR 3), 1 (IQR2). At first outpatient follow up 85.7% (n=12) demonstrated improvement in CASE scores as compared to 50% (n=7) in mRS scores.

Conclusions Longitudinal assessment of CASE scores from nadir of initial presentation may provide a better estimation of functional impairment related to AE, and is incompletely captured by mRS scores for individuals with seropositive and seronegative AE.

Authors/Disclosures
Ahya S. Ali, MD (Westchester Medical Center) PRESENTER	Dr. Ali has nothing to disclose.
Joao Vitor Mahler, MD	Dr. Mahler has received research support from The Sumaira Foundation.
Bruna Leles Vieira de Souza, MD (Work)	Miss Leles Vieira de Souza has nothing to disclose.
Giovanna Manzano, MD	Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gilead Sciences. Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for InfuCare Rx.

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