Acute disseminated encephalomyelitis review in 8 pediatric patients

Aim: Acute disseminated encephalomyelitis (ADEM) is a rare, immune-mediated inflammatory disease. We describe its clinical characteristics and review them in context of neurophysiological and neuroimaging features.

Methods: A retrospective (2001–2007), observational, single center multi-case report study was conducted. Clinical and neuroimaging features as well as biological markers of 8 patients (5 to 15 years), diagnosed with ADEM were evaluated.

Results: There was a male predominance (6/8). Four patients had associated infections and fever, four presented only neurological symptoms. Neurological presentation varied between ataxia, unilateral sensory deficit, altered level of consciousness, dysarthria, hemi- or monolimb paresis, seizures and diplopia. An infectious aetiology could not be established in the CSF of any of the patients, whereas the serology was positive in five patients: Mycoplasma, Enterovirus, Parainfluenza III, HSV, Influenza A. Oligoclonal banding (CSF) could be observed in a single patient. On MRI scans two patients presented 2–3 lesions, five patients presented more than three lesions. Lesions were localised in both hemispheres, corpus callosum, thalamus, the basal ganglia, pons, cerebellum and myelon. Four patients received high-dose steroids parenterally over 3–5 days followed by oral steroids. Complete Remission was found in five patients. Three patients showed discrete neurological sequelae. The number and localisation of MRI lesions did not correlate with the severity of acute clinical manifestation. There was no distinct effect of steroid therapy on neurological outcome.

Conclusion: ADEM is a potentially severe demyelinating disorder that shows a wide variety in clinical manifestation. No correlation between the number and localization of MRI scan lesions and severity of acute clinical manifestation was recognized. There was no difference in recovery (time and outcome) in patients with or without steroid treatment. Total remission seemed to be more likely if lesions were not located in the pons, thalamus or basal ganglia.