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Epidemiology and Management of Neuropsychiatric Disorders in Behçet’s Syndrome

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Abstract

Behçet’s syndrome (BS) is a systemic, chronic, relapsing vasculitis, typically characterized by recurrent orogenital ulcers, ocular inflammation and skin manifestations; articular, vascular, gastroenteric and neurological involvement may also occur. Besides the other clinical features of BS, it seems relatively frequent that patients with BS develop a neurobehavioural syndrome, characterized by euphoria, bipolar disorders and paranoid attitudes, loss of insight/disinhibition, and indifference to their disease, defined as ‘neuro-psycho-BS’. To date, the pathogenetic mechanism underlying neuro-psycho-BS has not been determined. It may be secondary to organic neurological involvement, or it may be related to poor quality of life and the relapsing course of the disease. Another engaging theory suggests that it could be related to the frequent observation of psychiatric symptoms during relapses or, in some cases, in the phases preceding reactivation of the disease; these elements suggest that psychiatric disorders in BS could represent a crucial element, whether a psychiatric subset or a distinct clinical feature of the disease. Moreover, it has been reported that cognitive impairment in BS can be seen with or without central nervous system involvement. Globally, psychiatric symptoms have been described as being multifaceted, ranging from anxiety disorders to depressive–bipolar disorders or to psychotic ones. In addition, some psychological characteristics of BS patients seem to predispose them to maladaptive stress management, which may lead to stress-related disorders, including anxiety and depression. Therefore, the aims of this review are to explore the epidemiology of neuro-psycho-BS by evaluating the relationship between the stress system and the multifaceted psychiatric manifestations in BS, and to summarize the therapeutic strategy used.

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Acknowledgments

No funding was provided for this study.

Rosaria Talarico, Laura Palagini, Anna d’Ascanio, Elena Elefante, Claudia Ferrari, Chiara Stagnaro, Chiara Tani, Angelo Gemignani, Marta Mosca, Mauro Mauri and Stefano Bombardieri have no conflicts of interest to declare.

The authors would like to thank Ms Wendy Doherty for her invaluable help in revising the manuscript.

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Authors and Affiliations

  1. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, 56126, Pisa, Italy

    Rosaria Talarico, Anna d’Ascanio, Elena Elefante, Claudia Ferrari, Chiara Stagnaro, Chiara Tani, Stefano Bombardieri & Marta Mosca

  2. Psychiatry Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy

    Laura Palagini, Angelo Gemignani & Mauro Mauri

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  1. Rosaria Talarico
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Correspondence to Rosaria Talarico.

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Talarico, R., Palagini, L., d’Ascanio, A. et al. Epidemiology and Management of Neuropsychiatric Disorders in Behçet’s Syndrome. CNS Drugs 29, 189–196 (2015). https://doi.org/10.1007/s40263-015-0228-0

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