The clinical spectrum and immunobiology of parainfectious neuromyelitis optica (Devic) syndromes

Abstract

In a subgroup of patients with neuromyelitis optica (NMO), a severe inflammatory demyelinating disorder of autoimmune origin characterized by recurrent attacks of optic neuritis and longitudinally extensive transverse myelitis, a parainfectious pathogenesis may play a central role. We systematically evaluated such reports in the literature published between 1975 and 2009 in order to characterize parainfectious NMO syndromes. Identified were 25 cases, whereof 11 were in association with viral and 14 with bacterial pathogens. Sufficient clinical and paraclinical information was available in 16 patients (11 women). Median age was 8 years for children and 32 years for adults. Acute febrile illness preceding or in close relation with neurological symptoms was most common and the association with varicella-zoster virus and Mycobacterium pneumonia most frequent. In the majority, the course was monophasic (88%) and disability sustained (with complete recovery in only 25%). Seven patients fulfilled the revised NMO diagnosis criteria of 2006; none was seropositve for aquaporin-4 antibodies. Immune mechanisms potentially involved in parainfectious NMO syndromes include bystander activation, molecular mimicry, and the exacerbation of a pre-existing central nervous system (CNS) disorder by a systemic infection. However, current studies are not sufficient to define the place of parainfectious NMO syndromes within the spectrum of inflammatory disorders of the CNS.