Clinical outcome of children presenting with a severe manifestation of acute disseminated encephalomyelitis.
BACKGROUND
Acute disseminated encephalomyelitis (ADEM) is an acute, inflammatory-demyelinating disorder of the CNS with a favourable outcome in the majority of cases.


OBJECTIVE
The aim of this study was to examine the long-term outcome of children with an initially severe form of ADEM.


METHODS
Children with ADEM according to the criteria of the International Pediatric MS Study Group (IPMSSG) referred to the rehabilitation centre Vogtareuth were included. Neurological impairment was evaluated with a standardized telephone-based interview assessing the EDSS score. Neuropsychological outcome was assessed with review of the medical records and a standardized parental questionnaire (KOPKIJ).


RESULTS
Twelve children (1 year 9 months to 13 years of age) were included. All children had focal-neurological signs and changes in mental status at presentation and an MRI of the brain showing a range of white and gray matter lesions. 11/12 patients with a mean follow-up of 6.2 years (2-13.6 years) had a monophasic course of the disease. One child had a multiphasic ADEM. Two children had an EDSS score of 0, three an EDSS of 2, five an EDSS between 3 and 5 and two children had an EDSS score of 6 and 9. Results of a standardized parental questionnaire (KOPKIJ) revealed that 7 children had deficits in the categories alertness, memory, school performance, visual-spatial skills and/or impulse control.


CONCLUSIONS
The results of our study indicate that children with an initially severe manifestation of ADEM continue to have in the majority of cases neurological and neuropsychological handicaps.
Rostásy
K
K
Department of Pediatrics IV, Division of Pediatric Neurology, Inherited Metabolic Disorders and Developmental Neurology, Medical University Innsbruck, Innsbruck, Austria. Kevin.Rostasy@uki.at
Nagl
A
A
Lütjen
S
S
Roll
K
K
Zotter
S
S
Blaschek
A
A
Korenke
G C
GC
Karenfort
M
M
Gotwald
T
T
Holthausen
H
H
D000293:Adolescent; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D015331:Cohort Studies; D004673:Encephalomyelitis, Acute Disseminated / Q000150:complications* / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009460:Neurologic Examination / Q000379:methods; D009483:Neuropsychological Tests; D013116:Spinal Cord / Q000473:pathology