STOCKHOLM — Older age, brainstem syndrome, and polyfocal-specific magnetic resonance imaging (MRI) presentation at onset are predictive of relapse risk in children with demyelinating diseases (DD) of the central nervous system (CNS), study results presented at the 35th Congress of the European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS), held September 11-13, 2019 in Stockholm, Sweden, suggests.

Researchers developed an observational cohort between 1999 and 2018 that consisted of 75 children (46 girls, 29 boys; median age 12 years) with a first demyelinating event (FDE). All patients had the first focal neurologic dysfunction event with a probable demyelinating cause. Using these patient data, investigators examined neurologic status, MRI findings, and second relapse risk. Predictors of relapse risk were examined in the final analysis.

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Mean patient follow-up was 5.2±4 years (range 0.5 to 17 years). In the overall cohort, initial symptoms included optic neuritis (n=36), transverse myelitis (n=15), brainstem syndrome (n=14), cerebellar disorder (n=13), motor dysfunction (n=13), and sensor dysfunction (n=4).

After 1 to 60 months (median 8.5 months), approximately 56% of patients (n=42) had a second attack. A median of 3 relapses were identified during follow-up. At final diagnosis, patients had multiple sclerosis (n=32), relapsing optic neuritis (n=7), polyphasic acute disseminated encephalomyelitis (n=2), and neuromyelitis optica (n=1).

Factors associated with relapse outcome included older age (≥11 years) at the initial event (risk ratio [RR], 2.61; 95% Cl, 1.42-4.79), clinical presentation with brainstem syndrome (RR, 10.21; 95% Cl, 1.41-74.14), and partial transverse myelitis (RR=3,43; 95% Cl, 1,04-11,82).

Predictors of relapse risk based on MRI findings included >5 lesions (RR, 3.18; 95% CI, 1.18-8.58), periventricular localization of lesions (RR, 2.55; 95% CI, 1.3-4.97), subcortical localization of lesions (RR, 1.97; 95% CI, 1.29-2.9), and brainstem localization of lesions (RR, 1.9; 95% CI, 1.17-2.95), well-defined lesions (RR, 1.76; 95% CI, 1.05-3.16), Dawson fingers sign (RR, 1.85; 95% CI, 1.38-2.49), and lateralized spinal lesions (RR, 35; 95% CI, 1.02-11.96).

Study limitations include its observational nature, as well as the relatively small sample size.

The researchers concluded that these findings show a 65% risk for relapse in children with FDE. They add that “age, brainstem, partial spinal signs and polyfocal specific MRI presentation at onset may determine the long-term prognosis of relapse outcome.”

Reference

Nevmerzhitskaya K, Volkova L, Sergeev A. Clinical predictors of relapse in children with the first demyelinating event. Abstract presentation at: 35th Congress of the European Committee for Treatment and Research in Multiple Sclerosis; September 11-13, 2019; Stockholm, Sweden. Abstract P1111.