Original articleClinicopathologic features and outcomes of neuro-Behçet disease in Spain: A study of 20 patients
Introduction
Behçet disease (BD) is a chronic inflammatory systemic disease of unknown cause, characterized by recurrent oral and genital ulcers, and uveitis (1). Although this classic triad remains diagnostically important, it has become increasingly apparent that the inflammatory lesions can arise in almost any tissue, resulting in cutaneous, vascular, articular, gastrointestinal, renal, cardiopulmonary, or neurologic involvement [1], [2], [3], [4]. Classification criteria for this condition have been proposed by the International Study Group (ISG) for Behçet disease (5).
Neuro-Behçet disease (NBD) predominates in men and usually appears about 5 years after the onset of BD symptoms, although it may be the first manifestation or even precede other manifestations of the disease by several years [3], [6]. The frequency of NBD among BD patients varies widely from 1.3% to 59% [2], [7], [8], [9]. An autopsy series of 170 cases of BD showed pathological evidence of neurologic involvement in 20% of cases (10). Neurologic and major vessel involvement are the main causes of mortality in BD [2], [10].
Pathological studies investigating central nervous system (CNS) involvement in BD have mainly reported perivascular lymphocytic infiltration [10], [11], [12]. Because of the nonspecific pathology and lack of pathognomonic neuroimaging findings, the diagnosis of NBD requires a high index of clinical suspicion. Genetic and environmental factors are thought to modulate the expression of both BD and NBD. Hence, a familiarity with the clinical presentation of the disease in different geographic areas would be useful for diagnostic purposes (13).
The aim of this study was to describe the characteristics of NBD in a series of consecutively hospitalized adult patients with this condition in a tertiary care teaching hospital in Barcelona (Spain).
Section snippets
Materials and methods
This is a retrospective study covering a 20-year period (September 1988 to December 2008), including all adult patients (18 years and older) admitted to Hospital Universitari de Bellvitge (L'Hospitalet de Llobregat, Barcelona, Spain) with a diagnosis of NBD. Complete data from our NBD inpatient records were simultaneously evaluated by two internal medicine specialists (AR-M and AV) and two neurologists (SM-Y and AM-Y). We selected patients with a first neurologic attack who showed objective
Epidemiology
Twenty patients met the inclusion criteria for NBD (13 males and 7 females; M/F ratio, 1.8), representing 18% of patients with BD attended in our hospital over the study period. Sixteen patients were Caucasian and 4 were of African ethnic origin. On admission, BD had been established in only 7 of the 20 patients, with a mean age at diagnosis of 34 (19–54) years. The mean interval between the diagnoses of BD and NBD was 2.5 (0.2–4) years. Prior to admission for NDB symptoms, 12 of the remaining
Discussion
The percentage of BD patients in our series with neurologic involvement and the demographic characteristics of this population are similar to the data reported in earlier studies from different geographical areas [2], [10], [16]. However, the predominance of affected males, as was noted here, was not found in two recent European series [16], [17]. As in other studies from Western Europe, the percentage of NBD cases in patients without a prior diagnosis of BD was higher than the values recorded
Learning points
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In this Spanish series of adult patients hospitalized for NBD, fever, headache, motor weakness, and cranial nerve palsy were each present in approximately 60% of patients. There was a low prevalence of behavioral changes (5%), seizures (5%), and sphincter incontinence (0%). In contrast, meningism was present in a quarter of our patients, a finding reported less frequently in other studies.
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In our patients, all but 2 relapses occurred in the same location and patients presented symptoms similar
Acknowledgment
The authors thank Prof. Hasan Yazici, Division of Rheumatology, Cerrahpaşa Medical School, University of Istanbul, Istanbul, Turkey for his critical review of the manuscript.
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