Acute disseminated encephalomyelitis in children: clinical features and HLA-DR linkage

This study includes 90 children ( 41 female and 49 male) in the age range of 2 - 16 years with acute disseminated encephalomyelitis (ADEM). Thirty-three patients developed ADEM following rubella infection, 26 children following varicella infection, 20 suspected viral aetiology ADEM and 11 multiphasic disseminated encephalomyelitis (MDEM). All patients had neurological, routine laboratory and viral serology study with an enzyme-linked immunosorbent assay. Brain and/or spinal cord magnetic resonance imaging (MRI) were performed in 14 children. A follow-up study was in 1 - 5 years. Typing of DRB1 gene HLA class II was performed in 38 patients. We established that the varicella and rubella groups had preferential patterns. Rubella ADEM is characterized by acute explosive onset, seizures, coma and moderate pyramidal signs, whereas varicella infection is characterized by cerebella ataxia and mild pyramidal dysfunction. The suspected viral aetiology ADEM was characterized by polisymptomatic presentation. MDEM was characterized by older age of patients (11.6 +/- 2.8 years), more severe and prolonged local neurological symptoms, including myelitis symptoms and marked extrapyramidal signs, with distinct demyelination in MRIs. As a whole, ADEM is associated with DRB1*01 and DRB1* 017( 03) in the Russian population. Thus, ADEM is a separate autoimmune condition with a specific mechanism due to the type of genetic immunoregulatory base and specificity of viral trigger.