Longitudinally extensive transverse myelitis suspected for isolated Neuro-Behçet: A diagnostic conundrum

Sawaf, Abdullah Al; Berger, Joseph

doi:10.1016/j.msard.2015.07.003

Cited by 7 publications

(5 citation statements)

References 23 publications

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Section: Differential Diagnosis Of Nmosdmentioning

confidence: 99%

“…145 Nevertheless, neurological manifestations in BD can sometimes precede its systemic manifestations, thereby delaying the proper diagnosis of NBD. 146 Recently, two levels of diagnostic criteria for NBD -definite and probable NBD -were proposed in those without systemic manifestations. 143 The distinctive features of NBD, from those of NMOSD, includes the presence of headache with or without meningoencephalitis (about 70%), 142 a progressive course (38%), 147 and severe brainstem/cerebral atrophy and/or leukoencephalopathy in brain MRI.…”

Section: Cns Involvement In Patients With Systemic Autoimmune Diseasementioning

confidence: 99%

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Differential diagnosis of neuromyelitis optica spectrum disorders

Kim

Lee

et al. 2017

Ther Adv Neurol Disord

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Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients.

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Section: Differential Diagnosis Of Nmosdmentioning

confidence: 99%

Section: Cns Involvement In Patients With Systemic Autoimmune Diseasementioning

confidence: 99%

Differential diagnosis of neuromyelitis optica spectrum disorders

Kim

Lee

et al. 2017

Ther Adv Neurol Disord

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“…[7203989118149] The diagnosis of Behçet's normally includes ulcers, but the neuropathic symptoms can appear before the systemic symptoms and are not included in the recent diagnostic criteria. [561186] Behçet's is similar to NMOSD with headache, a progressive disease course and requires treatment. [556123126301] Gluten enteropathy, or celiac disease, is also studied but causation has not been shown in NMOSD.…”

Section: Clinical Differentiation and Comorbidities Of Neuromyelitis mentioning

confidence: 99%

Comprehensive review of neuromyelitis optica and clinical characteristics of neuromyelitis optica patients in Puerto Rico

Zarei¹,

Eggert²,

Franqui-Dominguez³

et al. 2018

Surg Neurol Int

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Neuromyelitis optica (NMO) is an immune-mediated inflammatory disorder of the central nervous system. It is characterized by concurrent inflammation and demyelination of the optic nerve (optic neuritis [ON]) and the spinal cord (myelitis). Multiple studies show variations in prevalence, clinical, and demographic features of NMO among different populations. In addition, ethnicity and race are known as important factors on disease phenotype and clinical outcomes. There are little data on information about NMO patients in underserved groups, including Puerto Rico (PR). In this research, we will provide a comprehensive overview of all aspects of NMO, including epidemiology, environmental risk factors, genetic factors, molecular mechanism, symptoms, comorbidities and clinical differentiation, diagnosis, treatment, its management, and prognosis. We will also evaluate the demographic features and clinical phenotype of NMO patients in PR. This will provide a better understanding of NMO and establish a basis of knowledge that can be used to improve care. Furthermore, this type of population-based study can distinguish the clinical features variation among NMO patients and will provide insight into the potential mechanisms that cause these variations.

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“…The prevalence of spinal cord involvement in NBD ranges from 2.5 to 30% [7][8][9][10]. In the reported cases, the main site of involvement was the cervical and thoracic spinal cord with myelitis-like inflammatory lesions continuing for more than two segments and extending to the brainstem in some cases [7][8][9][10]. The MRI of patient 1 showed a continuous lesion extending from the midbrain to the entire spinal cord.…”

Section: Discussionmentioning

confidence: 84%

“…We diagnosed the two patients on the basis of the criteria proposed by the international consensus recommendation for NBD [3,4]. The prevalence of spinal cord involvement in NBD ranges from 2.5 to 30% [7][8][9][10]. In the reported cases, the main site of involvement was the cervical and thoracic spinal cord with myelitis-like inflammatory lesions continuing for more than two segments and extending to the brainstem in some cases [7][8][9][10].…”

Section: Discussionmentioning

confidence: 99%

Two Cases of Probable Neuro-Behçet’s Disease with Longitudinally Extensive Transverse Myelitis

et al. 2021

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We report 2 cases of probable neuro-Behçet’s disease (NBD) with longitudinally extensive transverse myelitis (LETM). In both cases, the patients presented paraplegia, as well as sensory, bladder, and rectal disturbances. Magnetic resonance imaging (MRI) of patient 1 showed continuous high signal intensity extending from the midbrain to the entire spinal cord in the central part of the cord on T2-weighted imaging (T2WI). Spinal MRI of patient 2 revealed high signal intensity extending from Th2 to Th10 in the central part of the cord on T2WI. Both patients received high-dose methylprednisolone. A continuous lesion from the midbrain to the entire spinal cord as in patient 1 has not been previously reported. Patient 2 dramatically improved by infliximab therapy. The present cases suggest that NBD should be considered as a differential diagnosis in patients with LETM.

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Longitudinally extensive transverse myelitis suspected for isolated Neuro-Behçet: A diagnostic conundrum

Cited by 7 publications

References 23 publications

Differential diagnosis of neuromyelitis optica spectrum disorders

Differential diagnosis of neuromyelitis optica spectrum disorders

Comprehensive review of neuromyelitis optica and clinical characteristics of neuromyelitis optica patients in Puerto Rico

Two Cases of Probable Neuro-Behçet’s Disease with Longitudinally Extensive Transverse Myelitis

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