Cognitive impairment in Behc ¸et’s disease patients without overt neurological involvement Roberto Monastero a , Cecilia Camarda a , Carmela Pipia a , Gianluca Lopez a , Lawrence K.C. Camarda a , Valentina Baiamonte a , Angelo Ferrante b , Giovanni Triolo b , Rosolino Camarda a, * a Department of Neurology and Rehabilitation, Institute of Neuropsychiatry, University of Palermo, Via La Loggia 1, 90129, Palermo, Italy b Department of Internal Medicine, Rheumatology and Clinical Immunology Unit, University of Palermo, Palermo, Italy Received 5 August 2003; received in revised form 20 February 2004; accepted 23 February 2004 Abstract We investigated the prevalence of cognitive impairment in patients with Behc ¸et’s disease (BD) without overt neurological involvement. The influence of disease duration, disease activity, prednisone dosage, and anxiety and depression levels was evaluated. Twenty-six consecutive BD outpatients and 26 healthy controls matched for age, education and sex completed a comprehensive neuropsychological battery including tests of memory, visuospatial and constructional abilities, language, attention and psychomotor speed, non-verbal reasoning and executive functioning. The Hamilton scales for anxiety and depression were administered. Disease activity was assessed using the Behc ¸et’s Disease Current Activity Form (BDCAF). Compared to controls, BD patients were significantly impaired on tasks evaluating long-term verbal and non-verbal memory, and visuospatial skills. In addition, BD patients were significantly more anxious and depressed than controls. Cognitive impairment was evident in 46.1% of BD patients compared with none of control subjects ( p < 0.0001), with memory representing the cognitive domain most affected. Both high disease activity (OR 1.3, 95% CI 1.0 – 1.5, p < 0.04) and high prednisone dosage (OR 1.3, 95% CI 1.0 – 1.7, p < 0.03) were independently associated with cognitive impairment in BD after adjustment for demographic variables. Cognitive impairment, involving mainly memory functions, occurs frequently in BD patients. It may occur independently of clinically overt neurological involvement, and is more common in patients with an active disease and in those receiving prednisone. D 2004 Elsevier B.V. All rights reserved. Keywords: Behc ¸et’s disease; Neuropsychology; Cognitive impairment; Corticosteroids; Disease activity 1. Introduction Behc ¸et’s disease (BD) is a chronic multisystem inflam- matory disorder of unknown aetiology, characterized by recurrent oral and genital ulceration, and ocular, arthritic, vascular and neurological involvement [1]. The etiopatho- genesis of the disease still remains unclear, although infec- tious, immunological and coagulation abnormalities have been taken into account [2,3]. A large percentage of patients with BD show severe neurological problems indicating central nervous system involvement [1]. Neurological manifestations of BD (name- ly Neuro-Behc ¸et syndrome) are very different, ranging from headache to severe life threatening conditions [4,5], and are considered an adverse prognostic factor [6]. Studies of cognition in BD are limited [7–10] and some of them have not included a standardized cognitive evalu- ation [7,9]. The few reports that have collected objective neuropsychological data in patients with BD suggest a characteristic pattern of neuropsychological deficits, involv- ing mainly memory, attention and executive functioning [8,10]. Further, to our knowledge, there are no reports of cognitive functioning in BD patients without neurological involvement. In addition, there seems to be no studies that examined the relationships between cognitive impairment and factors which may affect cognitive functioning such as 0022-510X/$ - see front matter D 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2004.02.021 * Corresponding author. Tel.: +39-91-655-51-12-20; fax: +39-91-655- 51-13. E-mail address: rcamarda@neuro.unipa.it (R. Camarda). www.elsevier.com/locate/jns Journal of the Neurological Sciences 220 (2004) 99 – 104