The Prevalence of Long Spinal Cord Lesions and Anti-Aquaporin 4 Antibodies in Neuromyelitis Optica Patients in Taiwan

2011 ◽  
Vol 65 (2) ◽  
pp. 99-104 ◽  
Author(s):  
Kai-Chen Wang ◽  
Ching-Piao Tsai ◽  
Chao-Lin Lee ◽  
Shao-Yuan Chen ◽  
Shyi-Jou Chen
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Aquaporin 4 ◽  
Spinal Cord Lesions

Cortical oscillopsia without nystagmus, an isolated symptom of neuromyelitis optica spectrum disorder with anti-aquaporin 4 antibody

2011 ◽  
Vol 18 (2) ◽  
pp. 244-247 ◽  
Author(s):  
Sung-Min Kim ◽  
Ji-Soo Kim ◽  
Young Eun Heo ◽  
Hye-Ran Yang ◽  
Kyung Seok Park
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Ocular Manifestation ◽  
Initial Manifestation ◽  
Ocular Symptoms ◽  
Head Tremor ◽  
Aquaporin 4 Antibody

Neuromyelitis optica (NMO), mainly affecting optic nerve and spinal cord, can also manifest diverse ocular symptoms due to brain abnormalities. We present a cortical oscillopsia without nystagmus or head tremor in a patient with neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin 4 antibody. This rare ocular manifestation, which is easily underestimated owing to absence of the typical nystagmus, can be an initial manifestation of NMOSD.

scholarly journals Holocord involvement with sparing of the peripheral white matter rim in longitudinally extensive spinal cord lesions of neuromyelitis optica

2015 ◽  
Vol 6 (S1) ◽  
pp. 78-79
Author(s):  
Shotaro Hayashida ◽  
Katsuhisa Masaki ◽  
Takuya Matsushita ◽  
Mitsuru Watanabe ◽  
Ryo Yamasaki ◽  
...  
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Neuromyelitis Optica ◽  
Spinal Cord Lesions

scholarly journals Poor Responses to Interferon-Beta Treatment in Patients with Neuromyelitis Optica and Multiple Sclerosis with Long Spinal Cord Lesions

PLoS ONE ◽  
2014 ◽  
Vol 9 (6) ◽  
pp. e98192 ◽  
Author(s):  
Kai-Chen Wang ◽  
Kuan-Hsiang Lin ◽  
Tzu-Chi Lee ◽  
Chao-Lin Lee ◽  
Shao-Yuan Chen ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Interferon Beta ◽  
Spinal Cord Lesions

scholarly journals Memantine ameliorates motor impairments and pathologies in a mouse model of neuromyelitis optica spectrum disorders.

2020 ◽  
Author(s):  
Leung-Wah Yick ◽  
Chi-Ho Tang ◽  
Oscar Ka-Fai Ma ◽  
Jason Shing-Cheong Kwan ◽  
Koon Ho CHAN
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Neurotrophic Factor ◽  
Water Channel ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Glutamate Excitotoxicity ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Motor Impairments ◽  
Spectrum Disorders

Abstract Background: Neuromyelitis optica spectrum disorders (NMOSD) are central nervous system (CNS) autoimmune inflammatory demyelinating diseases characterized by recurrent episodes of acute optic neuritis and transverse myelitis. Aquaporin-4 immunoglobulin G (AQP4-IgG) autoantibodies, which target the water channel aquaporin-4 (AQP4) on astrocytic membrane, are pathogenic in NMOSD. Glutamate excitotoxicity, which is triggered by internalization of AQP4-glutamate transporter complex after AQP4-IgG binding to astrocytes, is involved in early NMOSD pathophysiologies. We studied the effects of memantine, a N-methyl-D-aspartate (NMDA) receptor antagonist, on motor impairments and spinal cord pathologies in mice which received human AQP4-IgG. Methods: Purified IgG from AQP4-IgG-seropositive NMOSD patients were passively transferred to adult C57BL/6 mice with disrupted blood-brain barrier. Memantine was administered by oral gavage. Motor impairments of the mice were assessed by beam walking test. Spinal cords of the mice were assessed by immunofluorescence and ELISA. Results: Oral administration of memantine ameliorated the motor impairments induced by AQP4-IgG, no matter the treatment was initiated before (preventive) or after (therapeutic) disease flare. Memantine profoundly reduced AQP4 and astrocyte loss, and attenuated demyelination and axonal loss in the spinal cord of mice which had received AQP4-IgG. The protective effects of memantine were associated with inhibition of apoptosis and suppression of neuroinflammation, with decrease in microglia activation and neutrophil infiltration and reduction of increase in levels of proinflammatory cytokines including interleukin-1β (IL-1β), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF- α). In addition, memantine elevated growth factors including brain-derived neurotrophic factor (BDNF), glial cell line-derived neurotrophic factor (GDNF) and vascular endothelial growth factor (VEGF) in the spinal cord. Conclusions: Our findings support that glutamate excitotoxicity and neuroinflammation plays important roles in complement-independent pathophysiology during early development of NMOSD lesions, and highlight the potential of oral memantine as a therapeutic agent in NMOSD acute attacks.

Lymphocyte Subsets Are Associated with Disease Status in Neuromyelitis Optica Spectrum Disorders

2021 ◽  
pp. 1-10
Author(s):  
Hong Yang ◽  
Wei Liu ◽  
Yi-Fan Wu ◽  
De-Sheng Zhu ◽  
Xia-Feng Shen ◽  
...  
Keyword(s):  
Spinal Cord ◽  
B Cells ◽  
Neuromyelitis Optica ◽  
Peripheral Blood ◽  
Lymphocyte Subsets ◽  
Disease Status ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Spinal Cord Lesions ◽  
Cross Sectional ◽  
Spectrum Disorders

<b><i>Objective:</i></b> At present, studies on lymphocytes are mostly conducted on CD19<sup>+</sup> B cells and CD27<sup>+</sup> B cells in neuromyelitis optica spectrum disorders (NMOSDs), but the exact changes in lymphocyte subsets (CD19<sup>+</sup> B cells, CD3<sup>+</sup> T cells, CD4<sup>+</sup> Th cells, CD8<sup>+</sup> Ts cells, the CD4<sup>+</sup>/CD8<sup>+</sup> ratio, and NK [CD56+ CD16] cells) have rarely been studied. This study aimed to assess lymphocyte subset changes in patients with NMOSD. <b><i>Methods:</i></b> We performed a cross-sectional study of consecutive patients with acute NMOSD (<i>n</i> = 41), chronic NMOSD (<i>n</i> = 21), and healthy individuals (<i>n</i> = 44). Peripheral blood samples were obtained upon admission, and lymphocyte subsets were analyzed by flow cytometry. Levels of lymphocyte subsets among 3 groups were compared and its correlation with the length of spinal cord lesions was analyzed. <b><i>Results:</i></b> The levels of peripheral blood CD19<sup>+</sup> B cells were significantly higher in patients with acute and chronic NMOSD than in healthy controls (HCs) (17.91 ± 8.7%, 13.08 ± 7.562%, and 12.48 ± 3.575%, respectively; <i>p</i> &#x3c; 0.001) and were positively correlated with the length of spinal cord lesions in acute NMOSD (<i>r</i> = 0.433, <i>p</i> &#x3c; 0.05). The peripheral blood CD4<sup>+</sup>/CD8<sup>+</sup> ratio was significantly lower in patients with acute NMOSD and chronic NMOSD than in HCs (1.497 ± 0.6387, 1.33 ± 0.5574, and 1.753 ± 0.659, respectively; <i>p</i> &#x3c; 0.05), and the levels of peripheral blood NK (CD56+ CD16) cells were significantly lower in patients with acute and chronic NMOSD than in HCs (13.6 ± 10.13, 11.11 ± 7.057, and 14.7 [interquartile range = 9.28], respectively; <i>p</i> &#x3c; 0.01). <b><i>Conclusions:</i></b> The levels of certain subsets of peripheral blood lymphocytes are associated with disease status in NMOSD.

scholarly journals IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel

2005 ◽  
Vol 202 (4) ◽  
pp. 473-477 ◽  
Author(s):  
Vanda A. Lennon ◽  
Thomas J. Kryzer ◽  
Sean J. Pittock ◽  
A.S. Verkman ◽  
Shannon R. Hinson
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Blood Brain Barrier ◽  
Neuromyelitis Optica ◽  
Immunoglobulin G ◽  
Protein Complex ◽  
Demyelinating Disease ◽  
Water Channel ◽  
Aquaporin 4 ◽  
Specific Marker

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that selectively affects optic nerves and spinal cord. It is considered a severe variant of multiple sclerosis (MS), and frequently is misdiagnosed as MS, but prognosis and optimal treatments differ. A serum immunoglobulin G autoantibody (NMO-IgG) serves as a specific marker for NMO. Here we show that NMO-IgG binds selectively to the aquaporin-4 water channel, a component of the dystroglycan protein complex located in astrocytic foot processes at the blood-brain barrier. NMO may represent the first example of a novel class of autoimmune channelopathy.

scholarly journals Devic’s neuromyelitis optica: a critical review

2008 ◽  
Vol 66 (1) ◽  
pp. 120-138 ◽  
Author(s):  
Marco Aurélio Lana-Peixoto
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Water Channel ◽  
Demyelinating Diseases ◽  
Aquaporin 4 ◽  
Igg Antibody ◽  
Vascular Walls ◽  
Devic’S Neuromyelitis Optica

Devic's neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by predominant involvement of the optic nerves and spinal cord. In Asian countries relapsing NMO has been known as opticospinal multiple sclerosis. It has long been debated if NMO is a variant of multiple sclerosis (MS) or a distinct disease. Recent studies have shown that NMO has more frequently a relapsing course, and results from attack to aquaporin-4 which is the dominant water channel in the central nervous system, located in foot processes of the astrocytes. Distinctive pathological features of NMO include perivascular deposition of IgG and complement in the perivascular space, granulocyte and eosinophil infiltrates and hyalinization of the vascular walls. These features distinguish NMO from other demyelinating diseases such as MS and acute demyelinating encephalomyelopathy. An IgG-antibody that binds to aquaporin-4, named NMO-IgG has high sensitivity and specificity. Magnetic resonance imaging (MRI) studies have revealed that more frequently there is a long spinal cord lesion that extends through three or more vertebral segments in length. Brain MRI lesions atypical for MS are found in the majority of cases. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosupressive agents are recommended for prophylaxis of relapses.

Central vein sign and other radiographic features distinguishing myelin oligodendrocyte glycoprotein antibody disease from multiple sclerosis and aquaporin-4 antibody-positive neuromyelitis optica

2021 ◽  
pp. 135245852110070
Author(s):  
John R Ciotti ◽  
Noah S Eby ◽  
Matthew R Brier ◽  
Gregory F Wu ◽  
Salim Chahin ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Optic Nerve ◽  
Neuromyelitis Optica ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Aquaporin 4 ◽  
Central Vein ◽  
Nerve Involvement ◽  
Fluid Attenuated Inversion Recovery ◽  
Optic Nerve Involvement

Background: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) can radiographically mimic multiple sclerosis (MS) and aquaporin-4 (AQP4) antibody-positive neuromyelitis optica spectrum disorder (NMOSD). Central vein sign (CVS) prevalence has not yet been well-established in MOGAD. Objective: Characterize the magnetic resonance imaging (MRI) appearance and CVS prevalence of MOGAD patients in comparison to matched cohorts of MS and AQP4+ NMOSD. Methods: Clinical MRIs from 26 MOGAD patients were compared to matched cohorts of MS and AQP4+ NMOSD. Brain MRIs were assessed for involvement within predefined regions of interest. CVS was assessed by overlaying fluid-attenuated inversion recovery (FLAIR) and susceptibility-weighted sequences. Topographic analyses were performed on spinal cord and orbital MRIs when available. Results: MOGAD patients had fewer brain lesions and average CVS+ rate of 12.1%, compared to 44.4% in MS patients ( p = 0.0008). MOGAD spinal cord and optic nerve involvement was lengthier than MS (5.8 vs 1.0 vertebral segments, p = 0.020; 3.0 vs 0.5 cm, p < 0.0001). MOGAD patients tended to have bilateral/anterior optic nerve pathology with perineural contrast enhancement, contrasting with posterior optic nerve involvement in NMOSD. Conclusion: CVS+ rate and longer segments of involvement in the spinal cord and optic nerve can differentiate MOGAD from MS, but do not discriminate as well between MOGAD and AQP4+ NMOSD.

An acute onset of paraparesis in 65 years old Croatian woman – a case report

2018 ◽  
Author(s):  
V. Kosta
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Neuromyelitis Optica ◽  
Demyelinating Disease ◽  
Acute Onset ◽  
Spinal Cord Lesions ◽  
The Central Nervous System ◽  
High Doses

Neuromyelitis optica (NMO, Devic`sdisease) is a rare inflammatory, demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. Seropositivity for NMO-IgG (aquaporin 4antybodies) and longitudinally extensive spinal cord lesions (3 or more segments) are characteristics of NMO. We described a 65-year old woman with an acute onset of paraparesis that was not recognized as NMO at the beginning. The diagnosis was made three months later when she was readmitted because of the relapse.Despite the treatment with high doses of methylprednisolone, plasmapheresis and immunoglobulins her condition stayed unchanged – she was paraplegic and incontinent.

Longitudinally extensive spinal cord lesions: not always neuromyelitis optica

2018 ◽  
Vol 118 (2) ◽  
pp. 327-329
Author(s):  
Antoine Ruyssen ◽  
Nicolas Mulquin ◽  
Thierry Gustin ◽  
Caroline Fervaille ◽  
Frédéric London
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Spinal Cord Lesions
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