Autoimmune Aquaporin-4 Myopathy in Neuromyelitis Optica Spectrum

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Autoimmune aquaporin-4 myopathy in neuromyelitis optica spectrum.

IMPORTANCE Documentation of muscle pathology compatible with targeting of sarcolemmal aquaporin-4 (AQP4) by complement-activating IgG implies involvement of organs beyond the central nervous system in neuromyelitis optica spectrum disorders. OBSERVATIONS We report on a 51-year-old woman who had relapsing optic neuritis, transverse myelitis, AQP4-IgG seropositivity, and recurrent myalgias with...

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Anti-myelin Oligodendrocyte Glycoprotein in Aquaporin-4 Negative Neuromyelitis Optica Spectrum Disorder

Background: The absence of Aquaporin-4 Antibody (AQP4-Ab) in a fraction of the Neuromyelitis Optica Spectrum Disorder (NMOSD) patients has led to a search for other serologic markers. Myelin Oligodendrocyte Glycoprotein (MOG) is a protein component of the myelin sheets encapsulating the neural fibers. Objectives: We aimed to compare the presence and levels of anti-MOG (Ig-G) in a group of s...

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Neuromyelitis optica and the evolving spectrum of autoimmune aquaporin-4 channelopathies: a decade later.

The discovery of AQP4-IgG (a pathogenic antibody that targets the astrocytic water channel aquaporin-4), as the first sensitive and specific biomarker for any inflammatory central nervous system demyelinating disease (IDD), has shifted emphasis from the oligodendrocyte and myelin to the astrocyte as a central immunopathogenic player. Neuromyelitis optica (NMO) spectrum disorders (SDs) represent...

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Convergence spasm due to aquaporin-positive neuromyelitis optica spectrum disorder

A female 27 presented with nausea and diplopia for 1 week. On examination she had normal vertical gaze but would develop convergence with miosis whenever she made horizontal saccades. Pupils were 6 mm and unreactive to light. MRI showed extensive hyperintensity in the dorsal midbrain and thalamus. Spinal MRI and CSF were both normal. Serum aquaporin-4-antibody was positive. She was treated with...

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Antibody to Aquaporin 4 in the Diagnosis of Neuromyelitis Optica

BACKGROUND Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system (CNS) of putative autoimmune aetiology. Early discrimination between multiple sclerosis (MS) and NMO is important, as optimum treatment for both diseases may differ considerably. Recently, using indirect immunofluorescence analysis, a new serum autoantibody (NMO-IgG) has been detected in NMO patients....

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ژورنال

عنوان ژورنال: JAMA Neurology

سال: 2014

ISSN: 2168-6149

DOI: 10.1001/jamaneurol.2014.775