A case study of Neuro-psycho-Behcet's Syndrome presenting with psychotic attack
ABSTRACT Behçet's disease is a multisystem inflammatory disorder that presents with a classic triad of recurrent oral and genital ulcerations and uveitis with hypopyon. The initial symptom of Behçet's disease is neurological in only 3% of cases. Although Neuro-Behçet's Syndrome commonly presents with focal neurological symptoms, it is possible that psychiatric symptoms could be the first manifestation of the disease. To our knowledge, this is the first case report of Neuro-Behçet's Syndrome that presents with an acute psychotic attack.
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ABSTRACT: Behçet disease is a systemic vasculitis of unknown etiology that can affect the neurologic system. Neuro-Behçet disease is not well defined in children and adolescents, and the diagnosis is difficult to make in this population as they often present with insufficient symptoms to meet diagnostic criteria. Psychiatric symptoms as the initial manifestation of neuro-Behçet disease has rarely been reported. We describe a 17-year-old boy who presented with acute psychosis and was subsequently diagnosed with neuro-Behçet disease. A rare combination of both cerebral venous thrombosis and parenchymal central nervous system involvement was identified by neuroimaging. Although treatment guidelines for neuro-Behçet disease are limited, the patient made demonstrative clinical and radiographic improvement with a combination of corticosteroids, anticoagulation, and immunosuppressants, including a tumor necrosis factor-α (TNFα) blocking agent.Journal of child neurology 10/2013; 29(9). DOI:10.1177/0883073813504137 · 1.67 Impact Factor
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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.CNS Drugs 02/2015; 29(3). DOI:10.1007/s40263-015-0228-0 · 4.38 Impact Factor
Clinical Neurology and Neurosurgery 10/2014; 127. DOI:10.1016/j.clineuro.2014.09.010 · 1.25 Impact Factor