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

2012 ◽  
Vol 70 (10) ◽  
pp. 807-813 ◽  
Author(s):  
Marco A Lana-Peixoto ◽  
Dagoberto Callegaro

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.

2015 ◽  
Vol 22 (3) ◽  
pp. 312-319 ◽  
Author(s):  
Álvaro Cobo-Calvo ◽  
María Sepúlveda ◽  
Raphael Bernard-Valnet ◽  
Anne Ruiz ◽  
David Brassat ◽  
...  

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.


2011 ◽  
Vol 303 (1-2) ◽  
pp. 139-141 ◽  
Author(s):  
Diego Franciotta ◽  
Elisabetta Zardini ◽  
Roberto Caporali ◽  
Laura Piccolo ◽  
Elisa Alberici ◽  
...  

2016 ◽  
Vol 2 ◽  
pp. 205521731667563 ◽  
Author(s):  
Lekha Pandit ◽  
Douglas Kazutoshi Sato ◽  
Sharik Mustafa ◽  
Toshiyuki Takahashi ◽  
Anitha D’Cunha ◽  
...  

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.


2011 ◽  
Vol 69 (5) ◽  
pp. 824-828 ◽  
Author(s):  
Douglas Sato ◽  
Kazuo Fujihara

Neuromyelitis optica (NMO) is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4) has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks.


2011 ◽  
Vol 17 (11) ◽  
pp. 1384-1386 ◽  
Author(s):  
S Jarius ◽  
B Wildemann

We discuss a forgotten report by the famous British neuroanatomist, neuropathologist and neurologist Jacob Augustus Lockhart Clarke (1817–1880) about a 17-year-old girl with bilateral optic neuritis and longitudinally extensive transverse myelitis. This report, which appeared in 1865, i.e. 15 years prior to Wilhelm Erb’s much-cited paper on the coincidence of optic neuritis and acute myelitis, represents the first known account of a case of Devic’s syndrome or neuromyelitis optica in the English-language medical literature.


2008 ◽  
Vol 65 (7) ◽  
Author(s):  
Patrick Waters ◽  
Sven Jarius ◽  
Edward Littleton ◽  
Maria Isabel Leite ◽  
Saiju Jacob ◽  
...  

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