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Clin Exp Immunol. 2014 May;176(2):149-64. doi: 10.1111/cei.12271.

Neuromyelitis optica: clinical features, immunopathogenesis and treatment.

Author information

1
Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany.

Abstract

The term 'neuromyelitis optica' ('Devic's syndrome', NMO) refers to a syndrome characterized by optic neuritis and myelitis. In recent years, the condition has raised enormous interest among scientists and clinical neurologists, fuelled by the detection of a specific serum immunoglobulin (Ig)G reactivity (NMO-IgG) in up to 80% of patients with NMO. These autoantibodies were later shown to target aquaporin-4 (AQP4), the most abundant water channel in the central nervous system (CNS). Here we give an up-to-date overview of the clinical and paraclinical features, immunopathogenesis and treatment of NMO. We discuss the widening clinical spectrum of AQP4-related autoimmunity, the role of magnetic resonance imaging (MRI) and new diagnostic means such as optical coherence tomography in the diagnosis of NMO, the role of NMO-IgG, T cells and granulocytes in the pathophysiology of NMO, and outline prospects for new and emerging therapies for this rare, but often devastating condition.

KEYWORDS:

Devic syndrome; NMO-IgG; aquaporin-4 antibodies (AQP4); diagnosis; neuromyelitis optica; pathogenesis; pathophysiology; treatment

PMID:
24666204
PMCID:
PMC3992027
DOI:
10.1111/cei.12271
[Indexed for MEDLINE]
Free PMC Article

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