Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles,Pathophysiology and Therapeutic Choices

Awad, Amer; Stüve, Olaf

doi:10.2174/157015911796557948

Cited by 50 publications

(55 citation statements)

References 166 publications

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“…27 Demyelination and axonal loss occur, often involving the gray matter, a finding that is supported by neuroimaging in adults and children. 28,29 Necrosis and cavitation can result in severe disability, especially in NMO. 30 The mechanisms of cellular and humoral autoimmune responses contributing to spinal cord inflammation and degeneration remain unclear.…”

Section: Differential Diagnosis and Evaluationmentioning

confidence: 99%

“…32 IL-6 is secreted following activation of astrocytes and microglia, exerting its effect on oligodendroglia and axons and mediating cellular injury in spinal cord culture sections. 28 There is a correlation between elevated IL-6 levels and disability in patients with ATM. 33 Positive results from early-phase intervention studies with monoclonal antibodies to attenuate IL-6 responses in NMO have direct implications on patients with ATM with elevated IL-6.…”

Section: Differential Diagnosis and Evaluationmentioning

confidence: 99%

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Pediatric transverse myelitis

et al. 2016

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Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder and contributes to 20% of children experiencing a first acquired demyelinating syndrome (ADS). ATM must be differentiated from other presentations of myelopathy and may be the first presentation of relapsing ADS such as neuromyelitis optica (NMO) or multiple sclerosis (MS). The tenets of the diagnostic criteria for ATM established by the Transverse Myelitis Consortium Working Group can generally be applied in children; however, a clear sensory level may not be evident in some. MRI lesions are often centrally located with high T2 signal intensity involving gray and neighboring white matter. Longitudinally extensive ATM occurs in the majority. Asymptomatic lesions on brain MRI are seen in more than one-third and predict MS or NMO. The role of antibodies such as myelin oligodendrocyte glycoprotein in monophasic and relapsing ATM and their significance in therapeutic approaches remain unclear. ATM is a potentially devastating condition with variable outcome and presents significant cumulative demands on health and social care resources. Children generally have a better outcome than adults, with one-half making a complete recovery by 2 years. There is need for standardization of clinical assessment and investigation protocols to enable international collaborative studies to delineate prognostic factors for disability and relapse. There are no robust controlled trials in children or adults to inform optimal treatment of ATM, with one study currently open to recruitment. This review provides an overview of current knowledge of clinical features, investigative workup, pathogenesis, and management of ATM and suggests future directions. Neurology ® 2016;87 (Suppl 2):S46-S52 GLOSSARY ADS 5 acquired demyelinating syndrome; AFM 5 acute flaccid myelitis; AQP4 5 aquaporin-4; ASIA 5 American Spinal Injury Association; ATM 5 acute transverse myelitis; GBS 5 Guillain-Barré syndrome; IL 5 interleukin; IVIg 5 IV immunoglobulin; LETM 5 longitudinally extensive TM; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; PLEX 5 plasmapheresis; SLE 5 systemic lupus erythematosus; TMCWG 5 Transverse Myelitis Consortium Working Group.Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder classically described as demyelinating. ATM comprises a subgroup of the noncompressive transverse myelopathies.1,2 It is a potentially devastating condition with variable outcome. 3 ATM must be differentiated from other, rarer presentations of noncompressive myelopathy.4,5 ATM may be the first presentation of relapsing acquired demyelinating syndromes (ADS) such as neuromyelitis optica (NMO) or multiple sclerosis (MS). A PubMed search using the terms "pediatric or paediatric transverse myelitis" revealed that more than 200 articles have been published in English on the topic between 1976 and 2015. In this article we discuss current knowledge on clinical features, pathogenesis, and investigative and management ...

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Section: Differential Diagnosis and Evaluationmentioning

confidence: 99%

Section: Differential Diagnosis and Evaluationmentioning

confidence: 99%

Pediatric transverse myelitis

et al. 2016

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“…A study made by Young et al demonstrated a bimodal distribution with two distinct peaks: 10-19 and 30-39 years old [5]. Racial/familial or gender predilection did not exist despite pediatric population has been affected in 28% of cases [5].…”

Section: Introductionmentioning

confidence: 95%

Review of the Etiological Causes and Diagnosis of Myelitis and Its Medical Orientation Protocol

Besteiro¹,

Guimarães²

2017

J Neurol Neurophysiol

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Transverse myelitis (TM) is an inflammatory condition caused by a range of possibilities which can lead to a deregulation of our motor, sensory and autonomic systems. Therefore, it creates a major impact on personal and professional life. Because it causes great social and professional disability, it is crucial that the clinicians quickly recognize this condition so that treatment can be started thereby minimizing these negative effects. Thus, the aim of this review is to provide a practical clinical approach to the most important causal diseases of TM, clinical presentation and to elaborate a diagnostic algorithm that helps classifying and simplifying the work of the clinicians when facing a TM episode.

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“…This finding explained the development of humoral response against optic nerve, spinal cord and periventricular spaces [6]. Clinical consequences of the NMO are: decrease of visual acuity, amaurosis, paresia or/and sphincters dysfunction, all of them produce a negative influence in physical, emotional, productive and social patient's life [2,3,7,8]. NMO´s acute treatment consists in intravenous methylprednisolone 1 or 2 g per day, 3 to 5 dose.…”

Section: Introductionmentioning

confidence: 99%

Optic Neuritis Unresponsive to Steroids: Prevalence, Characteristics and Plasma Exchange Treatment

Rito¹,

Gil²,

Catalina³

2015

J Mult Scler (Foster City)

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Background: Neuromyelitis optica (NMO) is a demyelinating disease associated to the presence of anti-aquaporin 4 (AQP4) antibodies and damage to the optic nerve and spinal cord. Sometimes Steroids do not stop the autoimmune response. Plasma exchange (PE) is a purification technique used to remove high weight molecules (e.g. antibodies), and it has shown efficacy in NMO unresponsive to steroids. However, the roll of PE in optic neuritis (ON) unresponsive to steroids secondary to NMO has been less studied.

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Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles,Pathophysiology and Therapeutic Choices

Cited by 50 publications

References 166 publications

Pediatric transverse myelitis

Pediatric transverse myelitis

Review of the Etiological Causes and Diagnosis of Myelitis and Its Medical Orientation Protocol

Optic Neuritis Unresponsive to Steroids: Prevalence, Characteristics and Plasma Exchange Treatment

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