1/103. "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 = cerebral (Clic here for more details about this article) |
2/103. A bad eye and a sore lip.A 48-year-old woman developed painful visual loss in the left eye, meningismus, and painful oral ulcers. magnetic resonance imaging of the brain with gadolinium demonstrated enhancement of the left optic nerve. Lumbar puncture showed a lymphocytic pleocytosis, and a biopsy specimen of one of the oral ulcerations was consistent with Behcet's disease. epidemiologic factors and diagnostic criteria for Behcet's disease are discussed.- - - - - - - - - - ranking = 0.028641487668297keywords = brain (Clic here for more details about this article) |
3/103. Central diabetes insipidus with Behcet's disease.We encountered a case of Behcet's disease complicated with central diabetes insipidus. A hypothalamopituitary dysfunction is rare in Behcet's disease; only three cases of this association have been reported in the literature. magnetic resonance imaging of the brain showed a dilatation of the left Sylvian vein and thickening of the pituitary stalk, which suggested intracranial vasculitic processes and lymphocytic infundibuloneurohypophysitis. The possible relationship of this disease combination is discussed as a form of autoimmune disease.- - - - - - - - - - ranking = 0.028641487668297keywords = brain (Clic here for more details about this article) |
4/103. spinal cord Neurobehcet's disease detected on magnetic resonance imaging.Imaging studies in Neurobehcet's disease have to date focused on the brain, with only four previous case reports of documented spinal cord involvement on MRI being published. A fifth case is documented here, together with a review of the literature.- - - - - - - - - - ranking = 0.028641487668297keywords = brain (Clic here for more details about this article) |
5/103. 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 = 0.20049041367808keywords = brain (Clic here for more details about this article) |
6/103. 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 = 0.29570749122181keywords = haemorrhage (Clic here for more details about this article) |
7/103. Cerebral aneurysms associated with Behcet's disease: a case report.Cerebral aneurysms in Behcet's disease are very rare. The role of vasculitis in the aetiology of these aneurysms has not been clarified. A 57 year old man with Behcet's disease is described, who had a subarachnoid hemorrhage due to rupture of a peripheral middle cerebral artery aneurysm. He underwent a successful aneurysmal clipping. Three years later he had seizures and was found to have a new aneurysm on the contralateral peripheral middle cerebral artery as well as some radiological features of vasculitis. After 3 months of steroid therapy, the aneurysm disappeared. Although surgical treatment is the first choice for ruptured aneurysms, steroid therapy may be effective for unruptured small aneurysms in patients with Behcet's disease.- - - - - - - - - - ranking = 2keywords = cerebral (Clic here for more details about this article) |
8/103. Widespread nocardiosis in two patients with Behcet's disease.nocardia spp., a group of gram-positive variably acid-fast aerobic bacteria, are opportunistic pathogens in immunocompromised hosts. We here-within describe 2 cases of widespread nocardiosis in patients with Behcet's disease. In addition to endogen endophthalmitis in case 1, both cases developed lung, brain and skin involvement. Despite brain involvement, the prognosis was good, although vision was completely lost in case 1, which was directly attributable to a delay in both diagnosis and treatment.- - - - - - - - - - ranking = 0.057282975336594keywords = brain (Clic here for more details about this article) |
9/103. Unusual presentation of neonatal Behcets disease.Intrauterine growth retardation and neonatal transient mucocutaneous lesions ("transient behcet syndrome") have been reported in pregnancies complicated by Behcets disease (BD). Neonatal neurological manifestations have not been reported in such pregnancies. Vascular and neurological involvement is known to worsen the prognosis in adults with BD. The clinical course and outcome of a 34-weeks' gestation neonate born to a mother with BD is reported. Progressive recovery from minimal respiratory distress syndrome was followed by catastrophic presentation on 6th day of life with generalized seizures. Cranial ultrasound revealed multiple hyperechoic lesions in the frontal, parietal, and periventricular regions with a few surrounded by a ring of reduced echogenicity suggesting haemorrhage into ischemic areas. death occurred after withdrawal of life support on Day 9, after extensive discussions with parents in view of the progressive deterioration in the neonates' general condition and the cranial ultrasound findings. Strong family history of BD, clinical course, and laboratory results (no evidence of disseminated intravascular coagulation, normal levels of protein C and S, absence of factor v Leiden and anticardiolipin antibodies) suggested neurological manifestations of BD as the most probable diagnosis.- - - - - - - - - - ranking = 0.29570749122181keywords = haemorrhage (Clic here for more details about this article) |
10/103. 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 = cerebral (Clic here for more details about this article) |
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