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Sequential PET studies in neuro-Behçet's syndrome.

A case of neuro-Behcet's syndrome is presented with sequential positron emission tomography (PET) studies. Regional cerebral blood flow (rCBF) and oxygen consumption (rCMRO2) were decreased in the brain lesion; however, on follow-up studies 3 months after steroid therapy rCBF and rCMRO2 had increased in the lesion, which demonstrated the reversibility of this disease. Such monitored improvement may accurately reflect the early stage of the disease and its response to steroid therapy.

Behcet Syndrome↗

Behçet's syndrome: a review for the genitourinary medicine physician.

Behcet's syndrome is a multisystem disorder due to vasculitis of unknown aetiology. It may present to genitourinary medicine physicians with genital ulceration. Careful history-taking and clinical examination should allow a correct diagnosis which may be confirmed by reference to the new diagnostic criteria including performing a pathergy test. Of particular importance is a prompt referral to an ophthalmologist for an ocular slit-lamp assessment as eye involvement can be severe and therapy needs to be started early in the course of the illness. The management of Behçet's syndrome may also involve rheumatologists, gastroenterologists, neurologists and immunologists. Close liaison between these clinicians may be necessary to provide optimum care for the patient with Behçet's syndrome.

Adult↗

Constitutional trisomy 8 mosaicism with myelodysplastic syndrome complicated by intestinal Behcet disease and antithrombin III deficiency.

Trisomy 8 is the most common acquired chromosomal abnormality associated with myeloid malignancy. As a constitutional trisomy 8 mosaicism (T8M), it exhibits an extremely variable phenotype. In addition, Behcet disease (BD) has been reported as an unusual complication of myelodysplastic syndrome (MDS). To our knowledge, 12 case reports of various hematologic malignancies in patients with T8M and 18 case reports of MDS with acquired trisomy 8 complicated by BD have been published to date. We report a case of constitutional T8M with MDS complicated by intestinal BD and antithrombin III deficiency.

Antithrombin III Deficiency↗

Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anticoagulant.

The lupus anticoagulant was found in the plasma of 31 of 60 patients with systemic lupus erythematosus and other connective tissue disorders (mixed connective tissue disease, systemic vasculitis, polyarteritis nodosa, primary sicca syndrome, discoid lupus, Behcet's syndrome, and systemic sclerosis). Strong associations were found with biological false positive seroreaction for syphilis and thrombocytopenia. The most striking association, however, was with the high prevalence of thrombosis. This tendency to thrombosis was independent of disease activity of systemic lupus erythematosus. The lupus anticoagulant appears to be a useful marker for a subset of patients with systemic lupus erythematosus at risk for the development of thromboembolic complications.

Adolescent↗

Unicentric Castleman's lymphadenopathy presenting with Behçet's syndrome: a case report.

We describe a male patient with Behcet's syndrome and Castleman's lymphadenopathy. He was affected by severe oral and genital ulcers, bilateral uveitis, necrotic purulent nodules on both hands. A CT scan of the mediastinum showed the presence of a mediastinal mass. On the basis of histological and immunohistological findings, a diagnosis of Castleman's disease, angiofollicular type, was formulated. The lymphadenopathy was unicentric. After the surgical excision of the mediastinal mass (January, 1989) until now, Behcet's syndrome is in complete remission with a low dosage of prednisone.

Adult↗

Audio-vestibular disturbances in Behcet's patients: report of 62 cases.

UNLABELLED: This study was carried out to determine the characteristics and incidence of hearing loss and vestibular disturbance in Behcet's syndrome with a large number of patients. Sixty-two patients with Behcet's syndrome were included in this study, 34 men and 28 women whose mean age was 33.7 (15-60). Sixty-two healthy normal control subjects (38 male and 24 female) were included. Patient and control groups were questioned about any history of audio-vestibular disturbance and underwent physical and ENT examination and the following audiologic tests: pure tone audiometric test (0.25, 0.5, 1, 2, 4, and 6 kHz), tympanogram, speech discrimination, short increment sensitivity index, tone-decay test, auditory brainstem response. Vestibular system was evaluated by videonistagmogram and caloric test. Cranial and brainstem magnetic resonance imagine (MRI) of patients who have vestibular disturbances were practiced to examine the central nervous system. Both the patient and the control groups were tested with the HLA-B51 antigen. Pure tone audiogram showed sensory-neural hearing loss (25 dB hearing level in at least two frequencies) in 20 of the 62 (32%) Behcet's patients while the control group were normal. There was a hearing loss involving high frequencies in the audiograms of Behcet's patients with hearing disturbances. The recruitment investigation tests and auditory brain stem response confirmed cochlear involvement in all 20 patients. Caloric stimulation tests revealed a normal vestibular function in all patient and control group. In electronystagmography, 21 (34%) patients had hypometric or hypermetric saccades and smooth pursuit tests showing that 4 (6%) patients had pathological changes while the control group was normal. HLA-B51 antigen was found positive in 15 of 20 Behcet's patient with hearing loss. CONCLUSION: (1) The hearing and vestibular disturbances in Behcet's syndrome is more prevalent than previously recognized; (2) Hearing loss in high frequencies in Behcet's patients is an indicator of cochlear involvement in this disease; (3) There is a higher prevalence of central vestibular syndrome in Behcet's patients than it was thought before; (4) HLA-B51 antigen may be able to be a prognostic factor for sensorineural hearing loss in Behcet's patients.

Adolescent↗

Behcet's disease.

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Behcet Syndrome↗

Behcet's disease.

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Behcet Syndrome↗