Invited Presentations Invited Abstracts

HT06.02 - Presentation 02

Speakers
  • D. Sato
Authors
  • D. Sato
Presentation Number
HT06.02
Presentation Topic
Invited Presentations
Lecture Time
10:27 - 10:39

Abstract

Abstract

Since the discovery of antibodies against aquaporin-4 (AQP4-IgG) in patients with Neuromyelitis Optica Spectrum Disorders (NMOSD), an increasing number of AQP4-IgG+ NMOSD patients were described having limited forms such as longitudinally extensive myelitis or recurrent and/or bilateral optic neuritis, area postrema attacks with persistent (> 48 hours) hiccups, nausea and vomiting and those patients with associated diencephalic, brainstem and cerebral lesions “typical” for NMOSD. According to the international consensus diagnostic criteria for NMOSD published on 2015, the diagnosis of NMOSD for AQP4-IgG positive cases is possible if there is a suggestive attack with involvement 1 of 6 core locations (optic nerve, spinal cord, area postrema of the dorsal medulla, brainstem, diencephalon or cerebrum). In seronegative patients, two or more core locations must be affected, with at least one of the attacks in the optic nerve, spinal cord or the area postrema, and additional magnetic resonance imaging (MRI) criteria should be fulfilled. The 2015 NMOSD diagnostic criteria recommends cell-based assays (CBA) to detected AQP4-IgG. However, the availability of diagnostic tests such as AQP4-IgG by CBA is still limited in low income countries, as well as long-term treatments recently approved for NMOSD. More recently, antibodies against the myelin oligodendrocyte glycoprotein (MOG-IgG) have been incorporated in the clinical practice in many developed countries as an important tool to differentiate patients with MOG-IgG associated Optic Neuritis, Encephalitis, and/or Myelitis (MONEM) from seronegative NMOSD and other demyelinating CNS disorders. Nevertheless, the availability of MOG-IgG CBAs is even more restricted than AQP4-IgG, as it is limited to research laboratories in many countries. The management of these patients require an international effort to support patients from countries without access to antibody testing for AQP4-IgG and MOG-IgG, as well as provide drugs to reduce the risk of further attacks and disability.

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