Quantitative analysis of brainstem atrophy on magnetic resonance imaging in chronic progressive neuro-Behçet's disease

Kikuchi, Hirotoshi; Takayama, Maki; Hirohata, Shunsei

doi:10.1016/j.jns.2013.11.020

Cited by 20 publications

(11 citation statements)

References 24 publications

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“…Acute NBD is characterized by acute meningoencephalitis with focal lesions on MRI and increased neutrophil cell counts in the CSF. In contrast, chronic progressive NBD is characterized by intractable slowly progressive dementia, ataxia and dysarthria with persistent elevation of the CSF IL-6 and progressive brain stem atrophy on MRI [85]. Acute NBD responds well to high doses of corticosteroid, while chronic progressive NBD does not respond to steroid, but dose respond to low doses of methotrexate [86].…”

Section: Neuro-behçet Diseasementioning

confidence: 99%

Blood–brain barrier dysfunction in immuno-mediated neurological diseases

Shimizu

Nishihara

Kanda

2018

Immunological Medicine

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The blood-brain barrier (BBB) is the brain-specific endothelial cell barrier that is important for maintaining brain homeostasis and preventing the entry of toxic substances. Pathological BBB dysfunction is a critical step of the disease process in several immuno-mediated neurological diseases, including multiple sclerosis (MS), neuromyelitis optica (NMO), neuropsychiatric systemic lupus erythematosus (NPSLE) and neuro-Behçet diseases. The pathological findings from patients with secondary progressive (SP) MS, NMO and NPSLE showed leaky BBB in the active lesions. NMO is a disease with strong evidence of disease-specific and pathogenic autoantibodies (aquaporin 4 [AQP4] autoantibodies). In the development of NMO, circulating AQP4 autoantibodies need to pass through the BBB in order to reach AQP4 on the astrocyte endfeet. Strong evidence suggests that NPSLE is associated with the disruption of the BBB and NPSLE patients frequently have antibodies bound to endothelial cells in their sera. We recently identified two BBB-reactive autoantibodies in immuno-mediated neurological diseases: galectin-3 autoantibodies in SPMS and GRP78 autoantibodies in NMO. In the present review article, we describe the basic structure and cellular biology of the BBB, discuss recent insights regarding the pathophysiology of the BBB breakdown in the setting of immuno-mediated neurological diseases, and describe our recent findings of autoantibody-mediated BBB breakdown.

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Section: Neuro-behçet Diseasementioning

confidence: 99%

Blood–brain barrier dysfunction in immuno-mediated neurological diseases

Shimizu

Nishihara

Kanda

2018

Immunological Medicine

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“…148,149 Of note, as spinal cord involvement in NBD is considered a poor prognostic factor (60% of patients became dependent or died after a mean follow-up of 67 months), 150 NBD patients mimicking NMOSD need careful monitoring and intensive treatment.…”

Section: Differential Diagnosis Of Nmosdmentioning

confidence: 99%

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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“…in Turkey also showed similar CSF findings in A-P-NBD and CP-P-NBD, but not NP-NBD 23 .Furthermore, Kikuchi et al . showed that the progression rate of brain stem atrophy evaluated by MRI was closely correlated with CSF IL-6 elevation 38 . Interestingly, CSF IL-6 could be an activity marker for P-NBD patients even for those having normal range of CSF cell count and protein level 23 .…”

Section: Discussionmentioning

confidence: 91%

Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis

Ishido

Horita

Takeuchi

et al. 2017

Sci Rep

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Neuro-Behçet’s disease (NBD) is subcategorized into parenchymal-NBD (P-NBD) and non-parenchymal-NBD types. Recently, P-NBD has been further subdivided into acute P-NBD (A-P-NBD) and chronic progressive P-NBD (CP-P-NBD). Although an increasing number of studies have reported the various clinical features of A-P-NBD and CP-P-NBD over the last two decades, there was a considerable inconsistency. Two investigators systematically searched four electrical databases to detect studies that provided sufficient data to assess the specific characteristics of A-P-NBD and CP-P-NBD. All meta-analysis was carried out by employing the random-model generic inverse variance method. We included 11 reports consisted of 184 A-P-NBD patients and 114 CP-P-NBD patients. While fever (42% for A-P-NBD, 5% for CP-P-NBD, p < 0.001, I2 = 93%) was more frequently observed in A-P-NBD cases; sphincter disturbances (9%, 34%, P = 0.005, I2 = 87%), ataxia (16%, 57%, P < 0.001, I2 = 92%), dementia (7%, 61%, P < 0.001, I2 = 97%), confusion (5%, 18%, P = 0.04, I2 = 76%), brain stem atrophy on MRI (4%, 75%, P < 0.001, I2 = 98%), and abnormal MRI findings in cerebellum (7%, 54%, P = 0.02, I2 = 81%) were more common in CP-P-NBD. Cerebrospinal fluid cell count (94/mm3, 11/mm3, P = 0.009, I2 = 85%) was higher in A-P-NBD cases. We demonstrated that A-P-NBD and CP-P-NBD had clearly different clinical features and believe that these data will help future studies investigating P-NBD.

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Quantitative analysis of brainstem atrophy on magnetic resonance imaging in chronic progressive neuro-Behçet's disease

Cited by 20 publications

References 24 publications

Blood–brain barrier dysfunction in immuno-mediated neurological diseases

Blood–brain barrier dysfunction in immuno-mediated neurological diseases

Differential diagnosis of neuromyelitis optica spectrum disorders

Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis

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