Highlights
- •Nearly 1/4 of children with moderate to severe ADEM had poor functional recoveries.
- •Prodromal headache was an indicator for good outcomes.
- •Dystonia, myoclonus, and cerebellar lesions on MRI suggested poor recoveries.
- •Immunotherapies may be adjusted in children prone to poor functional recoveries.
Abstract
Background
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated encephalopathy with
heterogeneous disease courses. However, clinical characteristics for a prognostication
of functional recovery from acute episodes of ADEM remain limited. The study aims
to characterize the clinical presentations and neuroimaging findings of children with
poor functional recoveries from acute episodes of moderate to severe ADEM.
Methods
The multicenter retrospective cohort study included children under 18 years of age
who presented with moderate to severe ADEM (modified Rankin Scale [mRS] ≥ 3 at nadir)
from 2002 to 2019. Children were assigned to a good recovery group (mRS ≤ 2) and a
poor recovery group (mRS ≥ 3) after mean 4.3 months of follow-up. The clinical presentations
and the distribution of brain lesions on magnetic resonance imaging were compared
between the two groups by the t-test for numerical variables and Fisher's exact test
for categorical variables. Analyses of logistic regression were conducted and significant
variables in the multivariate model were examined by the receiver operating characteristic
curve for the prediction of functional recovery.
Results
Among the 73 children with moderate to severe ADEM, 56 (77%) had good functional recoveries
and 17 (23%) showed poor functional recoveries. Children with poor recoveries had
a lower rate of prodromal headache (12% vs. 39%, p = 0.04), and presented with higher proportions of dystonia (29% vs. 9%, p = 0.046), myoclonus (24% vs. 2%, p = 0.009), and cerebellar lesions on neuroimages (59% vs. 23%, p = 0.01). The multivariate analyses identified that a lack of prodromal headache (OR
0.1, 95% CI 0.005 - 0.7, p = 0.06) and the presentations of myoclonus (OR 21.6, 95% CI 1.7 – 874, p = 0.04) and cerebellar lesions (OR 4.8, 95% CI 1.3 - 19.9, p = 0.02) were associated with poor functional recoveries. These three factors could
prognosticate poor outcomes in children with moderate to severe ADEM (area under the
receiver operating characteristic curve 0.80, 95% CI 0.68 – 0.93, p = 0.0002).
Conclusion
Nearly one-fourth of children with moderate to severe ADEM had a poor functional recovery
from acute episodes, who were characterized by a lack of prodromal headache, the presentation
of myoclonus, and the neuroimaging finding of cerebellar lesions. The clinical variables
associated with poor functional recoveries could assist in the planning of immunotherapies
during hospitalization for a better outcome in moderate to severe ADEM.
Keywords
Abbreviations:
ADEM (acute demyelinating encephalomyelitis), MOG (myelin oligodendrocyte glycoprotein), mRS (modified rankin scale), NMDAR (N-methyl-D-aspartate receptor)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: July 12, 2022
Accepted:
July 11,
2022
Received in revised form:
June 25,
2022
Received:
January 27,
2022
Identification
Copyright
© 2022 Elsevier B.V. All rights reserved.
