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Purpose. – To describe epidemiological and clinical characteristics of neurological involvement in Behc¸et’s disease (BD) and to determine a subgroup of patients at high risk for this complication.Patients and methods. – The medical notes of 105 patients with BD fulfilling the criteria of the international Study Group for Behc¸et’s disease were retrospectively reviewed. Patients were divided into two groups according to the presence (group 1) or not (group 2) of neurological and/or psychiatric involvement attributable to BD. The epidemiological, clinical and genetic (HLA B51 and MICA 6 frequency) features in the two groups were analysed and compared using the Kruskall-Wallis and the chi-square tests.Results. – Twenty-seven patients (25.7%) had clinical evidence of neurological involvement. They were 20 men and 7 women. The mean age at neurological onset was 34.26 ± 8.79 years. Nineteen patients (70.3%) had meningoparenchymal “MP” central nervous system involvement (brainstem: 9, hemispheric involvement: 6, spinal cord: 4, psychiatric involvement: 2, isolated pyramidal signs: 1, aseptic meningitis:1). Seven patients (25.9%) had cerebral large vessels involvement that is cerebral angio-Behc¸et “CAB” (intracranial hypertension: 5 cases due to cerebral venous thrombosis: 3 and pseudotumor cerebri: 2, cerebral haemorrhage: 1, cerebral arterial thrombosis: 1). One patient (3.7%) had both “MP” and “CAB” involvement. Headache was significantly more frequent in non-parenchymal patients. In group 1, complete recovery or improvement with mild neurological impairment was seen in 13 cases, improvement with severe disability in 3 cases, worsening in 1case, the course was stationary in 1 case and 3 patients died (11.2%). Arterial aneurysms were significantly more frequent in “CAB” subgroups than in subgroup 2.Conclusion. – Frequency of neurological involvements in BD was high in our study. Arterial aneurysms seem to be a risk factor to these complications. Cerebral angio-Behc¸et appears to be a protector factor against uveitis. [ABSTRACT FROM AUTHOR]

Abstract: We report a patient with Sjögren''s syndrome who presented with urticarial hypocomplementemic vasculitis. A 46-year-old female was admitted for assessment of ascitis. Clinical examination and computed tomographic scan disclosed evidence of multiple peripheral and intra abdominal lymph nodes. During her admission, she developed several bouts of acute angioedema and urticarial skin lesions. Minor salivary gland biopsy showed focal sialadenitis, stage IV of Chisholm. Schirmer''s test was positive. Laboratory examination found low levels of C1q and high levels of C1q antibodies. Therapy with prednisone and hydroxychloroquine was initiated. Six months later, the patient presented with lower limb oedema. Urinalysis showed proteinuria (1g/day) and renal biopsy revealed membranous nephropathy with favorable outcome with corticosteroids. [Copyright &y& Elsevier]