Tumefactive Demyelination Associated with Bilateral Optic Neuritis in Neuromyelitis Optica Spectrum Disorders

AbstractTumefactive demyelination is an uncommon neurological disorder mimicking tumors. It is one of the rare varieties of demyelinating disorders, often causing diagnostic dilemma among neuroscientists. The literature tells us about approaching these patients added by peculiar neuroimaging findings. Neuromyelitis optica is an immune mediated inflammatory clinical disorder, typically involving optic nerves bilaterally and longitudinally extensive transverse myelitis. With the revelation of aquaporin four channels, its distribution in the brain and related antibody, the concept of neuromyelitis optica spectra disorders has been evolved. In this case report, our intention is to present a young female who presented with bilateral vision loss with tumor-like mass lesion in cerebral cortex. Such an association of bilateral optic neuropathy involving chiasmatic region, suggestive of neuromyelitis optica spectrum disorder (NMOSD) with tumefactive demyelination is rarely reported in the literature.

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Occurrence of acute large and edematous callosal lesions in neuromyelitis optica

Multiple Sclerosis Journal ◽

10.1177/1352458509103301 ◽

2009 ◽

Vol 15 (6) ◽

pp. 695-700 ◽

Cited By ~ 60

Author(s):

M Nakamura ◽

T Misu ◽

K Fujihara ◽

I Miyazawa ◽

I Nakashima ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Medical Records ◽

Brain Magnetic Resonance Imaging ◽

Transverse Myelitis ◽

Resonance Imaging ◽

Longitudinally Extensive Transverse Myelitis ◽

Lower Margin ◽

Chronic Stage ◽

The Brain ◽

Extensive Transverse Myelitis

Background The corpus callosum is commonly involved in multiple sclerosis (MS), but the characteristics of callosal lesions in neuromyelitis optica (NMO) are unknown. Objective To reveal the features of callosal lesions in NMO in comparison to MS. Methods We retrospectively reviewed the medical records and the brain magnetic resonance imaging films of 56 patients with MS and 22 patients with NMO. Results In MS, 36 (64.3%) of 56 patients had callosal lesions, but only four patients had acute lesions. All such acute lesions were small, isolated and non-edematous, and the intensity was homotonic. Chronic lesions were observed in 34 patients with MS, and 32 (94%) of them presented small lesions located at the callosal lower margin (“hemi-oval pattern”). Meanwhile, four (18.2%) patients with NMO had callosal lesions, and three of them had acute lesions. Those acute lesions were multiple, large edematous ones with heterogeneous intensity (“marbled pattern”). In the chronic stage, the lesions shrank or disappeared. Conclusions Acute large, edematous callosal lesions occasionally occur in NMO. Similar to longitudinally extensive transverse myelitis, such callosal lesions may reflect severe edematous inflammation in NMO, and may provide additional evidence that the pathogenesis in NMO is different from that in MS.

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Systemic lupus erythematosus with the development of neuromyelitis optica (Devic's syndrome) is a rare combination of autoimmune diseases

Modern Rheumatology Journal ◽

10.14412/1996-7012-2019-4-89-95 ◽

2019 ◽

Vol 13 (4) ◽

pp. 89-95 ◽

Cited By ~ 1

Author(s):

E. S. Vinogradova ◽

A. P. Panova ◽

N. M. Bulanov ◽

P. I. Novikov ◽

S. V. Moiseev

Keyword(s):

Systemic Lupus Erythematosus ◽

Autoimmune Diseases ◽

Neuromyelitis Optica ◽

Lupus Erythematosus ◽

Demyelinating Disease ◽

Transverse Myelitis ◽

Young Female ◽

Systemic Lupus ◽

Devic’S Syndrome ◽

Immune Mediated

Neuromyelitis optica ((NMO), Devic's syndrome) is an immune-mediated inflammatory demyelinating disease characterized by transverse myelitis and optic neuritis. Determination of the level of antibodies to aquaporin 4 (NMO-IgG) is presently one of the key methods for the diagnosis and assessment of the activity of ONM, which allows this disease to be differentiated from multiple sclerosis and other demyelinating CNS lesions. ONM can occur not only as an independent disease, but also as a syndrome in different systemic diseases, such as: systemic lupus erythematosus (SLE), antineutrophilic cytoplasmic antibody-associated vasculitides, Sjögren's disease, etc. (up to 50–70%). In such situations, the clinician is always confronted with a question as whether the patient can have two rare autoimmune diseases or develop ONM as a systemic manifestation of rheumatic disease.The paper describes a clinical case of a young female patient with SLE concurrent with a CNS lesion, the manifestations of which corresponded to ONM. The patient had focal changes in the substance of the brain and spinal cord, as evidenced by magnetic resonance imaging, as well as high NMO-IgG titers. The development of ONM worsens SLE prognosis and requires active immunosuppressive therapy. The patient received three plasmapheresis sessions, ultrahigh-dose glucocorticoid and cyclophosphamide therapy, followed by replacement with azathioprine, causing a stable clinical and laboratory disease remission to be achieved.

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Longitudinal extensive transverse myelitis—it's not all neuromyelitis optica

Nature Reviews Neurology ◽

10.1038/nrneurol.2011.176 ◽

2011 ◽

Vol 7 (12) ◽

pp. 688-698 ◽

Cited By ~ 81

Author(s):

Corinna Trebst ◽

Peter Raab ◽

Elke Verena Voss ◽

Paulus Rommer ◽

Mazen Abu-Mugheisib ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Transverse Myelitis ◽

Longitudinal Extensive Transverse Myelitis ◽

Extensive Transverse Myelitis

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MRI Features of Aquaporin-4 Antibody–Positive Longitudinally Extensive Transverse Myelitis: Insights into the Diagnosis of Neuromyelitis Optica Spectrum Disorders

American Journal of Neuroradiology ◽

10.3174/ajnr.a5551 ◽

2018 ◽

Vol 39 (4) ◽

pp. 782-787 ◽

Cited By ~ 7

Author(s):

C.G. Chee ◽

K.S. Park ◽

J.W. Lee ◽

H.W. Ahn ◽

E. Lee ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Transverse Myelitis ◽

Aquaporin 4 ◽

Neuromyelitis Optica Spectrum Disorders ◽

Longitudinally Extensive Transverse Myelitis ◽

Mri Features ◽

Spectrum Disorders ◽

Aquaporin 4 Antibody ◽

Extensive Transverse Myelitis

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Atypical myelitis in patients with multiple sclerosis: Characterization and comparison with typical multiple sclerosis and neuromyelitis optica spectrum disorders

Multiple Sclerosis Journal ◽

10.1177/1352458520906995 ◽

2020 ◽

pp. 135245852090699

Author(s):

K Bigaut ◽

C Lambert ◽

L Kremer ◽

C Lebrun ◽

M Cohen ◽

...

Keyword(s):

Multiple Sclerosis ◽

Neuromyelitis Optica ◽

Transverse Myelitis ◽

First Episode ◽

Neuromyelitis Optica Spectrum Disorders ◽

Disability Status ◽

Plane Transverse ◽

Spectrum Disorders ◽

Extensive Transverse Myelitis

Background: Atypical myelitis in multiple sclerosis (MS) is characterized by extensive myelitis in the longitudinal (longitudinally extensive transverse myelitis) or axial plane (transverse myelitis). Objective: To characterize a cohort of MS patients with atypical myelitis. Methods: Atypical myelitis was extracted from the French and Luxembourg MS databases and compared to two cohorts of MS patients with typical myelitis and neuromyelitis optica spectrum disorders (NMOSDs) patients with myelitis. Results: We enrolled 28 MS patients with atypical myelitis, 68 MS patients with typical myelitis and 119 NMOSD patients with a first episode of myelitis. MS patients with atypical myelitis were characterized by a mean age of 34.0 (±10.7) years and 64.3% were women. In 82.1% of the patients, atypical myelitis was the first episode of MS. Mean Expanded Disability Status Scale (EDSS) scores at nadir and 3–6 months after onset were 4.1 ± 2.1 and 3.3 ± 2, respectively. Differences between groups revealed a predominance of cervicothoracic myelitis and a higher level of disability in NMOSD patients. Disability in MS patients with atypical myelitis was more severe than in the MS patients with typical myelitis; 28% had already converted to progressive MS within our mean follow-up of 39.6 (±30.4) months. Conclusion: Atypical myelitis may be the first presentation of MS and is associated with poorer prognosis.

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Neuromyelitis optica spectrum disorder and systemic lupus erythematosus

Lupus ◽

10.1177/0961203319888692 ◽

2019 ◽

Vol 28 (14) ◽

pp. 1722-1726 ◽

Cited By ~ 1

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.

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