Relapsing optic neuritis and isolated transverse myelitis are the predominant clinical phenotypes for patients with antibodies to myelin oligodendrocyte glycoprotein in India

Background Clinical phenotypes of patients with antibodies to myelin oligodendrocyte glycoprotein (anti-MOG+) are unknown in India. Objectives Retrospectively to characterise anti-MOG+ patients with inflammatory central nervous system disorders in India. Method A total of 87 patients with non-multiple sclerosis demyelinating disorders (excluding acute disseminated encephalomyelitis) who were seronegative for anti-aquaporin 4 antibody were retrospectively analysed using a cell-based assay for anti-MOG+ status. Results Twenty-five patients were anti-MOG+ in this cohort. They represented 28.7% (25/87) of patients who tested negative for anti-AQP4+. Sixty-four per cent (16/25) of anti-MOG+ patients were men and had a relapsing course. Patients with recurrent optic neuritis and those with a single attack of acute longitudinally extensive transverse myelitis were the most common phenotypes. Conclusion Relapsing optic neuritis was the most common phenotype, contrasting with a lower risk of relapses in transverse myelitis.

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Antibodies to myelin oligodendrocyte glycoprotein in aquaporin 4 antibody seronegative longitudinally extensive transverse myelitis: Clinical and prognostic implications

Multiple Sclerosis Journal ◽

10.1177/1352458515591071 ◽

2015 ◽

Vol 22 (3) ◽

pp. 312-319 ◽

Cited By ~ 45

Author(s):

Álvaro Cobo-Calvo ◽

María Sepúlveda ◽

Raphael Bernard-Valnet ◽

Anne Ruiz ◽

David Brassat ◽

...

Keyword(s):

Optic Neuritis ◽

Transverse Myelitis ◽

Myelin Oligodendrocyte Glycoprotein ◽

Aquaporin 4 ◽

First Episode ◽

Longitudinally Extensive Transverse Myelitis ◽

Increase Risk ◽

Cerebrospinal Fluid Pleocytosis ◽

Aquaporin 4 Antibody ◽

Extensive Transverse Myelitis

Objective: We aimed to investigate the frequency and clinical significance of antibodies to myelin oligodendrocyte glycoprotein (MOG-abs) in patients who presented with a first episode of seronegative aquaporin 4 antibody (AQP4-ab) longitudinally extensive transverse myelitis (LETM). Methods: Epidemiological, clinical, and paraclinical data of 56 patients from three European centres were analysed. Patients were retrospectively tested for MOG-abs and AQP4-abs, by cell-based assays. Findings: Thirteen (23.2%) patients were MOG-ab positive. Among the 56 patients, six (10.7%) converted to neuromyelitis optica (NMO), one (1.8%) to multiple sclerosis (MS), nine (16.1%) had recurrent LETM, and 40 (71.4%) remained as monophasic LETM. Compared with seronegative patients, those with MOG-abs were younger (median: 32.5 vs 44 years; p=0.007), had cerebrospinal fluid pleocytosis more frequently (94% vs 45%, p=0.003) and had better outcome (median Expanded Disability Status Scale (EDSS) 2.0 vs 3.0, p=0.027). MOG-ab positive patients also showed an increase risk of optic neuritis relapse and NMO conversion ( p=0.010). Conclusion: Patients with MOG-abs in AQP4-ab seronegative LETM have clinical distinctive features, higher risk of optic neuritis relapses, and better outcome than patients seronegative.

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The expanded spectrum of neuromyelitis optica: evidences for a new definition

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2012001000010 ◽

2012 ◽

Vol 70 (10) ◽

pp. 807-813 ◽

Cited By ~ 20

Author(s):

Marco A Lana-Peixoto ◽

Dagoberto Callegaro

Keyword(s):

Optic Nerve ◽

Optic Neuritis ◽

Neuromyelitis Optica ◽

Brain Mri ◽

Transverse Myelitis ◽

Aquaporin 4 ◽

Longitudinally Extensive Transverse Myelitis ◽

Index Event ◽

Aquaporin 4 Antibody ◽

Extensive Transverse Myelitis

Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

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Quantitative analysis of aquaporin-4 antibody in longitudinally extensive transverse myelitis

Journal of Neuroimmunology ◽

10.1016/j.jneuroim.2014.12.006 ◽

2015 ◽

Vol 278 ◽

pp. 26-29 ◽

Cited By ~ 7

Author(s):

Jing Wang ◽

Xin Li ◽

Da-Qi Zhang ◽

Chun-Sheng Yang ◽

Yuan Qi ◽

...

Keyword(s):

Quantitative Analysis ◽

Transverse Myelitis ◽

Aquaporin 4 ◽

Longitudinally Extensive Transverse Myelitis ◽

Aquaporin 4 Antibody ◽

Extensive Transverse Myelitis

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P.049 Medically refractory longitudinally extensive transverse myelitis successfully treated with cyclophosphamide induction

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2016.153 ◽

2016 ◽

Vol 43 (S2) ◽

pp. S32-S33

Author(s):

LJ Baxter ◽

S Chen ◽

JM Burton

Keyword(s):

Respiratory Failure ◽

Plasma Exchange ◽

Immune Therapy ◽

Transverse Myelitis ◽

Acute Disseminated Encephalomyelitis ◽

High Dose ◽

Longitudinally Extensive Transverse Myelitis ◽

Intravenous Methylprednisolone ◽

Intensive Care Admission ◽

Extensive Transverse Myelitis

Background: Longitudinally extensive transverse myelitis (LETM) is a demyelinating condition that is associated with diseases such as neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, collagen vascular disease, or can be idiopathic. LETM can be severe enough to cause quadraparesis, marked sensory dysfunction, and respiratory failure. Rarely, these patients are unresponsive to conventional immune therapy. Methods: We report two cases of severe LETM with acute development of quadraparesis and respiratory failure requiring intensive care admission and failure to respond to high-dose corticosteroids, plasma exchange, IVIg and rituximab. Disease cessation and ultimately, significant recovery, was achieved after an 8-day cyclophosphamide induction. Results: A 21 yo female with antibody positive NMOSD and a 19 yo male with idiopathic LETM remained quadraparetic and ventilator dependent with active MRIs despite multiple courses of intravenous methylprednisolone, plasma exchanges, and in the NMOSD patient, IVIg and a 4-week course of rituximab. Both patients ultimately improved significantly and are now ambulatory with subsequent cyclophosphamide induction. Conclusions: In patients with severe LETM of presumed immune origin, who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent provides a more robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are further discussed.

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