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Long-term outcome in children with Guillain-Barré syndrome
Jiri Vajsar1, Darcy Fehlings, Derek Stephens
1Division of Neurology, Department of Pediatrics, Population Health Sciences, Ontario, Canada. jiri.vajsar@sickkids.ca
Insights
Even after treatment with intravenous immunoglobulin (IVIG), 23% of children with Guillain-Barré syndrome (GBS) experience long-term mild muscle weakness. Young age and rapid disease progression are predictors of this weakness.
Area of Science:
- Pediatric Neurology
- Neuromuscular Disorders
- Clinical Immunology
Background:
- Guillain-Barré syndrome (GBS) is an autoimmune disorder affecting the peripheral nervous system.
- Intravenous immunoglobulin (IVIG) is a common treatment for GBS.
- Understanding long-term outcomes and predictors of sequelae in pediatric GBS is crucial for patient management.
Purpose of the Study:
- To evaluate the long-term sequelae in children with GBS after the implementation of IVIG therapy.
- To identify early predictors of these long-term sequelae.
Main Methods:
- A cross-sectional case series design was employed.
- 34 muscle groups were assessed using manual muscle strength testing in children at least 2 years post-recovery.
- Functional independence was evaluated using the Functional Independence Measure (FIM) score.
- Chart review identified acute factors potentially predicting sequelae.
Main Results:
- Out of 47 identified GBS cases, 30 received IVIG.
- 23% of children exhibited persistent long-term muscle weakness (grade 6 strength).
- All patients achieved perfect FIM scores, indicating minimal functional impact.
- Younger age and rapid progression to maximal weakness were significant predictors (P=.03).
Conclusions:
- A significant percentage of children (23%) with GBS show evidence of long-term mild muscle weakness despite IVIG treatment.
- This weakness had minimal impact on overall functional independence.
- Early clinical factors, specifically young age and rapid disease progression, predict long-term outcomes in pediatric GBS.
Objective:
To determine the long-term sequelae and early predictors of sequelae for children with Guillain-Barré syndrome (GBS) after the introduction of intravenous gammaglobulin (IVIG). Study design A cross-sectional case series of children with GBS, at least 2 years after recovery, was performed. Manual muscle strength testing was done on 34 muscle groups. The functional independence measure was used to assess function. A chart review was completed, identifying acute factors that may predict long-term sequelae.
Results:
Forty-seven children with GBS were identified, of whom 30 received IVIG. Persisting long-term muscle weakness with at least one muscle group at a grade 6 level (muscle moves the joint against gravity but only minimal resistance to an applied force) was found in 23% of cases. Each patient had a perfect score on the functional independence measure. Long-term muscle weakness was predicted by young age (P =.03) and a rapid progression to maximal weakness (P =.03).
Conclusions:
Despite the introduction of IVIG, 23% of children with GBS had evidence of long-term mild muscle weakness, with minimal impact on function. Young age and a rapid progression during the acute GBS period predicted long-term sequelae.