2024 American Academy of Neurology Abstract Website

Objective:

To investigate the relationship between clinician-reported outcomes (ClinROs) and patient-reported outcomes (PROs) in patients with LGI1-IgG autoimmune encephalitis (LGI1-IgG-AE).

Background:

In LGI1-IgG-AE research, outcomes include disease severity, measured by Clinical Assessment Scale in Autoimmune Encephalitis [CASE], and disability, measured by modified Rankin score (mRS). PROs may have a role in capturing additional longitudinal symptomatology.

Design/Methods:

mRS, CASE, and PROs (Neuro-QoL) were collected longitudinally from a retrospective LGI1-IgG-AE cohort. At last follow-up, proportion with Neuro-QoL abnormal scores (≥ 5 T-score change from median 50) were calculated, and ClinROs and PROs were correlated. Mixed-effects linear modeling evaluated age, sex, disease duration, and acute encephalitis severity and disability (CASE and mRS scores <1 month post-diagnosis) as predictors of longitudinal PROs.

Results:

Thirty-five patients were included (63% male, mean age=67.8 years [SD=11.6]). Most (89%) received immunosuppressive therapies. Disability (mRS mean=2.5, SD=1.1) and disease severity (CASE mean=5.1, SD=4.4) improved at last follow-up (mean=1.86, SD=1.17; mean=3.69, SD=3.07, respectively). Of patients with Neuro-QoL (n=14, obtained median 26 months [IQR=22-64] post-diagnosis), 71% had cognitive dysfunction and 50% reported impaired sleep, fatigue, anxiety, and/or depression.

At last follow-up, CASE and mRS correlated with Neuro-QoL domains of upper and lower extremity function, cognitive function, social roles participation and satisfaction (for all, p<.01).

Acute CASE and mRS predicted long-term patient-reported cognitive function (p<.05); additionally, acute CASE predicted anxiety (p=.026), and acute mRS predicted stigma (p=.018) at last follow-up.

Conclusions:

In LGI1-IgG-AE with median >2 years follow up, we demonstrate 1) symptoms of cognitive dysfunction, sleep, fatigue, anxiety, and/or depression are present in most screened patients; 2) at last follow-up, CASE and mRS correlated strongly with PROs of physical function, cognitive dysfunction, and social roles, but not these aforementioned symptoms, highlighting PROs as valuable additional outcome measures; 3) PROs regarding cognition, anxiety, and stigma are predicted by greater disease severity and disability at diagnosis.