Idiopathic severe recurrent transverse myelitis: a restricted variant of neuromyelitis optica

Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system characterized by severe episodes of optic nerve and spinal cord inflammation. NMO-IgG (anti-aquaporin-4) has been recently described as a sensitive and specific marker for NMO. As there have been prior published reports of an association between NMO and systemic autoimmune diseases, the prognostic value of the antibody test in these cases is uncertain. We describe a 47-year old woman with recurrent transverse myelitis and a long-standing history of systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APLS). While she did not have a history of optic neuritis, serological testing for the NMO-IgG was positive when she was admitted for her second episode of transverse myelitis. Testing for the NMO-IgG in cases of isolated or recurrent transverse myelitis attributed to current SLE and APLS may help clarify the diagnosis of a distinct disease process likely to cause recurrent and severe disability, warranting more aggressive immunotherapy. Multiple Sclerosis 2008; 14: 425—427. http://msj.sagepub.com

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Meningitis as a recurrent manifestation of anti-AQP4/anti-MOG negative neuromyelitis optica spectrum disorder: a case report

BMC Neurology ◽

10.1186/s12883-021-02133-8 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Chenyang Zhang ◽

Kang Zhang ◽

Bing Chen ◽

Jiao Yin ◽

Miaomiao Dong ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Area Postrema ◽

Neurological Diseases ◽

Brain Magnetic Resonance Imaging ◽

Transverse Myelitis ◽

Corticosteroid Treatment ◽

Neck Stiffness ◽

Mixed Cell ◽

Glucose Levels ◽

The Right

Abstract Background Neuromyelitis optica spectrum disorders (NMOSD), a group of autoimmune neurological diseases, involve the optic nerve, spinal cord, and brain. Meningitis is rarely reported as the primary clinical manifestation of both anti-aquaporin-4 (AQP4)/ anti-myelin oligodendrocyte glycoprotein (MOG) antibody-negative NMOSD (NMOSDneg). Case presentation A 30-year-old man initially presented with fever, headache, and neck stiffness. Lumbar puncture revealed mixed cell reaction and decreased glucose levels. As a result, tuberculous meningitis was suspected. After 1 month, the patient developed longitudinally extensive transverse myelitis and area postrema syndrome. This was followed by the presentation of meningitis-like symptoms once again in the third attack, but his condition eventually improved after corticosteroid treatment without relapse for 2 years. However, he was readmitted to our hospital owing to symptoms of diplopia, hiccup, and numbness in the right hand. Brain magnetic resonance imaging (MRI) revealed that the area postrema still contained lesions. Spinal MRI revealed several segmental enhancements at the C4–C5, T1, and T5 levels. Anti-AQP4 and anti-MOG antibodies were persistently absent in the serum and cerebrospinal fluid (CSF). The patient was finally diagnosed with NMOSDneg. Conclusions Meningitis could be a recurrent manifestation of NMOSDneg and requires more careful evaluation.

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Elucidating distinct clinico-radiologic signatures in the borderland between neuromyelitis optica and multiple sclerosis

Journal of Neurology ◽

10.1007/s00415-021-10619-1 ◽

2021 ◽

Author(s):

Maciej Juryńczyk ◽

Elżbieta Klimiec-Moskal ◽

Yazhuo Kong ◽

Samuel Hurley ◽

Silvia Messina ◽

...

Keyword(s):

Multiple Sclerosis ◽

Neuromyelitis Optica ◽

Unmet Need ◽

Transverse Myelitis ◽

Overlap Syndrome ◽

Brain Lesions ◽

Central Vein ◽

Normal Brain ◽

Group 4 ◽

Group 2

Abstract Background Separating antibody-negative neuromyelitis optica spectrum disorders (NMOSD) from multiple sclerosis (MS) in borderline cases is extremely challenging due to lack of biomarkers. Elucidating different pathologies within the likely heterogenous antibody-negative NMOSD/MS overlap syndrome is, therefore, a major unmet need which would help avoid disability from inappropriate treatment. Objective In this study we aimed to identify distinct subgroups within the antibody-negative NMOSD/MS overlap syndrome. Methods Twenty-five relapsing antibody-negative patients with NMOSD features underwent a prospective brain and spinal cord MRI. Subgroups were identified by an unsupervised algorithm based on pre-selected NMOSD/MS discriminators. Results Four subgroups were identified. Patients from Group 1 termed “MS-like” (n = 6) often had central vein sign and cortical lesions (83% and 67%, respectively). All patients from Group 2 (“spinal MS-like”, 8) had short-segment myelitis and no MS-like brain lesions. Group 3 (“classic NMO-like”, 6) had high percentage of bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM, 80% and 60%, respectively) and normal brain appearance (100%). Group 4 (“NMO-like with brain involvement”, 5) typically had a history of NMOSD-like brain lesions and LETM. When compared with other groups, Group 4 had significantly decreased fractional anisotropy in non-lesioned tracts (0.46 vs. 0.49, p = 0.003) and decreased thalamus volume (0.84 vs. 0.98, p = 0.04). Conclusions NMOSD/MS cohort contains distinct subgroups likely corresponding to different pathologies and requiring tailored treatment. We propose that non-conventional MRI might help optimise diagnosis in these challenging patients.

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

Multiple Sclerosis Journal ◽

10.1177/1352458517705479 ◽

2017 ◽

Vol 23 (14) ◽

pp. 1950-1954 ◽

Cited By ~ 3

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.

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First Presentation of an Acquired Demyelinating Syndrome

Journal of Pediatric Neurology ◽

10.1055/s-0037-1606380 ◽

2017 ◽

Vol 16 (03) ◽

pp. 164-170

Author(s):

Rachel Gottlieb-Smith ◽

Amy Waldman

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Differential Diagnosis ◽

Optic Neuritis ◽

Clinical Features ◽

Neuromyelitis Optica ◽

Transverse Myelitis ◽

Acute Disseminated Encephalomyelitis ◽

The Central Nervous System

AbstractAcquired demyelinating syndromes (ADS) present with acute or subacute monofocal or polyfocal neurologic deficits localizing to the central nervous system. The clinical features of distinct ADS have been carefully characterized including optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. These disorders may all be monophasic disorders. Alternatively, optic neuritis, partial transverse myelitis, and acute disseminated encephalomyelitis may be first presentations of a relapsing or polyphasic neuroinflammatory disorder, such as multiple sclerosis or neuromyelitis optica. The clinical features of these disorders and the differential diagnosis are discussed in this article.

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Severe aquaporin 4-IgG-positive neuromyelitis optica with disseminated herpes zoster in a pregnant woman successfully treated with intravenous immunoglobulin

Multiple Sclerosis Journal - Experimental Translational and Clinical ◽

10.1177/2055217318758119 ◽

2018 ◽

Vol 4 (1) ◽

pp. 205521731875811

Author(s):

Yuki Matsumoto ◽

Mario Tsuchiya ◽

Shakespear Norshalena ◽

Chikako Kaneko ◽

Jin Kubo ◽

...

Keyword(s):

Herpes Zoster ◽

Pregnant Woman ◽

Intravenous Immunoglobulin ◽

Neuromyelitis Optica ◽

Rescue Therapy ◽

Transverse Myelitis ◽

Aquaporin 4 ◽

High Dose ◽

Healthy Baby ◽

Severe Infections

A 26-year-old, 17-week pregnant woman developed aquaporin-4-IgG-positive severe longitudinally extensive transverse myelitis during the course of disseminated herpes zoster and became quadriparetic. She was unresponsive to high-dose intravenous methylprednisolone but became able to walk without assistance after intravenous immunoglobulin. One and a half months later, left optic neuritis developed but her vision improved with intravenous immunoglobulin. The only sequela was left T5 girdle sensation, and she delivered a healthy baby. Intravenous immunoglobulin may be a rescue therapy in aquaporin-4-IgG-positive neuromyelitis optica attacks in pregnant women, especially those with severe infections.

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Recurrent transverse myelitis associates with anti-Ro (SSA) autoantibodies

Neurology ◽

10.1212/01.wnl.0000101715.32569.1a ◽

2004 ◽

Vol 62 (1) ◽

pp. 147-149 ◽

Cited By ~ 57

Author(s):

L. K. Hummers ◽

C. Krishnan ◽

L. Casciola-Rosen ◽

A. Rosen ◽

S. Morris ◽

...

Keyword(s):

Transverse Myelitis ◽

Recurrent Transverse Myelitis

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Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease: practical considerations

Practical Neurology ◽

10.1136/practneurol-2017-001787 ◽

2018 ◽

Vol 19 (3) ◽

pp. 187-195 ◽

Cited By ~ 23

Author(s):

Maciej Juryńczyk ◽

Anu Jacob ◽

Kazuo Fujihara ◽

Jacqueline Palace

Keyword(s):

Neuromyelitis Optica ◽

Inflammatory Diseases ◽

Transverse Myelitis ◽

Myelin Oligodendrocyte Glycoprotein ◽

Lesion Volume ◽

Imaging Features ◽

Visual Disability ◽

Permanent Disability ◽

Spectrum Disorders ◽

Aqp4 Antibody

The field of central nervous system (CNS) inflammatory diseases has recently broadened to include a new condition associated with pathogenic serum antibodies against myelin oligodendrocyte glycoprotein (MOG). This is distinct from multiple sclerosis (MS) and aquaporin-4 (AQP4) antibody neuromyelitis optica spectrum disorders (NMOSD). MOG antibody-associated disease phenotypes are varied and range from classical neuromyelitis optica to acute demyelinating encephalomyelitis and cortical encephalitis. The diagnosis depends on using a reliable, specific and sensitive assay of the antibody. Clinical and imaging features of MOG-associated syndromes overlap with AQP4 antibody NMOSD but can be usually distinguished from MS: in particular, the silent lesions typical of MS that progressively increase lesion volume are rare in MOG antibody disease. The disease can relapse but medium-term immunosuppression appears to be protective. Permanent disability, particularly severe ambulatory and visual disability, is less frequent than in AQP4 antibody NMOSD and usually results from the onset attack. However, sphincter and sexual dysfunction after a transverse myelitis is common. Here we review the practical aspects of diagnosing and managing a patient with MOG antibody-associated disease.

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