Neuromyelitis optica

Wildemann, Brigitte; Jarius, Sven; Paul, Friedemann

doi:10.1055/s-0031-1297261

Cited by 6 publications

(2 citation statements)

References 29 publications

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“…NMO is still an incurable disease. The goal of treating acute NMO events is to improve relapse symptoms and restore neurological functions; long‐term immunosuppression aims to prevent further attacks . Any treatment recommendations are limited by the small size of most studies, which were mostly retrospective case‐series.…”

Section: Treatmentmentioning

confidence: 99%

Neuromyelitis optica: clinical features, immunopathogenesis and treatment

Jarius

Wildemann

Paul

2014

Clinical and Experimental Immunology

312

221

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Neuromyelitis optica: clinical features, immunopathogenesis and treatment OTHER ARTICLES PUBLISHED IN THIS SERIESParaneoplastic neurological syndromes. Clinical and Experimental Immunology 2014, 175: 336-48. Disease-modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategies. Clinical and Experimental Immunology 2014, 175: 359-72. Monoclonal antibodies in treatment of multiple sclerosis. Clinical and Experimental Immunology 2014, 175: 373-84 SummaryThe 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.

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Section: Treatmentmentioning

confidence: 99%

Neuromyelitis optica: clinical features, immunopathogenesis and treatment

Jarius

Wildemann

Paul

2014

Clinical and Experimental Immunology

312

221

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“…Die in Asien im Vergleich zur konventionellen multiplen Sklerose relativ häufige sog. optikospinale multiple Sklerose wurde als mehrheitlich mit AQP4-IgG assoziiert erkannt und unterscheidet sich in diesen Fällen nicht von der konventionellen NMO [26,28,31].…”

Section: Epidemiologieunclassified

Neuromyelitis optica

Pache¹,

Wildemann²,

Paul³

et al. 2018

Neuroradiol Scan

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Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system (CNS) that predominantly affects the spinal cord and optic nerves. The neuropathologic hallmarks comprise deposits of antibodies and complement as well as loss of astrocytes, secondary degeneration of oligodendrocytes and neurons, and necrotic lesions with infiltration of neutrophilic and eosinophilic granulocytes. Pathognomonic serum autoantibodies against aquaporin-4 (AQP4-IgG, also termed NMO-IgG) are detectable in around 80?% of NMO patients and help to distinguish this rare entity from multiple sclerosis. The target antigen of NMO-IgG, the water channel protein AQP4, is ubiquitously expressed within the CNS and, as a component of the blood-brain barrier, highly concentrated in the endfeet of astrocytes. New international consensus criteria for NMO spectrum disorders, published in 2015, allow earlier diagnosis. Besides the two index manifestations, optic neuritis and transverse myelitis, involvement of the brainstem and diencephalon is relatively common in NMO. Inflammatory lesions of the area postrema typically cause intractable nausea and vomiting and/or hiccups. NMO mostly follows a relapsing course, especially in AQP4-IgG-positive cases. The treatment of acute exacerbations comprises intravenous methylprednisolone pulses and/or plasma exchange, and prevention of attacks requires long-term therapy with immunosuppressants and/or B-cell-depleting monoclonal antibodies.

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