Özgüler, Yeşim

Abstract Objective To examine the influence of demographics on organ damage in a broad, multiethnic cohort of patients with Behçet’s syndrome (BS). Methods In this cross-sectional ancillary analysis of the PROBE project, the investigated demographic variables were sex, age, education level and geographic area of residence. Damage was measured by the BS Overall Damage Index (BODI). Multivariate linear (β) and logistic (adjusted odds ratio [adjOR]) regression analyses examined associations between demographics and the extent and prevalence of damage. Results A total of 970 patients were enrolled. The median (interquartile range) age was 40 (31–50) years; 56.5% were males; 21.4% had a low level of education. The median BODI score was 1 (0–3), with 65.6% of patients having a BODI ≥1. Males had higher damage (β 0.103) and a higher prevalence of total (adjOR 1.7, per 10 years), ocular (adjOR 1.6), and vascular (adjOR 2.1) damage. Age was associated with greater damage (β 0.104), and a higher prevalence of overall (adjOR 1.4), neuropsychiatric (adjOR 1.2) and miscellaneous (adjOR 2.0) damage. Low education was associated with a greater frequency of overall (adjOR 1.9) and ocular (adjOR 1.6) damage. North African patients experienced greater damage than South European (β 0.400) and Middle Eastern (β 0.314) patients, as well as a higher risk of overall damage (adjOR 13.9 and 10.7, respectively) across all organ domains, except for the reproductive and gastrointestinal systems. Conclusions This study demonstrates how the extent, prevalence and characteristics of damage in BS vary with demographic factors, underscoring their importance in research and personalized management.

ObjectiveTo describe the clinical and distinctive imaging features of myelopathy associated with Behçet's disease (BD).MethodsWe evaluated the records of patients meeting the following criteria: (1) fulfillment of the International Study Group criteria for BD; (2) clinically suggestive of myelopathy; (3) simultaneous spinal cord and brain magnetic resonance images (MRIs) within 1 month of acute worsening of myelopathy; and (4) follow‐up duration ≥ 1 year after initial MRI evaluation. Patients not fulfilling all inclusion criteria and having MRIs with poor quality or missing sequences were excluded.ResultsIn 11 patients (9 men, 2 women), we studied 14 MRIs during distinct myelopathy episodes and nine follow‐up MRIs. Two distinct MRI patterns of spinal cord involvement were described according to T2‐weighted (T2W) axial images: (1) “Bagel Sign” pattern: a central lesion with hypointense core and hyperintense rim with or without contrast enhancement; and (2) “Motor Neuron” pattern: a symmetric involvement of the anterior horn cells. Bagel Sign was present in 13 of 14 myelopathy episodes whereas Motor Neuron pattern was observed in 1 of 14 MRIs. Of the 13 MRIs with Bagel Sign long myelopathy (n = 9), both long and short myelopathy (n = 2) and short myelopathy (n = 2) was observed. All patterns cleared with some residual lesions after steroid use and immunomodulation with associated clinical recovery in patients.InterpretationThe signal characteristics of the Bagel Sign potentially represent venous engorgement and/or acute blood products within the spinal cord. To our knowledge, Bagel Sign has not been observed in other forms of longitudinal myelopathy outside of BD, including neuromyelitis optica. Ann Neurol 2017;82:288–298