Neuropediatrics 2008; 39 - V19
DOI: 10.1055/s-0029-1215732

Clinical long term follow-up of children presenting with a severe acute disseminated encephalomyelitis (ADEM)

A Nagl 1, S Lütjen 2, S Zotter 1, A Blaschek 3, C Korenke 4, H Holthausen 2, K Rostasy 1
  • 1Medizinsche Universität Innsbruck, Department Kinder- und Jugendheilkunde, Univ.- Klinik für Pädiatrie IV, Schwerpunkt Neuropädiatrie, Entwicklungsneurologie und Angeborene Stoffwechselstörungen, Innsbruck, Austria
  • 2Behandlungszentrum Vogtareuth, Klinik für Neuropädiatrie und Neurologische Rehabilitation, Epilepsiezentrum für Kinder und Jugendliche, Tagesklinik für Neuropädiatrie, Vogtareuth, Germany
  • 3Dr. von Haunersches Kinderspital, Ludwig-Maximilians-Universität München, Pädiatrische Neurologie und Entwicklungsneurologie, München, Germany
  • 4Klinikum Oldenburg, Zentrum für Kinder- und Jugendmedizin- Elisabeth- Kinderkrankenhaus, Neuropädiatrie, Oldenburg, Germany

Aims: The acute disseminated encephalomyelitis (ADEM) is an acute, inflammatory- demyelinating disorder of the CNS. Outcome is regarded in the majority of cases as good.

Objective: To examine the long term outcome of children with ADEM, who were referred for further rehabilitation.

Methods: Children with ADEM according to the criteria of the International Society of MS in Children who were referred to the rehabilitation centre Vogtareuth were included. Neuropsychological outcome was assessed with review of the medical records and a standardized parental questionnaire (KOPKIJ). Functional and neurological impairment was evaluated with a standardized telephone- based interview (EDSS- Score).

Results: 12 children, with a mean age at onset of 6.9 years (1y10mo- 13y) were included. All children had focal- neurological symptoms and changes in mental status at presentation in addition to a compatible MRI of the brain.

11 patients with a mean follow-up time of 5.9 years had a monophasic course of the disease. 1 child had a multiphasic ADEM. At present 2 children have an EDSS- score of 0, 3 have an EDSS score between 1 to 2 (mild disability) and 7 have an EDSS- Score between 3 to 9 (moderate/severe disability).

Results of neuropsychological evaluation were available from 10 children. 5 children have cognitive deficits in the categories alertness (n=5), memory (n=1), school-performance (n=3), visual-spatial- skills (n=2) impulse control (n=2).

Conclusion: Clinical outcome and prognosis of ADEM is generally regarded as favourable. However, the results of our study indicate that children with a severe manifestation of ADEM and subsequent rehabilitation may have in the majority of cases neurological and neuropsychological handicaps.