Recurrent longitudinal extensive transverse myelitis in a neuro-Behçet syndrome treated with infliximab

Abstract

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.     

Recurrent longitudinal extensive transverse myelitis in a neuro-Beh?et syndrome treated with infliximab

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.     

Acute transverse myelitis at the conus medullaris level after rabies vaccination in a patient with Behçet's disease

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.     

A case of neurogenic bladder due to neuro-Behçet disease

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.     

Neuro-Behçet's disease manifesting as a neoplasm-like lesion--case report

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.     

Protracted fever of unknown origin as the presenting symptom of Behçet's disease. Report of a case

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.     

Malignant lymphoma of the tonsil in a patient with Behçet's disease

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.     

Behçet's disease, associated with subarachnoidal heamorrhage due to intracranial aneurysm

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.     

Supraannular aortic replacement for severe valve detachment attributable to Behçet's disease

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.     

Vascular complications after surgical repair of aneurysms in Behçet's disease

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.     

Vascular manifestations of Behçet's disease. Eighteen cases among 140 patients

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.     

Evaluation of peripheral vascular system disorders in vascular symptom-free Behçet's disease

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.     

The value of endografts in the surgical management of arterial lesions secondary to Behçet disease

Background

This study summarizes our experience in the surgical management of arterial lesions secondary to Behçet disease (BD) and assesses the value of endografts.

Sigmoid Colon Perforation as an Unusual Complication of Behçet's Syndrome: Report of a Case

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.