Humans ; Heart Ventricles ; Behcet Syndrome/complications* ; Heart ; Heart Diseases ; Ventricular Dysfunction, Left

A 40-year-old male patient was referred to our department with complains of recurrent oral ulcer for more than 20 years and vulvar ulcer for more than 10 years. He presented with a 3-month history of right external ophthalmoplegia. More than 10 days ago, the patient received ganglioside infusion. And one week ago, he developed numbness and pain of his lambs, and progressive myasthenia, accompanied by right blepharoptosis and dysuria. On exam, motor strength was graded 0/5 in the lower and the upper extremities. Deep tendon reflexes were diminished in extremities. His admission medical examination: hemoglobin (HGB), white cell and platelet counts were normal. C-reactive protein (CRP) was negative. Erythrocyte sedimentation rate (ESR) 53 mm/h. Antinuclear antibody (ANA), anti-dsDNA antibody, anti-Smith antibody, anti-cardiolipin antibody and human leucocyte antigen B51 were all within normal range. The etiological tests of influenza A pathogen, influenza B pathogen, parainfluenza virus, enterovirus and parvovirus were all negative. He tested positive for serum anti-GM1 IgG. Cerebrospinal fluid had a normal white cell count, an elevated protein content. Gram staining, culture and PCR detection for varicella-zoster virus, cytomegalovirus and herpes simplex virus were all negative. Antibodies associated with autoimmune encephalitis and paraneoplastic syndrome were negative in cerebrospinal fluid. Electromyography and nerve conduction studies showed a severe axonal damage affecting motor nerves. No obvious abnormalities were observed in his magnetic resonance imaging of brain and cavernous sinus. The patient was diagnosed with Behcet syndrome complicated with acute Guillain-Barré syndrome. He received intravenous methylprednisolone, intravenous immunoglobulin (IVIg) therapy, plasma exchange and rituximab treatment. After treatment, the patient's muscle strength of limbs was restored to grade 1, blepharoptosis and pain disappeared. The nervous system involvement of Behcet syndrome is relatively rare, especially combined with Guillain-Barré syndrome, which is easy to cause misdiagnosis. The treatment of Behcet syndrome complicated with acute Guillain-Barré syndrome includes the treatment of primary disease, plasma exchange and IVIg therapy. In addition, supportive treatment is very important for such patients. The focus of treatment is to avoid respiratory insufficiency, prevent deep vein thrombosis, monitor cardiac function and hemodynamics. Pain-relieving, physical exercise and psychological support are often under-recognized. The rehabilitation treatment is very important to improve the prognosis and quality of life of patients. What we need to learn is that when the symptoms and signs of the nervous system are difficult to be explained by neuro-Behcet syndrome alone, we should be alert to the possibility of other nervous system diseases.
Adult ; Animals ; Behcet Syndrome/complications* ; Guillain-Barre Syndrome ; Humans ; Immunoglobulins, Intravenous ; Male ; Methylprednisolone ; Quality of Life ; Sheep

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

PURPOSE: Behcet's disease (BD) is a chronic inflammatory disease characterized by orogenital ulcers, skin and ocular lesions, in addition to articular, vascular, and neurologic symptoms. Carpal tunnel syndrome (CTS), can also occur in BD patients secondary to inflammation in the connective tissues, vessels, and tendons, as well as nerve involvement in BD itself. However, reports of patients who have CTS in BD are rare. The aim of this study was to evaluate the clinical characteristics of CTS in BD patients. MATERIALS AND METHODS: Retrospective analysis of the medical records of 1750 BD patients, and 14 (0.8%) BD patients who were diagnosed with CTS was performed at the BD Specialty Clinic of Severance Hospital. Patient demographics, disease activity/severity for both diseases, and the clinical characteristics of CTS in BD were recorded and analyzed. RESULTS: All 14 BD patients with CTS were women. Twelve patients (85.7%) were diagnosed with active BD. The CTS was mild in 8 patients (57.2%), moderate in 3 patients (21.4%), and severe in 3 patients (21.4%). Ten patients (71.4%) had BD prior to the diagnosis of CTS, and these 10 patients all had active BD. CONCLUSION: CTS can occur as a result of the inflammation associated with BD and can also be the presenting symptom of nerve involvement in BD. CTS can also develop as the initial symptom of BD. Therefore, a higher degree of suspicion should be maintained for CTS in patients with BD and vice versa; however, the exact relationship is uncertain.
Adult ; Age Distribution ; Aged ; Behcet Syndrome/complications/diagnosis/*epidemiology ; Carpal Tunnel Syndrome/diagnosis/*epidemiology ; Female ; Humans ; Inflammation ; Male ; Middle Aged ; Retrospective Studies ; Sex Distribution

No abstract available.
Aneurysm, Dissecting/diagnosis/*etiology/physiopathology/therapy ; Anticoagulants/therapeutic use ; Aortic Aneurysm/diagnosis/*etiology/physiopathology/therapy ; Behcet Syndrome/*complications/diagnosis/drug therapy ; Cerebral Infarction/diagnosis/etiology ; Diffusion Magnetic Resonance Imaging ; Echocardiography, Doppler, Color ; Electrocardiography ; Hemodynamics ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; *Sinus of Valsalva/physiopathology/ultrasonography ; Thrombosis/diagnosis/drug therapy/*etiology/physiopathology ; Ventricular Septal Rupture/diagnosis/*etiology/physiopathology/therapy

No abstract available.
Behcet Syndrome/*complications/diagnosis/drug therapy ; Drug Therapy, Combination ; Hemianopsia/diagnosis/*etiology/physiopathology ; Humans ; Immunosuppressive Agents/administration & dosage ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pulse Therapy, Drug ; Steroids/administration & dosage ; Treatment Outcome ; Visual Field Tests ; Visual Fields

Behcet's disease (BD) involves multisystem vasculitis of unknown origin. Ocular manifestations of BD mostly include bilateral panuveitis and retinal vasculitis, which are very challenging to treat. Interferon alfa-2a (IFN) has been recently introduced for treating refractory Behcet uveitis, mainly in Germany and Turkey. Nonetheless, there is so far no consensus about the ideal treatment regimen of IFN for Behcet uveitis. We report our experience of IFN treatment in five Korean BD patients with refractory uveitis. All patients complained of oral ulcers; one patient had a positive pathergy test and 2 showed the presence of HLA-B51. Immunosuppressive agents used prior to IFN treatment included cyclosporine and methotrexate. The IFN treatment was commenced with a dose of 6-9 MIU/day for 7 days, adjusted according to individual ocular manifestations, tapered down to 3 MIU three times in a week, and then discontinued. All patients showed positive response to IFN treatment; 50% of them showed complete response without additional major ocular inflammation during the follow-up period. Other BD symptoms also improved after IFN treatment in most cases. After treatment, the relapse rate and the required dose of oral corticosteroid were decreased in most cases, showing a significant steroid-sparing effect. However, the visual acuity was not improved in most cases due to irreversible macular sequelae. Despite the small sample size of this study, we suggest that, in Korean patients, IFN is an effective treatment modality for BD uveitis as was observed in German and Turkish patients.
Adult ; Behcet Syndrome/complications/diagnosis/*drug therapy ; Chronic Disease ; Cyclosporine/therapeutic use ; Female ; Humans ; Immunosuppressive Agents/administration & dosage ; Interferon-alpha/*therapeutic use ; Male ; Recombinant Proteins/therapeutic use ; Recurrence ; Remission Induction ; Treatment Outcome ; Turkey ; Uveitis/diagnosis/*drug therapy/etiology ; Visual Acuity

BACKGROUND/AIMS: Anemia in patients with inflammatory bowel disease significantly affects the quality of life. The aim of this study was to investigate the frequency of and risk factors for anemia and to describe the management of anemia in patients with intestinal Behcet's disease (BD) in actual clinical practice. METHODS: We included 64 patients with intestinal BD who visited the outpatient clinic of a tertiary referral center in June 2011 and had available laboratory data for the subsequent 6 months. RESULTS: Anemia was detected in 26 patients (40.6%). After 6 months, anemia was still present in 14 of these patients (53.8%). The cause of anemia was investigated in eight patients (30.8%), and oral iron supplementation was prescribed to four patients (15.4%). Of these four patients, two (50%) recovered completely within 6 months. Anemia was associated with a high Disease Activity Index for Intestinal Behcet's Disease (DAIBD, p=0.024), erythrocyte sedimentation rate (p=0.003), and C-reactive protein (p=0.049) in univariate analysis. In multivariate analysis, the factor predictive for anemia in patients with intestinal BD was a higher DAIBD (> or =40; odds ratio, 4.08; 95% confidence interval, 1.21 to 13.71; p=0.023). CONCLUSIONS: Although anemia is common in intestinal BD patients, its clinical importance is overlooked in daily practice. Moderate to severe disease activity is predictive of anemia.
Adult ; Anemia/drug therapy/epidemiology/*etiology ; Behcet Syndrome/blood/*complications/pathology ; Blood Sedimentation ; C-Reactive Protein/analysis ; Dietary Supplements ; Disease Management ; Female ; Humans ; Intestinal Diseases/blood/*complications/pathology ; Iron/therapeutic use ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Predictive Value of Tests ; Risk Factors ; Severity of Illness Index ; Trace Elements/therapeutic use

No abstract available.
Adult ; Behcet Syndrome/*complications/therapy ; Colitis/*etiology/therapy ; Female ; *Hematopoietic Stem Cell Transplantation ; Humans ; Myelodysplastic Syndromes/*complications/therapy ; Transplantation, Homologous

Adult ; Behcet Syndrome ; complications ; Cochlear Implantation ; Cochlear Implants ; Deafness ; etiology ; rehabilitation ; Humans ; Male ; Treatment Outcome