Acute disseminated encephalomyelitis: a review of 18 cases in childhood.
OBJECTIVE
Acute disseminated encephalomyelitis (ADEM) is a treatable inflammatory demyelinating disorder seen more commonly in children than in adults. It typically presents to general paediatricians, often, like encephalitis, with non-specific cerebrospinal fluid findings. The brain computerized tomography scan is usually normal, so is falsely reassuring and delays the diagnosis, which might result in considerable morbidity. The present study was initiated to report on the various modes of presentation and raise the awareness of the diagnosis of ADEM among general paediatricians.


METHODS
A retrospective review of the case notes of 18 children with a diagnosis of ADEM established in a tertiary referral centre from 1995 to 2000 was undertaken with particular reference to clinical features, investigations and treatment.


RESULTS
The most common presenting features were ataxia (10 cases), followed by headache (eight cases) and weakness (five cases). Magnetic resonance imaging (MRI) of the brain was needed to confirm the diagnosis in all 18 children. Treatment usually included a course of intravenous methylprednisolone followed by a tapering dose of oral prednisolone over several weeks. Although the outcome for most of the children was generally good, two relapsed after cessation of steroids and five children had ongoing disabilities.


CONCLUSIONS
The investigation of choice for establishing the diagnosis of ADEM was MRI of the brain. Other investigations were seldom helpful in reaching the diagnosis. Early diagnosis and prompt treatment of ADEM will probably reduce morbidity.
Gupte
G
G
Department of Paediatrics Walsgrave Hospitals NHS Trust, Clifford, Bridge Road, Coventry, Nottingham, UK.
Stonehouse
M
M
Wassmer
E
E
Coad
N A G
NA
Whitehouse
W P
WP
D000208:Acute Disease; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004673:Encephalomyelitis, Acute Disseminated / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011859:Radiography; D016896:Treatment Outcome; D006113:United Kingdom