1/51. thalidomide responsiveness in an infant with Behcet's syndrome.BACKGROUND: Behcet's syndrome is a chronic relapsing vasculitis characterized by aphthous stomatitis, genital ulcerations, and uveitis. The disease is a multisystem illness, which may involve the skin, joints, gastrointestinal tract, blood vessels, central nervous system, and other organs. CASE STUDY: We report a case of an infant with features of Behcet's syndrome who did not respond to conventional immunosuppression with high-dose corticosteroids and cytotoxic agents. thalidomide, a potent immune response-modifying drug, was used with marked improvement and resolution of symptoms. CONCLUSION: This finding supports a trial of thalidomide treatment in patients with Behcet's syndrome who are unresponsive to other forms of immunosuppressive therapy or develop undesirable side effects.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/51. Demyelination of retinal myelinated nerve fibers in Behcet's disease.A 39 year-old Behcet's patient had demyelination of retinal myelinated nerve fibers after recurrent papillitis and vitritis. Oral prednisolone, cyclosporine, and later periocular corticosteroids and oral colchicine were used but demyelination continued over a 5 year-period. A pseudobulbar palsy with urinary incontinence and pyramidal tract signs developed and azathioprine and corticosteroids were used. Demyelination of retinal nerve fibers stopped and while treatment was underway, the central nervous system (CNS) signs were stable. While the ocular pathology of Behcet's can mirror the CNS signs, indeed optic nerve ischemic demyelination may signal the potential for CNS involvement; azathioprine with prednisone may be more effective in the long-term for optic nerve and CNS involvement than cyclosporine with prednisone.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/51. "Neuro-sweet disease": benign recurrent encephalitis with neutrophilic dermatosis.OBJECTIVE: To describe benign recurrent encephalitis in a case of sweet syndrome that also showed clinical features of Behcet disease. CASE REPORT: A 37-year-old Japanese man developed relapsing and remitting encephalitis and mucocutaneous symptoms mimicking Behcet disease. Magnetic resonance images showed at least 5 episodes of transient abnormal signal intensity in various cerebral regions over a period of 5 years. A skin biopsy specimen of the cutaneous edematous erythematous plaques revealed neutrophilic dermatitis compatible with sweet syndrome. HLA typing showed B54, which is frequent in sweet syndrome but rare in Behcet disease. Oral prednisolone therapy (10-60 mg/d) was remarkably effective for the encephalitis as well as for the mucocutaneous symptoms. CONCLUSION: We propose that there is an entity that is like Sweet disease, but with recurrent encephalitis characterized by an association with HLA-B54 and a high responsiveness to corticosteroid therapy, which we have tentatively named neuro-Sweet disease, that is distinct from the classic central nervous system involvement of Behcet disease.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
4/51. A case of coexisting Behcet's disease and ankylosing spondylitis.Behcet's disease (BD) is a chronic inflammatory condition involving several organs, such as skin, mucous membrane, eye, joint, intestine, lung and central nervous system. Ankylosing spondylitis (AS) is a prototype of seronegative spondyloarthropathy, and a chronic systemic inflammatory disorder of the axial skeleton, mainly affecting the sacroiliac joint and spine. In the latter, systemic complications may develop in addition to joint involvement. The coexistence of BD and AS has been rarely reported in the literature. The inclusion of BD among seronegative spondyloarthritides and whether sacroiliitis (SI) develops in BD are still being debated. We describe a 28-year-old man who has fulfilled the diagnostic criteria for BD and AS as well.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
5/51. Neuro-Behcet's syndrome in a patient not fulfilling criteria for Behcet's disease: clinical features and value of brain imaging.central nervous system involvement is rarely an initial presenting manifestation of Behcet's disease (BD). We report the case of a 33-year-old man with recurrent attacks of fever, oral mucosal ulcers, deep venous thrombosis, diplopia, vertigo and headache. Sequential brain magnetic resonance imaging (MRI) scans showed fluctuating lesions of the brain stem, mesencephalon and thalamus. F-18-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) revealed hypometabolism at the parieto-occipital cortex at both sides and the brain stem. Treatment with prednisone and cyclosporine A led to a complete remission and normalisation of MRI and FDG-PET lesions. The present case illustrates the difficulty in the differential diagnosis of early neuro-BD.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/51. Subpleural lung involvement in Behcet's disease: first localization of a systemic entity.Behcet's disease (BD) is a chronic multisystem vasculitis, affecting many organs and the vascular system, of unknown aetiology. Eyes, skin, joints, the oral cavity, the central nervous system, and, less frequently, heart, lung, kidney, the genital system and the gastrointestinal tract can be involved. Intrathoracic manifestations of BD consist mainly of thromboembolism of the superior vena cava and/or other mediastinal veins; aneurysms of the aorta and pulmonary arteries; pulmonary infarct and haemorrhage; pleural effusion; and, rarely, myocardial and/or hilar lymphoid involvement. In the present case, the patient presented with BD with an asymptomatic subpleural lung mass and bilateral pulmonary artery enlargement. The patient was treated with a combination of surgical and medical therapy with complete resolution of the lung involvement and without any parenchymal relapses after an 8-month follow-up.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/51. Neuro-Behcet's disease.Behcet's disease (BD) is a multifocal disorder with an immunogenetic basis, which persists over many years. Initial descriptions mentioned oral and genital ulcers with uveitis. Later a number of other manifestations were added, like skin, joint and neurological. The involvement of nervous system (Neuro-Behcet's) is reportedly uncommon. We hereby report four cases of Neuro-Behcet's, i.e.; two cases of strokes involving multiple areas of the central nervous system and two cases had features of benign intracranial hypertension. All cases had mucocutaneous lesions or other system involvement. Cases satisfied the international study group criteria for diagnosis of BD. All cases were pathergy test positive. In comparison with the literature from turkey and greece, which reports a high pathergy positivity, reports from india have shown only few cases to be positive. The prognosis of Neuro-Behcet used to be poor but has recently been improved with reduced mortality, although whether this can be attributed to treatment with steroids and/or cytotoxic agents remains uncertain.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
8/51. Atypical Behcet's syndrome in a patient with myelodysplastic syndrome.We report the case of a 67-year-old man with myelodysplastic syndrome (MDS), who presented with fever, painless penile and groin ulcers, as well as oral and esophageal ulcerations, all of which were exquisitely responsive to corticosteroids. Some cases of Behcet's syndrome and MDS have been reported in association with trisomy 8 and HLA B51, with the pathology varying from vasculitis to acute neutrophilic inflammation. Our patient with orogenital ulcers had neither trisomy 8 nor HLA B51. Also lacking were other features typical of Behcet's syndrome, such as uveitis, vasculitis, and central nervous system abnormalities. However, his response to corticosteroids on the initial presentation and subsequent episodes was dramatic. Esophageal ulcerations have not been described in Behcet's syndrome. In this respect, our patient was unique.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/51. Cerebral vein thrombosis in Behcet's disease.Behcet's disease is a chronic, relapsing multisystem disorder, and nervous system involvement is one of the serious manifestations. Neuro-Behcet is rarely reported in children and may present with a wide variety of symptoms because the entire neuraxis may be affected. A case of cerebral vein thrombosis secondary to Behcet's disease is presented. The patient has recovered without any visual loss and had no complaints at 1-year follow-up.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/51. osteonecrosis and bone infarction in association with Behcet's disease: report of two cases.We describe two cases with Behcet's disease (BD) developing osteonecrosis or bone infarction. One patient developed the extensive bone infarction of the left knee without the use of corticosteroids. The other patient had osteonecrosis at the right femoral head. He had had a past history of significant corticosteroid administration to treat several complications of BD such as central nervous system involvement, uveitis, gastrointestinal involvement, and pulmonary involvement. Anticardiolipin (aCL) antibodies were positive in these two patients. One was IgG type, and the other was IgM type. However, it remains unclear that there is relationship between the presence of aCL antibodies and occurrence of osteonecrosis.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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