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Acute disseminated encephalomyelitis in children: outcome and prognosis
1Hacettepe University Department of Pediatric Neurology, Ankara, Turkey. banlar@hacettepe.edu.tr
Insights
Acute disseminated encephalomyelitis (ADEM) in children often results in complete recovery, though relapses are common. Steroid treatment timing and duration significantly impact outcomes for pediatric ADEM patients.
Area of Science:
- Pediatric Neurology
- Demyelinating Disorders
- Neuroimmunology
Background:
- Acute disseminated encephalomyelitis (ADEM) is a primary demyelinating disease in children.
- Clinical presentations, prognosis, and treatment responses for childhood ADEM show variability in existing literature.
Purpose of the Study:
- To investigate clinical findings, disease course, recurrence patterns, and prognostic factors in pediatric ADEM.
- To analyze variables influencing the outcome of acute disseminated encephalomyelitis in children.
Main Methods:
- A multicenter study involving 7 tertiary referral centers, collecting data from 1990-2001.
- Evaluation of clinical, laboratory, and MRI features in a central database.
- Prognosis and course assessment based on a minimum 12-month follow-up for 46 pediatric patients.
Main Results:
- Most common initial symptoms involved motor deficits and altered consciousness.
- Complete recovery was observed in 71% of patients with a 12-month follow-up; 33% experienced relapses.
- High-dose methylprednisolone treatment correlated with complete recovery, while prolonged steroid tapering (>3 weeks) reduced relapse rates. Persistent MRI lesions were noted, particularly periventricular ones.
Conclusions:
- Childhood ADEM typically leads to complete recovery with rare severe complications, but a significant relapse rate exists.
- Early clinical indicators during the first relapse can predict multiple subsequent relapses.
- Steroid treatment's timing and duration are critical determinants of patient outcomes in ADEM.
Background:
Acute disseminated encephalomyelitis (ADEM) is the most common demyelinating disorder of childhood. Its clinical features, prognosis and treatment vary in different reports.
Objectives:
To examine a series of children with ADEM for clinical findings, course, recurrences, and possible variables affecting outcome.
Methods:
Multicentric data collected from 7 tertiary referral centers were registered and evaluated in a central database in 1990 - 2001 for clinical, laboratory, and MRI features. Course and prognosis were assessed in patients with at least 12 months' follow-up.
Results:
Forty-six patients were evaluated. Median age at onset was 8 years, M/F ratio, 1.7/1. Most common symptoms and signs pertained to the motor system and consciousness. Of 39 children with 12 months' follow-up, 71 % recovered completely. Thirteen (33 %) children had relapses. Patients who had more than one relapse (n = 4) presented with new symptoms at each attack. Treatment with high-dose methylprednisolone was associated with complete recovery, and tapering over more than 3 weeks, with a lower rate of relapses. MRI lesions could persist even in asymptomatic patients; in particular, periventricular lesions tended to disappear later than others.
Conclusions:
Complete clinical recovery is common and serious complications are rare in childhood ADEM, but the rate of relapses is considerable. Clinical picture at first relapse may help to identify patients likely to experience multiple relapses. The timing and duration of steroid treatment affects outcome.