CLINICAL FEATURES OF ACUTE DEMYELINATING DISEASE AND RECENT EFFICACY OF METHYPREDNISOLONE / PREDNISONE THERAPY IN CHILDREN

Zhang YC, Chen LQ, Lu YF, Lu Q

Shanghai Children's Hospital, Shanghai, China.

Objective: Advances in radiologic and immunologic technique have increased the recognition of acute demyelinating disease (ADD). However, little information is available on childhood. The aim of this investigation is to study the clinical features and evaluate the steroid therapy efficacy on ADD in children.

Methods: Eleven cases with ADD 3.8 to 8.1 years of age were enrolled to analyze the clinical records, Magnetic resonance imagine (MRI) finding, and observe the steroid therapy efficacy. The study covered the 6 years from May, 1994 to April, 2000. Five cases were male and 6 cases for female.

Results: Seven cases involved with fever, respiratory or gastrointestinal tracts symptoms before neurological abnormal. The predominant finding included fatigue, lethargy, headache, cranial nerves signs abnormal and paralyses. The pecentage of those abnormal were 63.6%（7 cases）,36.4%（4 cases）,36.4%（4 cases）,54.5% （6 cases）and 54.5%（6 cases）, respectively. MRI scanning shown low signal or isointense on T1WI and high signal on T2WI at white cerebrum, basal ganglia, brainstem and cerebellum. The symptom were catabatic after 4 to 12 weeks Methyprednisolone (10~20mg/kg.d) / prednisone (1~2mg/kg.d) therapy in all patients but 3 cases relapsed in 8 to 18 months later.

Conclusion: The investigation contains the following characteristics of ADD in childhood: ①The ADD may be associated with infection ; ② The clinic presentations were variable, but fatigue, lethargy, headache, cranial nerves and pyramidal system signs abnormal were predominant features ; ③ MRI scanning was one of the dominating diagnostic method; ④ The steroid therapy were effective in recent period .

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