Neurológia pre prax 1/2010

Neuromyelitis optica (Devic's disease) – first demyelinating aquaporin channelopathy?

Neuromyelitis optica (NMO) (also known as Devic‘s disease or Devic‘s syndrome) is an idiopathic inflammatory disorder of the central nervous system (CNS), usually with a relapsing-remittent course. This disease is characterised by recurrent uni- or bilateral optic neuritis (ON), or presents concurrently with transverse myelitis- typically a longitudinally extensive form (LETM). In patients with a full clinical manifestation, establishment of diagnosis is no major issue. At the beginning of the disease, an isolated manifestation of either recurrent optic neuritis or myelitis can lead to incorrect diagnosis of multiple sclerosis (MS). Currently, we are able to assess the serum antibodies against aquaporin 4 (NMO-IgG, also known as AQP4-IgG), which are highly sensitive (75–91 %) and specific (91–100 %) for NMO. These antibodies can significantly facilitate correct diagnosis already in the time of first symptoms, and, in case of isolated ON or LETM, they can predict the risk of relapse or full manifestation of NMO. NMO IgG positivity is one of the supportive criteria within the revised Wingerchuk‘ s criteria for diagnosis of NMO from 2006.

Keywords: neuromyelitis optica, aquaporin 4, NMO-IgG/AQP4-IgG, multiple sclerosis.