Objective: To retrospectively investigate the clinical data, radiological characteristics, treatment, and outcome of patients with parenchymal neuro-Behcet's disease (P-NBD) with particular emphasis on dizziness. Methods: This was a cross-sectional study of clinical data from 25 patients with a confirmed diagnosis of P-NBD who were admitted to the Department of Neurology of the First Medical Center of Chinese People's Liberation Army General Hospital between 2010 and 2022. The median age of the population was 37 years (range: 17-85 years). Clinical data were retrospectively analyzed, including gender, age of onset, disease duration, clinical manifestations, serum immune indicators, cerebrospinal fluid (CSF) routine biochemical and cytokine levels, cranial and spinal magnetic resonance imaging (MRI) findings, treatment, and outcome. Results: The majority of patients were male (16 cases; 64.0%), the mean age of onset was (28±14) (range: 4-58 years), and the disease course was acute or subacute. Fever was the most common clinical presentation, and the complaint of dizziness was not uncommon (8/25 patients). Analysis of serum immune indices, including complement (C3 and C4), erythrocyte sedimentation rate, interleukin-1 (IL-1), IL-6, IL-8 and tumor necrotic factor-alpha were abnormal in 80.0% of patients (20/25). Most of the 16/25 patients who underwent lumbar puncture tests had normal intracranial pressure and increased CSF white cell count and protein [median values were 44 (15-380) ×10⁶/L and 0.73 (0.49-2.81) g/L, respectively]. Of the five patients who underwent CSF cytokine tests, four patients had abnormal results; of these, an elevated level of IL-6 was most common, followed by IL-1 and IL-8. The most common site of involvement in cranial MRI was the brainstem and basal ganglia (60.0% respectively), followed by white matter (48.0%) and the cortex (44.0%). Nine cases (36.0%) showed lesions with enhancement and six cases (24.0%) showed mass-like lesions. Three patients (12.0%) patients had lesions in the spinal cord, most frequently in the thoracic cord. All patients received immunological intervention therapy; during follow up, the majority had a favorable outcome. Conclusions: P-NBD is an autoimmune disease with multiple system involvement and diverse clinical manifestations. The symptom of dizziness is not uncommon and can be easily ignored. Early treatment with immunotherapy is important and can improve the outcome of these patients.
Humans ; Male ; Female ; Child, Preschool ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Aged, 80 and over ; Behcet Syndrome/diagnosis* ; Interleukin-6 ; Retrospective Studies ; Cross-Sectional Studies ; Interleukin-8 ; Magnetic Resonance Imaging ; Neurology

Behçet’s disease (BD) is a multisystemic, chronic autoimmune inflammatory vasculitic disease with an unknown etiology. Although the literature reports that vascular involvement occurs in 7% to 38% of all BD cases, the arteries are rarely involved; however, arterial involvement is usually associated with significant mortality and morbidity. We report the case of a young female patient who presented to the emergency department with severe abdominal pain and a history of weight loss. The patient was evaluated using computed tomography angiography, which revealed a ruptured suprarenal aortic pseudoaneurysm with occlusion of both the superior mesenteric and celiac arteries. Urgent surgery was performed with aortic repair with an interposition graft and superior mesenteric artery embolectomy. The patient’s clinical history and radiological imaging findings were strongly suggestive of the diagnosis of BD with vascular involvement.
Abdominal Pain ; Aneurysm, False ; Angiography ; Arteries ; Behcet Syndrome ; Celiac Artery ; Diagnosis ; Embolectomy ; Emergency Service, Hospital ; Female ; Humans ; Mesenteric Artery, Superior ; Mortality ; Transplants ; Vasculitis ; Weight Loss

Behçet's disease is a systemic vasculitis of unknown etiology characterized by recurrent oral and genital ulcers and uveitis. The vascular involvement of Behçet's disease affects arteries, veins, and blood vessels of all sizes, and it can include venous or arterial thrombosis and arterial aneurysms. There are only a few reports of an aortic aneurysm invading a vertebral body in a patient with Behçet's disease. Here, we report the case of a 45-year-old man who was initially diagnosed with vertebral invasion of a mycotic aneurysm. He underwent vascular surgery and received empirical antibiotics, but all cultures were negative. However, he had persistent, recurrent deep vein thrombosis and elevated inflammatory markers. After reviewing the pathology, a final diagnosis of Behçet's disease was made. He was successfully treated with corticosteroids. This report presents a rare case of Behçet's disease mimicking vertebral invasion of a mycotic aneurysm.
Adrenal Cortex Hormones ; Aneurysm ; Aneurysm, Infected* ; Anti-Bacterial Agents ; Aortic Aneurysm ; Arteries ; Behcet Syndrome ; Blood Vessels ; Diagnosis ; Humans ; Middle Aged ; Osteomyelitis ; Pathology ; Systemic Vasculitis ; Thrombosis ; Ulcer ; Uveitis ; Veins ; Venous Thrombosis

Intestinal Behçet's disease (BD), generally accepted as a type of inflammatory bowel disease (IBD), could be diagnosed when patients with BD have objectively documented gastrointestinal symptoms and intestinal ulcerations. Similar to IBD, intestinal BD has an unpredictable disease course with relapse and remission and is often related to a poor prognosis. However, there is no single gold standard for assessment of the disease activity of intestinal BD, and its diagnosis and management depend heavily on expert opinions. The Korean IBD Study Group recently developed novel diagnostic criteria based on colonoscopy findings and clinical manifestations using a modified Delphi process to overcome drawbacks of previously used consensus for the diagnosis of intestinal BD. In addition, the recently developed disease activity index for intestinal BD, consisting of a relatively simple 8-point index, could also help in determining treatment strategies and monitoring therapeutic responses. In this review, the progress in the diagnosis and disease activity measurement of intestinal BD will be discussed.
Behcet Syndrome ; Colonoscopy ; Consensus ; Diagnosis* ; Expert Testimony ; Humans ; Inflammatory Bowel Diseases ; Prognosis ; Recurrence ; Ulcer

Subarachnoid hemorrhage due to a solitary spinal aneurysm is extremely rare, and diagnosis and treatment are challenging. We report a rare case of a ruptured radiculomedullary artery aneurysm in a patient with Behçet disease. A 49-year-old man presented with severe lower abdominal and leg pain. Magnetic resonance imaging was performed and an enhanced intradural-extramedullary lesion at the T12 spinal level with subarachnoid hemorrhage was identified. Diagnostic spinal angiography was performed to evaluate the vascular lesion, and a radiculomedullary artery aneurysm at the T12 level was identified. We performed surgical resection of the aneurysm and a good neurological outcome was obtained.
Aneurysm* ; Angiography ; Arteries* ; Behcet Syndrome ; Diagnosis ; Humans ; Leg ; Magnetic Resonance Imaging ; Middle Aged ; Subarachnoid Hemorrhage

BACKGROUND/AIMS: We prepared a cross-cultural adaptation of the Behcet's Syndrome Activity Scale (BSAS) and evaluated its reliability and validity in Korea. METHODS: Fifty patients with Behcet's disease (BD) who attended the Rheumatology Clinic of Gachon University Gil Medical Center were included in this study. The first BSAS questionnaire was administered at each clinic visit, and the second questionnaire was completed at home within 24 hours of the visit. A Behcet's Disease Current Activity Form (BDCAF) and a Behcet's Disease Quality of Life (BDQOL) form were also given to patients. The test-retest reliability was analyzed by intraclass correlation coefficients (ICC). To assess the validity, the total BSAS score was compared with the BDCAF score, the patient/physician global assessment, and the BDQOL by Spearman rank correlation. RESULTS: Twelve males and 38 females were enrolled. The mean age was 48.5 years and the mean disease duration was 6.7 years. Thirty-eight patients (76.0%) returned the questionnaire by mail. For the test-retest reliability, the two assessments were significantly correlated on all 10 items of the BSAS questionnaire (p < 0.05) and the total BSAS score (ICC, 0.925; p < 0.001). The total BSAS score was statistically correlated with the BDQOL, BDCAF, and patient/physician global assessment (p < 0.01). CONCLUSIONS: The Korean version of BSAS is a reliable and valid instrument to measure BD activity.
Academic Medical Centers ; Adult ; Behcet Syndrome/*diagnosis/physiopathology/psychology ; Cost of Illness ; Cultural Characteristics ; Female ; Humans ; Male ; Middle Aged ; *Patient Reported Outcome Measures ; Predictive Value of Tests ; Quality of Life ; Reproducibility of Results ; Republic of Korea ; Severity of Illness Index

Behcet's disease (BD) is a multi-systemic inflammatory disorder of an unknown etiology and shows a chronic recurrent clinical course. When the disease involves the alimentary tract, it is called intestinal BD because of its clinical importance. Intestinal BD is more frequently reported in East Asian countries than in Western or Middle Eastern countries. While any part of the gastrointestinal tract can be involved, the most common location of intestinal BD is the ileocecal area. A few, large, deep ulcerations with discrete border are characteristic endoscopic findings of intestinal BD. Currently, there is no single gold standard test or pathognomonic finding of intestinal BD. However, recently developed novel diagnostic criteria and a disease activity index have helped in assessing intestinal BD. As intestinal BD shares a lot of characteristics with inflammatory bowel disease, including genetic background, clinical manifestations, and therapeutic strategies, distinguishing between the two diseases in clinical practice is quite difficult. However, biologic agents such as anti-tumor necrosis factor alpha antibody shows a considerable efficacy similar to inflammatory bowel disease cases. It is important to distinguish and treat those two disease entities separately from the standpoint of precise medicine. Clinicians should require comprehensive knowledge regarding the similarities and differences between intestinal BD and inflammatory bowel disease for making an accurate clinical decision.
Behcet Syndrome/*diagnosis/therapy ; Diagnosis, Differential ; Gastrointestinal Diseases/*diagnosis/therapy ; Humans ; Inflammatory Bowel Diseases ; Male ; Middle Aged ; Systemic Vasculitis/*diagnosis/etiology ; Treatment Outcome ; Tumor Necrosis Factor-alpha/therapeutic use

Behçet's disease is a systemic chronic disease that occurs in tissues such as eyes, joints, organs and nerves, and it has been noted that symptoms may be observed in a variety of tissues. In previous studies, reports of blepharoptosis observed in patients with Behçet's disease have been rare. We would like to report a case where a patient among those who visited our hospital with blepharoptosis had a history of Behçet's disease. This patient had been diagnosed with Behçet's disease, and complained of bilateral blepharoptosis even at the time of diagnosis. He complained of dysfunctions in vision and hearing, and upon eye examination, an eye movement disorder was found in his left eye. From the symptoms, neuro-Behçet's disease was diagnosed. The oculomotor and levator palpebrae superioris muscles are both controlled by cranial nerve III, which may suggest that Behçet's disease in this patient occurred in cranial nerve III. The patient received an oral steroid, and the symptoms have improved without surgery. Since we could identify the correlation between Behçet's disease and blepharoptosis, we considered that sharing this case and its outcome would be helpful for plastic surgeons who treat eyelids.
Behcet Syndrome ; Blepharoptosis* ; Chronic Disease ; Diagnosis ; Eyelids ; Hearing ; Humans ; Joints ; Muscles ; Ocular Motility Disorders ; Oculomotor Nerve ; Plastics ; Surgeons

BACKGROUNDThe distinction between intestinal Behηet's disease (BD) and Crohn's disease (CD) is always challenging due to many overlapping clinical features. We conducted a retrospective study to reveal valuable strategies for the differential diagnosis between intestinal BD and CD in Chinese patients based on their clinical and colonoscopic features.

METHODSThirty-five intestinal BD patients and 106 CD patients hospitalized from January 1983 to January 2010, who had ulcerative lesions in the terminal ileum or colon under colonoscopy and no history of gastrointestinal operation except appendectomy before admission, were enrolled. Univariate and multivariate logistic regression analyses were conducted to find discriminating predictors among demographic data, clinical manifestations, and colonoscopic findings.

RESULTSBased on univariate analysis, massive gastrointestinal hemorrhage, fever, and extraintestinal systemic manifestations were more common in intestinal BD patients (P = 0.022, 0.048 and 0.001, respectively), while diarrhea, intestinal obstruction, and perianal lesions were more common in CD patients (P = 0.002, 0.010, and 0.027 respectively). Based on colonoscopy, focal involvement, ileocecal valve deformity, solitary ulcers, large ulcers (ulcer size > 2 cm), and circumferential ulcers were more common in intestinal BD patients (P = 0.003, 0.003, 0.014, 0,013, and 0.003, respectively), while segmental involvement, longitudinal ulcers, a cobblestone or nodular appearance, and pseudo-polyps were more common in CD patients (P = 0.003, 0.008, 0.023, and 0.002, respectively). Based on multivariate logistic regression analysis, diarrhea, extraintestinal manifestations, ulcer distribution, size, and type, and pseudo-polyps were independent discriminating predictors between the two groups (P = 0.048, 0.008, 0.006, 0.021, 0.002, and 0.041, respectively). The discriminating algorithm composed of the above independent predictors had the highest area under the curve of 0.987 for distinguishing between the two diseases.

CONCLUSIONSExtraintestinal systemic manifestations and the characteristic colonoscopic features, such as ulcer distribution, size and type, helped to distinguish intestinal BD from CD.

Adolescent ; Adult ; Asian Continental Ancestry Group ; Behcet Syndrome ; diagnosis ; Colonoscopy ; Crohn Disease ; diagnosis ; Diagnosis, Differential ; Female ; Humans ; Male ; Young Adult

No abstract available.
Adult ; Behcet Syndrome/*complications/diagnosis ; Follow-Up Studies ; Humans ; Male ; Optic Disk/*pathology ; Optic Neuritis/diagnosis/*etiology ; Time Factors ; Visual Acuity