Diagnosis and management of neuromyelitis optica

Background and purpose: Neuromyelitis optica (NMO) or Devic′s disease is a rare inflammatory and demyelinating autoimmune disorder of the central nervous system (CNS) characterized by recurrent attacks of optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM), which is distinct from multiple sclerosis (MS). The guidelines are designed to provide guidance for best clinical practice based on the current state of clinical and scientific knowledge.

Search strategy: Evidence was collected from searches of original articles, case reports and meta-analyses in the Medline and Cochrane databases. In addition, clinical practice guidelines of professional neurological and rheumatological organizations were studied.

Results: Different diagnostic criteria for NMO diagnosis (Wingerchuk et al. Revised NMO criteria, 2006; Miller et al. NMSS task force criteria, 2008) and features potentially indicative of NMO facilitate the diagnosis. In addition, guidance for the work-up and diagnosis of spatially limited NMO spectrum disorders is provided by the Task Force. Owing to a lack of studies fulfilling the requirement for the highest levels of evidence the Task Force suggests treatment of acute exacerbations and attack prevention based on expert opinion.

Conclusions: Studies of diagnosis and management of NMO fulfilling requirements for the highest levels of evidence (class I–III rating) are limited and diagnostic and therapeutic concepts based on expert opinion and consensus of the Task Force members were drawn up for this guideline.