International consensus diagnostic criteria for neuromyelitis optica spectrum disorders

Abstract

Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS.

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

“…In addition, AP syndrome can be caused by irritation of the CTZ at the molecular level which does not necessarily show up in lesions detected by magnetic resonance imaging (MRI) as known from drug‐induced AP syndrome 3. There is evidence about an association between AP syndrome and antibody‐mediated disorders like neuromyelitis optica and neuromyelitis optica spectrum disorders (NMO/NMOSD),12, 13, 14, 15 often presenting longitudinal brainstem lesions16 and AP syndrome was recognized as a core characteristic by the International Panel for NMO Diagnosis 17, 18. AP lesions in multiple sclerosis (MS)19 seem to provoke AP syndrome less frequently than comparable NMO lesions 20.…”

Neuromyelitis optica spectrum disorder and varicella-zoster infection

“…The discovery of NMO-IgG and its target aquaporin-4 (AQP4) as a marker of NMO allowed a better understanding of the disease and, recently, a new definition and diagnostic criteria for NMO spectrum diseases (NMOSD) have been proposed [1]. In a subgroup of patients with NMOSD, a parainfectious pathogenesis may play a central role [2].…”

Neuromyelitis optica does not impact periventricular venous density versus healthy controls: a 7.0 Tesla MRI clinical study

“…All NMOSD patients were seropositive for aquaporine-4-IgG. Inclusion criteria were diagnosis of NMOSD as defined by the current international consensus diagnostic criteria for NMOSD [1], age of at least 18 years, and no contraindications to 7T MRI. Sixteen patients with relapsing-remitting MS as defined by the current panel criteria [16], and sixteen age-matched healthy control subjects that were most comparable to the NMOSD cohort regarding gender, age and -if applicable -disease duration were selected from a research database of the NeuroCure Clinical Research Center (NCRC).…”

“…Neuromyelitis optica spectrum disorders (NMOSD) are often disabling neuroinflammatory, autoimmune central nervous system conditions predominantly affecting the spinal cords and optic nerves [1][2][3][4]. Prior to the discovery of a serum immunoglobulin G antibody targeting the aquaporine-4 water channel [5,6], it was often considered a variant of multiple sclerosis (MS) as the clinical presentation of these two distinct central nervous system (CNS) diseases may overlap [1].…”

“…Prior to the discovery of a serum immunoglobulin G antibody targeting the aquaporine-4 water channel [5,6], it was often considered a variant of multiple sclerosis (MS) as the clinical presentation of these two distinct central nervous system (CNS) diseases may overlap [1]. Indeed, patients with NMOSD are still often misdiagnosed with MS in clinical practice [7,8].…”