Clinical Outcome of Children Presenting with a Severe Manifestation of Acute Disseminated Encephalomyelitis

Abstract

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.

Conclusion: 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.

ADEM - children - follow-up - cerebrospinal fluid - EDSS

References

• 1 Banwell BL, Anderson PE. Neuropsychological features of pediatric multile sclerosis.  Neurology. 2002;  58 A173-A174
• 2 Dale RC, de SC, Chong WK. et al . Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children.  Brain. 2000;  12 2407-2422
• 3 Hahn CD, Miles BS, MacGregor DL. et al . Neurocognitive outcome after acute disseminated encephalomyelitis.  Pediatr Neurol. 2003;  29 117-123
• 4 Hynson JL, Kornberg AJ, Coleman LT. et al . Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children.  Neurology. 2001;  56 1308-1312
• 5 Gleissner U, Lendt M, Mayer S. et al . A new questionnaire for recognizing cognitive problems in children and adolescents.  Nervenarzt. 2006;  77 449-465
• 6 Jacobs RK, Anderson VA, Neale JL. et al . Neuropsychological outcome after acute disseminated encephalomyelitis: impact of age at illness onset.  Pediatr Neurol. 2004;  31 191-197
• 7 Khong PL, Ho HK, Cheng PW. et al . Childhood acute disseminated encephalomyelitis: the role of brain and spinal cord MRI.  Pediatr Radiol. 2002;  32 59-66
• 8 Krupp LB, Banwell B, Tenembaum S. Consensus definitions proposed for pediatric multiple sclerosis and related disorders.  Neurology. 2007;  68 ((16 Suppl 2)) S7-12
• 9 Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS).  Neurology. 1983;  33 1444-1452
• 10 Lechner-Scott J, Kappos L, Hofman M. et al . Can the expanded disability status scale be assessed by telephone?.  Mult Scler. 2003;  9 154-159
• 11 Menge T, Kieseier BC, Nessler S. et al . Acute disseminated encephalomyelitis: an acute hit against the brain.  Curr Opin Neurol. 2007;  20 247-254
• 12 Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients.  Neurology. 2002;  59 1224-1231
• 13 Wingerchuk DM, Lucchinetti CF. Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis.  Curr Opin Neurol. 2007;  20 343-350

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