Clinical and imaging features of spinal cord type of neuro Behçet disease

Liu, Hui-Miao; Dong, Ci; Zhang, Yongzhi; Tian, Ya-Yun; Chen, Hong‐Xu; Zhang, Sai; Li, Na; Gu, Ping

doi:10.1097/md.0000000000007958

Cited by 16 publications

(11 citation statements)

References 14 publications

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“…Neurological involvement in BD ranges from 2.2 to 49% and occurs within the first 10 years of disease ( 3 – 6 ). While brain injury is well documented with parenchymal and vascular involvement ( 7 ), there are also studies showing spinal cord and peripheral nerve participation ( 5 , 8 – 10 ). An axonal neuropathy has been demonstrated in nerve biopsy and electrophysiological studies in patients with BD ( 9 , 11 ) and a peripheral neuropathy has also been described in children with BD ( 12 ).…”

Section: Introductionmentioning

confidence: 99%

In Vivo Confocal Microscopic Evaluation of Corneal Nerve Fibers and Dendritic Cells in Patients With Behçet’s Disease

et al. 2018

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Central and peripheral nervous system involvement may occur during the course of Behçet’s disease (BD). In vivo corneal confocal microscopy (CCM) can detect corneal small fiber damage and immune cell density. The aim of this study was to assess central corneal sensitivity, corneal subepithelial nerve plexus morphology and dendritic cell (DC) density in patients with BD. Forty-nine consecutive patients with BD and 30 healthy control subjects were included in this cross-sectional study conducted at a tertiary referral university hospital. Central corneal sensitivity was measured using the contact corneal esthesiometer (Cochet-Bonnet; Luneau, France). The laser scanning CCM (Heidelberg, Germany) was used to quantify corneal nerve fiber density (NFD), nerve branch density (NBD), nerve fiber length (NFL), and DC density. There was a significant reduction in NFD (P = 0.001) and NFL (P = 0.031) and an increase in DC density (P = 0.038) in patients with BD compared to healthy controls, whereas corneal sensitivity (P = 0.066) and NBD (P = 0.067) did not differ significantly. There was no difference in corneal sensitivity, corneal nerve parameters, or DC density between BD patients with [n = 18 (36.7%)] and without a previous history of uveitis (P > 0.05 for all). Disease duration [median (IQR), 6.5 (4.0–14.5) years] correlated with corneal sensitivity (ρ = −0.463; P = 0.001) and NFD (ρ = −0.304; P = 0.034) and corneal sensitivity correlated with NFD (ρ = 0.411; P = 0.003) and NFL (ρ = 0.295; P = 0.039) in patients with BD. CCM demonstrates corneal sub-basal nerve fiber loss and increased DC density, providing a non-invasive ophthalmic means to identify peripheral neuropathy and inflammation in patients with BD.

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Section: Introductionmentioning

confidence: 99%

In Vivo Confocal Microscopic Evaluation of Corneal Nerve Fibers and Dendritic Cells in Patients With Behçet’s Disease

et al. 2018

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“…CLIPPERS and Behçet's disease commonly display an infratentorial distribution, while Behçet's lesions typically involve the mesodiencephalic or pontobulbar regions, basal ganglia, internal capsule and peduncles with sparing of the red nucleus [46], CLIPPERS lesions involve the pons and extend to the spinal cord, towards the basal ganglia and cerebellum [33]. Moreover, symmetrical T2 hyperintensities in the dentate nuclei and peridentate regions should raise the suspicion of ECD.…”

Section: Discussionmentioning

confidence: 99%

“…Typically, the lesions are clustered in the brainstem with a preference for the mesodiencephalic or pontobulbar regions (with typical sparing of the red nuclei), basal ganglia and cerebellar peduncles, and spinal cord involvement is very rare (Fig. 7) [46]. In later stages, brainstem atrophy is a characteristic feature [42].…”

Section: Behçet's Diseasementioning

confidence: 99%

Brain miliary enhancement

et al. 2020

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Purpose Miliary enhancement refers to the presence of multiple small, monomorphic, enhancing foci on T1-weighted postcontrast MRI images. In the absence of a clear clinical presentation, a broad differential diagnosis may result in invasive procedures and possibly brain biopsy for diagnostic purposes. Methods An extensive review of the literature is provided for diseases that may present with miliary enhancement on T1weighted brain MR images. Additional disease-specific findings, both clinical and radiological, are summarized and categorized by the presence or absence of perivascular space involvement. Results Miliary pattern of enhancement may be due to a variety of underlying causes, including inflammatory, infectious, nutritional or neoplastic processes. The recognition of disease spread along the perivascular spaces in addition to the detection or exclusion of disease-specific features on MRI images, such as leptomeningeal enhancement, presence of haemorrhagic lesions, spinal cord involvement and specific localisation or systemic involvement, allows to narrow the potential differential diagnoses. Conclusion A systematic approach to disease-specific findings from both clinical and radiological perspectives might facilitate diagnostic work-up, and recognition of disease spread along the perivascular spaces may help narrowing down differential diagnoses and may help to minimize the use of invasive diagnostic procedures.

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“…Parenchymal NBS (pNBS) mainly occur in the brain, but the spinal cord may rarely (in up to 10% of NBS in large series) also be affected in the form of a transverse myelitis (49). In the latter case, the leading clinical symptom is sensory disturbance, weakness, sphincter or sexual dysfunction, depending on the localization of the inflammatory lesions.…”

Section: Parenchymal Cns Manifestationsmentioning

confidence: 99%

Behçet‘s Syndrome Apart From the Triple Symptom Complex: Vascular, Neurologic, Gastrointestinal, and Musculoskeletal Manifestations. A Mini Review

Kötter

Lötscher

2021

Front. Med.

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Behçet‘s Syndrome (BS) is a variable vessel vasculitis according to the Chapel Hill Consensus Nomenclature (1) and may thus affect any organ, including major and minor arterial and venous vessels to a varying degree and with varying frequency. Although the main features of BS are recurrent oral and genital aphthous ulcers, cutaneous lesions, ocular inflammation and arthritis—major vessel and life—or organ threatening involvement of internal organs and the central and peripheral nervous system occur. In general, BS in Europe appears to form six phenotypes of clinical manifestations (2), which are (1) mucocutaneous only, (2) predominant arthritis/articular involvement, (3) vascular phenotype, (4) ocular manifestations, which are most likely associated with CNS manifestations and HLA-B51, (5) dominant parenchymal CNS manifestations (being associated with the ocular ones), and (6) gastrointestinal involvement. Mucocutaneous manifestations are present in almost all patients/all phenotypes. In the following review, we summarize the current knowledge concerning vascular, neurologic, gastrointestinal and musculoskeletal manifestations of the disease.

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Clinical and imaging features of spinal cord type of neuro Behçet disease

Cited by 16 publications

References 14 publications

In Vivo Confocal Microscopic Evaluation of Corneal Nerve Fibers and Dendritic Cells in Patients With Behçet’s Disease

In Vivo Confocal Microscopic Evaluation of Corneal Nerve Fibers and Dendritic Cells in Patients With Behçet’s Disease

Brain miliary enhancement

Behçet‘s Syndrome Apart From the Triple Symptom Complex: Vascular, Neurologic, Gastrointestinal, and Musculoskeletal Manifestations. A Mini Review

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