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Acquired demyelinating disorders of central nervous system: A pediatric cohort
Sheffali Gulati1, Biswaroop Chakrabarty1, Atin Kumar2
1Department of Pediatrics, Jai Prakash Narayan Apex Trauma Centre, All India Institute of Medical Sciences, New Delhi, India.
Insights
This study reviewed 35 children with acquired demyelinating disorders, finding that while steroids help acute episodes, recurrent conditions like multiple sclerosis and neuromyelitis optica require long-term immunomodulation for effective management.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Disorders
Background:
- Acquired demyelinating disorders (ADDs) in children present diagnostic and management challenges.
- Understanding recurrence patterns is crucial for long-term pediatric neurological care.
Purpose of the Study:
- To describe the clinical characteristics, management, and outcomes of pediatric patients with acquired demyelinating disorders.
- To detail the features and management of recurrent cases within this cohort.
Main Methods:
- Retrospective chart review of 35 consecutive pediatric patients with ADDs.
- Analysis of clinical presentations, diagnoses, treatments, and follow-up data.
Main Results:
- Out of 35 cases, 24 had single events (7 acute disseminated encephalomyelitis (ADEM), 17 clinically isolated syndromes).
- 11 patients experienced recurrent demyelination, predominantly multiple sclerosis (72.7%), followed by neuromyelitis optica (NMO) and multiphasic ADEM.
- Steroids improved acute episodes, but recurrent conditions necessitated long-term immunomodulation (e.g., azathioprine for NMO, interferon-β/glatiramer acetate for MS).
Conclusions:
- Current consensus definitions for pediatric ADDs require further validation.
- Larger studies are needed to identify predictors of recurrence in pediatric demyelinating disorders.
Objective:
This is a retrospective chart review of consecutive children with acquired demyelinating disorders presenting to a north Indian tertiary care hospital over 4 years. The aim of this review is to describe all the patients (with single event as well as those with recurrences) with detailed description of those who recurred.
Materials And Methods:
Overall 35 cases were reviewed and their clinical presentations, diagnosis, management, and follow-up are being presented.
Results:
Out of 35 cases, 24 did not show any recurrences (seven acute disseminated encephalomyelitis (ADEM) and 17 clinically isolated syndromes). Amongst the 11 patients with recurrent demyelination, majority were multiple sclerosis (8/11, 72.7%) followed by neuromyelitis optica (NMO; 2/11), and multiphasic ADEM (1/11). The median disease duration and follow-up since onset for those with recurrent episodes is 4 years (2.5-4.5 years). Steroids caused significant improvement in acute episodes of demyelination. However, recurrent demyelinating disorders like multiple sclerosis and NMO required long-term immunomodulation. Azathioprine currently is the most favored long-term immunomodulator used in NMO. Interferon-β and glatiramer acetate are currently recommended for multiple sclerosis. However, azathioprine may be a suitable alternative in a resource-limited setting.
Conclusion:
The consensus definitions for these groups of disorders need further validation in the pediatric age group. Studies with larger population size are required to characterize features that predict future recurrences.
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