Characteristics of neurological manifestations of Behçet's disease: A retrospective monocentric study in Tunisia
Introduction
Behçet's disease (BD) is a multisystem inflammatory disorder of unclear aetiology characterized by recurrent oral and genital ulcers, skin lesions and uveitis. Other manifestations include arthritis, a positive pathergy test, thrombophlebitis, central nervous system disease and gastrointestinal ulcerations.
Neurological involvement in BD was first reported in 1941 and the first autopsy case was described in 1944 in Berlin [1]. Since then, several singles cases and series have been reported with high variability of prevalence (1.3–59%) depending on diagnostic criteria, different methodology from one study to other and ethnic populations [2], [3].
The fact that there are ethnic and regional differences in BD presentation is well known. Not only the frequency but also the type of organ lesions seems to differ between regions. These differences could be related to genetic and environmental influences. Perhaps this is why some prefer to see in BD a syndrome rather than a disease [4].
Therefore, our aim was to analyze demographic, clinical and genetic features of Tunisian BD patients with neurological involvement through a retrospective study of a homogenous group of hospitalized patients observed in the same department; and to compare them with those of other ethnic and geographic groups.
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Patients and methods
We performed a retrospective review of the records of 430 cases diagnosed as BD hospitalized in the department of internal medicine, La Rabta University Hospital in Tunis, Tunisia (a tertiary referral centre), over a 20-years period (1990–2009). Diagnosis of BD was made according to the criteria of the international study group (ISG) for BD. Records were analyzed for age, gender, presentation, diagnostic criteria, investigations, complications and treatment. The onset of BD was defined as the
Results
Four hundred and thirty records of BD patients were retrospectively analyzed. The patients originated from all parts of Tunisia. They were 295 males and 135 females (M/F ratio = 2.2). The mean age at BD onset was 29.12 ± 10.24 years (range 6–58 years) and the mean age at diagnosis was 33.94 ± 10.27 (range 0–40 years). The frequencies of clinical manifestations in the patients with BD are given in Table 1.
Neurological involvement was observed in 121 patients (28.1%). They were 78 men and 43 women with
Discussion
The prevalence of neurological manifestations in BD is highly variable (2–44%) depending on diagnostic criteria and ethnic populations, this prevalence could be also biased by the speciality of the studies departments. We aimed to describe in this retrospective survey the frequency, the type and the relationship to systemic disease of the neurological features in a cohort of BD patients of Tunisian origin.
The frequency of NBD in our series was high (28.1%) which is consistent with the results
Conclusion
In summary, in our Tunisian monocentric cohort of NBD patients the main characteristic features were male predominance, a relatively high prevalence of CVT, a low prevalence of intra-cranial hypertension and a significant lower frequency of HLA-B51 haplotype.
Although our study was limited by being retrospective, it's the largest cohort of NBD patients in our country showing the full spectrum of the neurological involvements in BD.
References (27)
- et al.
Neurologic manifestations of Behcet's disease: analysis of a series of 27 patients
Revue de Medecine Interne
(2002) - et al.
Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature
Revista de Neurologia
(2006) - et al.
Neuro-Behçet's disease: epidemiology, clinical characteristics, and management
Lancet Neurology
(2009) - et al.
Neurological involvement in Behçet's disease
Revue de Medecine Interne
(2009) - et al.
Neuro-Behcet's disease in Iraq: a study of 40 patients
Journal of the Neurological Sciences
(1999) - et al.
Clinicopathologic features and outcomes of neuro-Behçet disease in Spain: a study of 20 patients
European Journal of Internal Medicine
(2010) Behcet's syndrome with involvement of central nervous system
Archiv für Dermatologie und Syphilis
(1944)- et al.
Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease
International Journal of Dermatology
(2003) - et al.
Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations
Journal of Neurology, Neurosurgery and Psychiatry
(1998) - et al.
Behçet syndrome
Current Opinion in Rheumatology
(2004)
Behçet's disease in Tunisia. Demographic clinical and genetic aspects in 260 patients
Clinical and Experimental Rheumatology
Behçet's disease: features of neurological involvement in a dedicated centre in Italy
Clinical and Experimental Rheumatology
Epidemiology and clinical characteristics of Behçet's disease in the US: a population-based study
Arthritis and Rheumatism
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