The following is a summary of “Development of a Composite Score for the Clinical Assessment of Anti-IgLON5 Disease,” published in the March 2024 issue of Neurology by Gaig, et al.

Researchers conducted a retrospective study to design a scoring system for evaluating the combined impact of various symptoms in anti-IgLON5 disease.

They developed the anti-IgLON5 disease composite score (ICS) to assess 17 symptoms categorized into 5 clinical domains (bulbar, sleep, movement disorders, cognition, and others). Symptoms were scored from 0 to 3 or 6, based on their impact on disability, with a maximum ICS of 69. The ICS was tested on patients from two cohorts (Barcelona, Spain, and GENERATE, Germany), including both internally examined cases and those with data collected from external neurologist questionnaires. Test-retest and interrater reliabilities of the ICS were evaluated using the intraclass coefficient (ICC) and the correlation between the ICS and modified Rankin scale (mRS) via the nonparametric Spearman rank coefficient. The Wilcoxon signed rank test compared ICS scores at anti-IgLON5 disease diagnosis and follow-up inaccessible patients.

The results showed 86 patients (46 from the Barcelona cohort and 40 from the GENERATE cohort) were enrolled. The median ICS stood at 15 (range 2–31). Compared to the German cohort, the Barcelona cohort exhibited a higher ICS (18 vs. 12, P<0.001), attributed to elevated partial scores in the sleep and movement disorder domains. No notable disparities in ICS were observed between internal and external patients (15 vs. 14, P=0.96). The ICS correlated with the mRS score (r = 0.429, P<0.001). Test-retest and interrater reliabilities were excellent, with an ICC of 0.997 (95% CI 0.992–0.999) and 0.973 (95% CI 0.925–0.990), respectively. During follow-up, the ICS was retested in 27 patients, revealing similarity to the diagnosis in 10 clinically stable patients (median ICS at diagnosis 11.5 vs. 11.5 at follow-up; P=1), elevation in 8 patients who worsened (12.5 vs. 18; P=0.012), and reduction in 9 patients who improved after immunotherapy (14 vs. 10; P=0.007).

Investigators concluded that the ICS effectively measured the breadth and intensity of diverse clinical features in anti-IgLON5 disease.

Source: neurology.org/doi/10.1212/WNL.0000000000208101