Characteristics of neurological manifestations of Behçet's disease: A retrospective monocentric study in Tunisia

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Abstract

Objective

The aim of the present study was to analyze demographic, clinical and genetic features of Behçet's disease patients with neurological involvement through a monocentric 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.

Methods

Four hundred and thirty Behçet's disease (BD) patients were retrospectively studied. Diagnosis of BD was made according to the international study group for Behçet's disease criteria. Patients with neurological findings suggestive of involvement of the nervous system by BD were further studied according to clinical examination, laboratory tests and neuroradiological investigations.

Results

Neurological involvement was observed in 121 patients (28.1%). The mean age at neuro-Behçet's disease (NBD) onset was 29.7 years. Average disease duration of BD before neurological manifestations onset was 6.4 years. Male to female ratio was 1.8. Of the 121 NBD patients, parenchymal involvement occurred in 74 patients (61%). Among them 26 (21.4%) presented with brainstem involvement, 24 (19.8%) with hemispheric involvement and 2 (1.6%) with spinal cord involvement. Non-parenchymal NBD occurred in 47 patients (39%). Involvement of the main vascular structures (Vasculo-NBD) was the most common non-parenchymal NBD lesion found in 35 patients (28.9%) consisting of cerebral vein thrombosis (CVT) in 24 cases and cerebral arterial thrombosis in 11 cases. Forty-nine (40.5%) patients with NBD have been followed-up for a median of 3 years (range 1–19 years). Forty-one of them recovered well without significant residual disability, 5 patients made no improvement and are left with severe neurological impairments and 3 died. Male gender and CNS parenchymal lesions occurrence were significantly associated with a poorer prognosis.

Conclusion

Clinical and epidemiological features of NBD are various. In our Tunisian 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.

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.

Section snippets

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.

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