Longitudinal extensive transverse myelitis and optic neuritis: the elements for the diagnosis of neuromyelitis optica

Neuromyelitis optica spectrum disorders (NMOSD) are characterized by severe optic neuritis and/or longitudinally extensive transverse myelitis, and some brain lesions are also unique to NMOSD. Serum autoantibodies against aquaporin-4 (AQP4) are detected in most cases of NMOSD. However, some patients with NMOSD remain seronegative despite repetitive testing during attacks with highly sensitive cell-based assays. The differential diagnosis of NMOSD is not restricted to multiple sclerosis and it includes many diseases that can produce longitudinally extensive myelitis and/or optic neuritis. We review the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSD with antibodies against myelin oligodendrocyte glycoprotein.

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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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Acute anosmia in neuromyelitis optica spectrum disorder

Multiple Sclerosis Journal ◽

10.1177/1352458520907909 ◽

2020 ◽

Vol 26 (14) ◽

pp. 1958-1960 ◽

Cited By ~ 1

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.

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A Rare Presentation of Acute Paraplegia, Longitudinal Extensive Transverse Myelitis, and Neuromyelitis Optica as Initial Neurological Presentation of Sjogren's Syndrome: A Case Report and Review of Literature

PM&R ◽

10.1016/j.pmrj.2013.08.484 ◽

2013 ◽

Vol 5 ◽

pp. S277-S278

Author(s):

Richard G. Chang ◽

Donna D'Alessio ◽

Vincent Huang

Keyword(s):

Case Report ◽

Neuromyelitis Optica ◽

Transverse Myelitis ◽

Sjogren's Syndrome ◽

Review Of Literature ◽

Rare Presentation ◽

Longitudinal Extensive Transverse Myelitis ◽

Neurological Presentation ◽

Acute Paraplegia ◽

Extensive Transverse Myelitis

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Neuromyelitis optica: phenotypic characteristics in a Brazilian case series

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20160193 ◽

2017 ◽

Vol 75 (2) ◽

pp. 81-86 ◽

Cited By ~ 7

Author(s):

Maria Cristina Del Negro ◽

Patricia Beatriz Christino Marinho ◽

Regina Maria Papais-Alvarenga

Keyword(s):

Neuromyelitis Optica ◽

Case Series ◽

Transverse Myelitis ◽

International Panel ◽

Longitudinal Extensive Transverse Myelitis ◽

Study Results ◽

Fourth Decade ◽

Spectrum Disorders ◽

Definition Of ◽

Extensive Transverse Myelitis

ABSTRACT The definition of neuromyelitis optica (NMO) is still evolving. In 2015, the International Panel for NMO Diagnosis was convened to develop revised diagnostic criteria. There have been few studies on NMO in the Brazilian population. Objective To describe the characteristics of 34 Brazilian NMO patients. To evaluate the contribution of the 2015 criteria to the diagnosis of NMO spectrum disorders (NMOSD) in 40 patients with longitudinal extensive transverse myelitis (LEMT). Methods This is a retrospective, descriptive and analytic study. Results Among NMO patients, there was a predominance of women, with onset in the fourth decade of life, and AQP4-IgG seropositivity in 73.5%. The diagnosis of NMOSD was established in 37.5% of LETM patients according to AQP4-IgG positivity and in 5% of LETM patients if the AQP4-IgG result was unknown. Conclusions The characteristics of this series are similar to those of other Western populations. The AQP4-IgG testing assists in the diagnosis of NMOSD.

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Atypical presentations of neuromyelitis optica

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2011000600019 ◽

2011 ◽

Vol 69 (5) ◽

pp. 824-828 ◽

Cited By ~ 16

Author(s):

Douglas Sato ◽

Kazuo Fujihara

Keyword(s):

Multiple Sclerosis ◽

Optic Neuritis ◽

Neuromyelitis Optica ◽

Transverse Myelitis ◽

Brain Lesions ◽

Longitudinally Extensive Transverse Myelitis ◽

Atypical Presentations ◽

Different Characteristics ◽

Extensive Transverse Myelitis ◽

Intractable Hiccups

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.

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An early case of neuromyelitis optica: on a forgotten report by Jacob Lockhart Clarke, FRS

Multiple Sclerosis Journal ◽

10.1177/1352458511411758 ◽

2011 ◽

Vol 17 (11) ◽

pp. 1384-1386 ◽

Cited By ~ 25

Author(s):

S Jarius ◽

B Wildemann

Keyword(s):

Optic Neuritis ◽

Neuromyelitis Optica ◽

English Language ◽

Medical Literature ◽

Transverse Myelitis ◽

Longitudinally Extensive Transverse Myelitis ◽

Devic’S Syndrome ◽

Early Case ◽

Extensive Transverse Myelitis

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.

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