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Factors Related to Long Term Motor, Behavioral, and Scholastic Outcome in Children with Acute Disseminated
Mary Iype1, Anish Ts1, Pa Mohammed Kunju1
1Government Medical College Trivandrum, Kerala, India.
Insights
Long-term outcomes for children with Acute Disseminated Encephalomyelitis (ADEM) can include motor deficits, cognitive issues, and seizures. While some risk factors were identified, ADEM is unlikely to convert to Multiple Sclerosis (MS).
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acute Disseminated Encephalomyelitis (ADEM) is an immune-mediated demyelinating disease affecting the central nervous system.
- Understanding the long-term sequelae of ADEM in children is crucial for prognosis and management.
Purpose of the Study:
- To investigate the long-term neurological and cognitive outcomes in children diagnosed with Acute Disseminated Encephalomyelitis (ADEM).
- To identify potential risk factors associated with adverse outcomes following ADEM.
Main Methods:
- A retrospective cohort study was conducted on 102 children diagnosed with ADEM according to the International Pediatric Multiple Sclerosis Study Group (IPMSSG) criteria.
- Follow-up ranged from one to 10 years, assessing motor deficits, scholastic performance, behavioral abnormalities, and recurrent demyelinating events.
- Multivariate analysis was used to identify predictors of sequelae, including modified Rankin Scale (mRS) and Expanded Disability Status Scale (EDSS) scores, and MRI findings.
Main Results:
- At follow-up, significant proportions of children experienced motor deficits (17.2%), attention deficits (25.3%), behavioral abnormalities (13.1%), seizures (7%), and poor learning skills (22.2%).
- Recurrent demyelination occurred in 7.1% of cases, with some evolving into chronic relapsing disorders but none meeting criteria for Multiple Sclerosis (MS).
- Thalamic lesions on MRI and mRS scores at discharge were associated with motor sequelae; ADEM with polyneuropathy predicted poor learning skills; tumefactive demyelination correlated with behavioral abnormality.
Conclusions:
- Children with ADEM can experience persistent motor, cognitive, and behavioral sequelae, as well as seizures and recurrent demyelinating events.
- Specific MRI findings and clinical parameters at discharge were linked to certain long-term outcomes.
- The low rate of conversion to MS suggests that ADEM is generally a monophasic illness with a low risk of developing Multiple Sclerosis.
Objective:
We studied the long-term outcome of Acute disseminated encephalomyelitis (ADEM).
Methods:
We performed a retrospective cohort study among children diagnosed with ADEM (fulfilling IPMSSG criteria). Major outcome variables were motor deficit, scholastic underperformance, and behavioral abnormality.
Results:
The inclusion criteria were fulfilled by 102 children. Three died in hospital. The follow-up ranged from one to 10 years (median 4 years). Motor deficit was seen in 17(17.2%), attention deficit in 25 (25.3%), behavioral abnormality in 13(13.1%), persistent seizures in seven (7%) invididuals and poor learning skills in 22 (22.2%). Recurrence of demyelination occurred in seven (7.1%). Two individuals had a recurrent demyelinating disorder (a chronic relapsing demyelinating disorder) that could not be classified as multiple sclerosis (MS), two had ADEM with sequential optic neuritis and three had multiphasic ADEM. At follow-up, the mean (SD) modified Rankin Scale (mRS) score was 0.556 (1.36) and Expanded Disability Status Scale score was 1.71(2.22). On multivariate analysis, the mRS score at discharge (p<0.01) and thalamic lesions on magnetic resonance imaging (MRI) (p<0.01) were associated with motor sequelae; poor learning skills with ADEM with concomitant polyneuropathy (p<0.02); and behavioral abnormality with tumefactive demyelination (p<0.02).
Conclusions:
Children who had ADEM may have motor or cognitive sequelae, seizures or recurrent demyelinating events on follow-up. We identified a few risk factors for these sequelae. Factors that affected outcome on discharge from hospital did not affect chances of having long-term sequelae. On follow-up, none of the children fulfilled the diagnostic criteria for MS, suggesting that the chance of conversion of ADEM to MS is less likely.
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