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1.
《The journal of spinal cord medicine》2013,36(1):111-114
AbstractBackgroundSpinal cord involvement is not common, but can be seen in neuro-Behçet's syndrome (NBS). The major site of involvement is the cervical spinal cord with the myelitis-like inflammatory lesions continuing more than two segments, and extending to the brainstem.CaseA 30-year-old male patient who has been followed with a diagnosis of Behçet's syndrome admitted to our neurology department clinically and radiologically suggestive of recurrent and extensive longitudinal myelitis. His anti-aquaporine antibody was negative. Because of insufficient effect of azathioprine, cyclophosphamide, and corticosteroids, infliximab was started. His clinical and radiological status is stationary for 3 years under infliximab treatment.DiscussionMyelitis such as that occurring in our patient may have a similar presentation like neuromyelitis optica (NMO), which should therefore be included in differential diagnosis. Myelitis observed in both NMO and NBS shows spinal cord lesions longer than three or more vertebrae. Anti-aquaporine antibody must be evaluated in all patients presenting with longitudinal myelitis. Anti-tumor necrosis factor agent infliximab might be an alternative treatment in severe form of NBS such as myelitis.ConclusionIn our case, successful treatment of recurrent and extensive longitudinal transverse myelitis in NBS with infliximab was demonstrated. 相似文献
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U?ur Uyguno?lu Maarya Pasha Sabahattin Saip Aksel Siva 《The journal of spinal cord medicine》2015,38(1):111-114
Background
Spinal cord involvement is not common, but can be seen in neuro-Behçet''s syndrome (NBS). The major site of involvement is the cervical spinal cord with the myelitis-like inflammatory lesions continuing more than two segments, and extending to the brainstem.Case
A 30-year-old male patient who has been followed with a diagnosis of Behçet''s syndrome admitted to our neurology department clinically and radiologically suggestive of recurrent and extensive longitudinal myelitis. His anti-aquaporine antibody was negative. Because of insufficient effect of azathioprine, cyclophosphamide, and corticosteroids, infliximab was started. His clinical and radiological status is stationary for 3 years under infliximab treatment.Discussion
Myelitis such as that occurring in our patient may have a similar presentation like neuromyelitis optica (NMO), which should therefore be included in differential diagnosis. Myelitis observed in both NMO and NBS shows spinal cord lesions longer than three or more vertebrae. Anti-aquaporine antibody must be evaluated in all patients presenting with longitudinal myelitis. Anti-tumor necrosis factor agent infliximab might be an alternative treatment in severe form of NBS such as myelitis.Conclusion
In our case, successful treatment of recurrent and extensive longitudinal transverse myelitis in NBS with infliximab was demonstrated. 相似文献3.
Acute transverse myelitis at the conus medullaris level after rabies vaccination in a patient with Behçet's disease
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Bir LS Eşmeli FO Cenikli U Erdoğan C Değirmenci E 《The journal of spinal cord medicine》2007,30(3):294-296
Case report:
A 25-year-old man with Behçet''s disease was admitted because of weakness of the lower limbs and difficulty in urination. He had received a rabies vaccination 2 months previous because he had been bitten by a dog.Findings:
Clinical and laboratory findings supported acute transverse myelitis. A hyperintense lesion and expansion at the level of conus medullaris was detected on spinal magnetic resonance imaging.Conclusion:
Although neurologic involvement is one of the main causes of mortality and morbidity in Behçet''s disease, the factors that aggravate the involvement of the nervous system are still unclear. Vaccination may have been the factor that had activated autoimmune mechanisms in this case. To our knowledge, involvement of the conus medullaris in Behçet''s disease after rabies vaccination has not been reported. 相似文献4.
Iida S Taniguchi N Nishihara M Miyata M Kaneko S Yachiku S 《Hinyokika kiyo. Acta urologica Japonica》2000,46(10):727-729
Beh?et disease is a systemic vasculitis of unknown cause with variable clinical features. The central nervous system may also be involved in about 10% of the patients with Beh?et disease. Half of them show marked central nervous system symptoms and are diagnosed as neuro-Beh?et disease. Voiding symptom is seen in about 5% of the patients with neuro-Beh?et disease. We report a case of neuropathic vesico-urethral dysfunction in a 39-year-old man with neuro-Beh?et disease. Its radiological and urodynamic features and treatment are also presented with some discussion. 相似文献
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Imoto H Nishizaki T Nogami K Sakamoto K Nomura S Akimura T Matsunaga T Suzuki M 《Neurologia medico-chirurgica》2002,42(9):406-409
A 50-year-old man presented with neuro-Beh?et's disease (NBD) manifesting as a large neoplasm-like lesion affecting the brainstem, basal ganglia, and white matter of the cerebral hemisphere. He had no history of disease except for psychoneurosis. On admission, neurological examination found left hemiparesis and dysarthria. Magnetic resonance (MR) imaging showed multiple small ring-like enhancement in the basal ganglia, brainstem, and deep white matter. Biopsy of the mass was performed. Histological examination revealed invasion of inflammatory cells in the white matter, especially around the blood vessels. After the brain biopsy, the patient developed oral aphthae, genital ulcers, and skin eruptions, which are indicative of Beh?et's disease. MR imaging after three courses of steroid pulse therapy revealed that the edematous lesion had become smaller with minimum midline shift. NBD should be considered in the differential diagnosis of lesions with multiple ring-like enhancement extending from the basal ganglia to the brainstem, because dermatological manifestations are sometimes obscured during periods of remission. 相似文献
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Niamane R Karim Moudden M Zyani M Hda A 《Joint, bone, spine : revue du rhumatisme》2005,72(2):175-176
We report a case of Beh?et's disease that presented as protracted fever of unknown origin. The diagnosis was established when a thromboembolic event and ora3l aphthous ulcers occurred simultaneously. Antibiotics had no effect on the fever, which resolved when glucocorticoid and anticoagulant therapy was given. Among causes of protracted fever of unknown origin, Beh?et's disease is exceedingly rare but should be considered together with the other vasculitides. Above all, the presence of a fever should prompt a search for a thromboembolic complication. 相似文献
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Several connective tissue diseases such as rheumatoid arthritis and polymyositis are associated with cancer. In contrast, cancer is rarely reported in patients with Behçet disease. We report a case of lymphoma during the course of Behçet disease. Etiopathogenic factors are discussed.A 46-year-old man with a 14-year history of Behçet disease was diagnosed with non-Hodgkin malignant lymphoma of the right tonsil. He met international criteria for Behçet disease, which manifested as refractory oral ulcers requiring dapsone treatment. He achieved a complete remission of the lymphoma after three chemotherapy courses and local radiation therapy (45 Gy). At last follow-up 4 years later, he was still in complete remission. 相似文献
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Beh?et's disease is an unusual medical condition in central Europe and North America, however more common in Turkey and Japan. It was originally described in Turkey, characterized by recurrent oral ulcers, genital ulcers and also uveitis. A variety of vascular lesions such as venous occlusions, arterial aneurysms and varices account for the high rate of morbidity and mortality with this disease. Arterial aneurysms most commonly occur in the abdominal aorta, femoral arteries and in the pulmonary arteries. To our knowledge there have been seventeen documented reports of patient's with Beh?et's disease combined with aneurysms of cerebral arteries. We describe a patient with Beh?et's disease and subarachnoid haemorrhage due to a ruptured cerebral aneurysm. 相似文献
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The occurrence of aortic regurgitation (AR) attributable to Beh?et's disease is rare, but it brings extraordinary complexity to surgical intervention due to the high risk of postoperative morbidity as the result of valve dehiscence or pseudoaneurysms caused by fragile aortic structures and inflamed tissue. The high rate of prosthetic valve detachment after aortic valve replacement is one of the most serious consequences of aortic regurgitation in Beh?et's disease, which results in severe aortic annular destruction, presenting a big challenge to cardiac surgeons. Under this condition, a conventional surgical technique is necessary to be modified for improving postoperative prognosis of patients. In this paper, we described the surgical treatment of 5 patients with Beh?et's aortitis and report their long-term outcomes. 相似文献
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Schneider F Gouny P Van Laere O Duedal V Nussaume O 《The Journal of cardiovascular surgery》2002,43(4):501-505
The poor prognosis of vasculo Behcet's disease is often due to postoperative vascular complications (false aneurysm and graft occlusion). We report a case of an abdominal aortic aneurysm associated with an aneurysm of the left common femoral artery in a 23-year-old Portuguese man. The primary treatment was surgical (aneurysmectomy and prosthetic revascularization). The early occlusion of the left femoral revascularized artery was treated surgically with a new bypass. The occlusion of the right limb of the aortoiliac graft was asymptomatic and was not treated. Two months after admission to our hospital, the stenosis of the infrarenal aorta successfully treated by angioplasty via the occluded right limb of the graft. The patient was followed up for 18 months. He could only walk a short distance and had rest pain in the left foot. Magnetic resonance angiography showed a false aneurysm of the infrarenal aorta, and an occlusion of the remaining left limb of the aortoiliac graft. The endovascular treatment performed does not avoid the need for surgical treatment, because occlusion and false aneurysm may occur after dilatation. The endovascular approach can also be used during a sudden inflammatory surge, and makes it possible to wait for a quiescent period when surgery can be performed. 相似文献
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OBJECTIVES: To describe the features, prognosis, and treatment of vascular involvement in Beh?et's disease (BD). PATIENTS: Among 140 patients with BD seen at the H?tel-Dieu Hospital in Beirut between 1980 and 2000, 18 (13%) had vascular involvement and were included in this retrospective study. All these patients fulfilled International Study Group criteria for BD. RESULTS: Men with BD were more likely to have vascular involvement (13/77, 17%) than women (5/63, 8%) (P = 0.12) and were younger at diagnosis of vascular disease (32 +/- 7 vs. 36 +/- 7.5 years; P < 0.01). Many patients had vascular disease at more than one site: 17 had thrombophlebitis, 10 had arterial thromboses, and one had an aneurysm. Thrombophlebitis was more common in men (82% vs. 18%; P < 0.03) and arterial occlusion in women (70% vs. 30%; P > 0.05). Caval thrombosis and arterial occlusions were the most serious complications. Combined treatment with glucocorticoids, anticoagulants, and immunosuppressants was effective in superior vena cava syndrome and extracranial arterial occlusion. CONCLUSION: Vascular manifestations of BD are common in Lebanon, particularly venous lesions. Aneurysms are seen less often than arterial occlusions. Medical treatment may be sufficient in superior vena cava syndrome and arterial occlusion. 相似文献
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《Cardiovascular surgery (London, England)》1996,4(3):381-383
Vascular involvement in Behçet's disease has been reported in 7–40% of patients in the literature and nearly 8% of these will have serious vascular complications during follow-up. So far, little information is available on the extent of vascular involvement in patients with Behçet's disease who are free of vascular symptoms. The study comprised 34 individuals with Behçet's disease who were free of vascular symptoms and 47 healthy volunteers. Both groups underwent non-invasive peripheral vascular examination consisting of Doppler, photoplethysmography, and segmental pressure measurements and determination of ankle—brachial pressure and femoro-brachial pressure indices. Venous insufficiency was detected in 22 of 68 lower extremities examined in the Behçet's disease group and in ten of 94 in the controls (P < 0.001). The number of extremities with an ankle-brachial pressure index of less than 1.00 for both the anterior and posterior tibial arteries, was significantly higher in the Behçet's disease group (P < 0.001). The femoro-brachial pressure index was significantly higher in the controls than in the Behçet's disease group (1.32 versus 1.23 for the right leg, P < 0.05; 1.31 versus 1.22 for the left leg, P < 0.03). Vascular symptom-free patients with Behçet's disease seem to have a high incidence of peripheral vascular involvement compared with healthy controls. 相似文献
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Henghui Yin Songqi Li Mian Wang Zuojun Hu Jinsong Wang Chen Yao Guangqi Chang Shenming Wang 《Journal of vascular surgery》2017,65(2):471-477
Background
This study summarizes our experience in the surgical management of arterial lesions secondary to Behçet disease (BD) and assesses the value of endografts.Methods
Data from BD patients with arterial lesions managed surgically in our center from January 1998 to December 2015 were studied retrospectively. Surgical procedures, graft selection, graft-related complications, and retreatments were analyzed.Results
We recruited 33 patients (29 men and 4 women; male-to-female ratio, 7.25:1) with an average age of 36.7 years (range, 25-51 years). The arterial lesions included 27 aneurysms in 24 patients and nine stenotic or occlusive lesions in nine patients. Immunosuppressive therapy was administered routinely preoperatively and postoperatively as recommended. Altogether, 15 great saphenous veins (GSVs), 8 synthetic grafts, and 13 endografts were used in 36 primary procedures. The mean follow-up duration was 3.8 ± 2.9 years. Graft-related pseudoaneurysm was seen in three GSVs (20%) and in three synthetic grafts (38%) at the anastomosis, but not in endograft implantations (log-rank, P = .171). Graft occlusions were observed in 1 GSV (7%), 2 synthetic (25%), and 2 endografts (15%; log-rank, P = .881). Graft infection occurred in one synthetic graft (13%) and in one endograft (8%) but not in the GSVs (log-rank, P = .689). Graft-related artery rupture occurred in only one endograft (8%). Two patients died, giving a mortality rate of 6.1%.Conclusions
In the surgical management of arterial lesions secondary to BD, endografts were superior to GSV and synthetic grafts in decreasing anastomotic pseudoaneurysm. However, improvements are needed to enhance the long-term patency and reduce infections. 相似文献19.
A 47-year-old man with long-standing Beh?et's syndrome presented with an acute abdomen, and was found to have perforation of the sigmoid colon. Laparotomy revealed gangrenous changes in the sigmoid colon and perforation in the center of the affected segment. This is a very rare complication of Beh?et's disease, and we report this case to stress the importance of performing careful abdominal examination while evaluating patients with Beh?et's disease. 相似文献