Longitudinally Extensive Transverse Myelitis: A Rare Neuromyelitis Optica Spectrum Disorder

Consultant ◽  
2020 ◽  
Vol 60 ◽  
Author(s):  
Aman Deep ◽  
Andrew N. Wilner
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Extensive Transverse Myelitis

Neuromyelitis optica spectrum disorder and systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1722-1726 ◽  
Author(s):  
M M Thabah ◽  
Sekar D ◽  
R Pranov ◽  
M M V Moulitej ◽  
A Ramesh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Systemic Lupus ◽  
Autoimmune Conditions ◽  
Inflammatory Syndrome ◽  
Extensive Transverse Myelitis

Neuromyelitis optica spectrum disorder is an inflammatory syndrome that is associated with many autoimmune conditions. We present the case of a patient who had longitudinally extensive transverse myelitis and antibodies to aquaporin 4 IgG (AQP4-IgG). Based on presence of lymphopenia, further workup revealed strong ANA positivity, anti-Sm antibodies, and low serum complements suggesting presence of systemic lupus erythematosus. The patient promptly responded to intravenous pulse methylprednisolone and five sessions of plasma exchange. At 1 year, she is on maintenance treatment with low dose prednisolone, azathioprine, and hydroxychloroquine, she has had no relapse and no other clinical features of lupus. This case is an illustration that neuromyelitis optica spectrum disorder can be the first manifestation of systemic lupus erythematosus.

Acute anosmia in neuromyelitis optica spectrum disorder

2020 ◽  
Vol 26 (14) ◽  
pp. 1958-1960 ◽  
Author(s):  
Joseph Marshall ◽  
Iris Kleerekooper ◽  
Indran Davagnanam ◽  
S Anand Trip
Keyword(s):  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Resonance Imaging ◽  
Longitudinal Extensive Transverse Myelitis ◽  
Magnetic Resonance Imaging Mri ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

The cardinal features of neuromyelitis optica spectrum disorder (NMOSD) are optic neuritis, longitudinal extensive transverse myelitis and area postrema syndrome. Olfactory dysfunction is not listed as a feature in the NMOSD diagnostic criteria. Here, we present an aquaporin-4 antibody positive patient who, in addition to classical features of NMOSD, developed acute anosmia with magnetic resonance imaging (MRI) evidence of olfactory bulb abnormalities. While the association of anosmia and NMOSD has been rarely noted previously, to our knowledge, no prior cases have found this to be one of the presenting features of a relapse nor have they identified acute radiological correlates.

Neuromyelitis optica spectrum disorder as a paraneoplastic syndrome: a rare and challenging diagnosis

2021 ◽  
Vol 14 (5) ◽  
pp. e239389
Author(s):  
Mukhtar Gibril ◽  
Richard Walters
Keyword(s):  
Neuromyelitis Optica ◽  
Cell Lymphoma ◽  
Transverse Myelitis ◽  
Inflammatory Lesion ◽  
B Cell Lymphoma ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Large B Cell Lymphoma ◽  
The Central Nervous System ◽  
Extensive Transverse Myelitis

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system, often associated with aquaporin-4 antibodies (AQP4-Ab). NMOSD may present as a subacute myelopathy, progressing over days with MRI revealing a contiguous inflammatory lesion of the spinal cord, ≥3 vertebral segments, a longitudinally extensive transverse myelitis. We describe an unusual paraneoplastic form of AQP4-Ab NMOSD that developed in a patient with an advanced diffuse large B-cell lymphoma. The patient had an unusual hyperacute onset, reaching a clinical nadir within hours.

scholarly journals Neuromyelitis optica spectrum disorder with delayed lesion on spinal cord magnetic resonance imaging

2018 ◽  
Author(s):  
Jeong Yeon Kim
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Magnetic Resonance ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Resonance Imaging ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

The term Neuromyelitis optica spectrum disorder (NMOSD) is a recently proposed for neuromyelitis optica and related syndromes. Along with serum anti-aquaporin 4 antibody status, detection of a lesions extending over ≥3 contiguous spinal cord segments (longitudinally extensive transverse myelitis, LETM) associated with acute myelitis is the most specific neuroimaging characteristic of NMOSD. 1 We present a case of NMOSD with delayed lesion on spinal cord magnetic resonance imaging.

scholarly journals Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder with recurrent short partial transverse myelitis and favorable prognosis: Two new cases

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Attack Phase ◽  
Long Term Prognosis

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.

scholarly journals Neuromyelitis Optica Spectrum Disorder and Anti-MOG Syndromes

Biomedicines ◽  
2019 ◽  
Vol 7 (2) ◽  
pp. 42 ◽  
Author(s):  
Marco A. Lana-Peixoto ◽  
Natália Talim
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Water Channel ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Immunosuppressive Drugs ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system that frequently involve the optic nerves and the spinal cord. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Some agents used for MS treatment may be deleterious to NMOSD. NMOSD is frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, located in the astrocytic processes at the blood-brain barrier (BBB). On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization.

Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis

2006 ◽  
Vol 59 (3) ◽  
pp. 566-569 ◽  
Author(s):  
Brian G. Weinshenker ◽  
Dean M. Wingerchuk ◽  
Sandra Vukusic ◽  
Linda Linbo ◽  
Sean J. Pittock ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Extensive Transverse Myelitis
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