Neurological Manifestation of Behcet s Disease in a Tunisian Cohort

Research Article

Austin Crit Care Case Rep. 2022; 6(1): 1037.

Neurological Manifestation of Behçet’s Disease in a Tunisian Cohort

Douma B¹*, Bedoui I¹, Mariem E¹, Mrissa NF², Derbali H¹ and Mrissa R¹

¹Department of Neurology, Military Hospital of Tunis, Tunis, Tunisia

²Laboratory of Hematology, Molecular Biology Unit (UR17DN06), Military Hospital of Tunis, Tunis, Tunisia

*Corresponding author: Bissene Douma, Department of Neurology, Military Hospital of Tunis, Tunis, Tunisia

Received: January 11, 2022; Accepted: February 09, 2022; Published: February 16, 2022


Behçet’s disease (BD) is a multi-systemic vasculitis characterized by attacks of acute inflammation, which can affect multiple area of the body. Neurological involvement known as “neuro-Behcet’s” (NB) is rare and is one of the main causes of long-term morbidity and mortality. The aim of our work was to study the epidemiological, clinical, paraclinical and therapeutic characteristics of patients with BD with neurological involvement. We conducted a retrospective study of NB patients in the Neurology and Internal Medicine departments of the Military Instruction Hospital of Tunis. We collected 35 patients from a population of 150 patients with BD. Neurological manifestations had inaugurated the BD in 55% of the cases. Ninety-four percent of patients had central nervous system involvement and 6% had peripheral polyradiculoneuritis. Parenchymal involvement occurred in 85% of cases and non-parenchymal NB in 3% of cases. Brain magnetic resonance imaging (MRI) showed predominant demyelinating lesions in periventricular and brain stem in 15 patients, a pseudotumor appearance in 3 patients, and vascular lesions in 2 patients. All patients received corticosteroid and 30 patients were treated with immunosuppressive therapy. Three patients had received an immunomodulatory treatment by anti- TNF alpha. The outcome was unfavorable in patients with diffuse disease, brain stem damage, spinal cord injury, polyradiculoneuropathy and mixed impairment. Neurological manifestations of BD are various and are typically of poor outcomes with a high mortality and heavy sequelae. The prognosis depends on parenchymal involvement and time to initiate treatment.

Keywords: Behçet’s disease; Neuro-Behcet’s; Central nervous system; Parenchymal involvement


Behçet’s disease (BD) is a multisystem inflammatory disorder of unclear etiology [1] affecting young people of male sex [2]. It is characterized by recurrent oral and genital ulcers, skin lesions and uveitis. Other manifestations include arthritis, gastrointestinal ulcerations, thrombophlebitis and central nervous system disease [1].

Clinical diagnosis is based on the criteria of the international Behçet’s disease study group of 1990 [3] and those of the International Criteria for Behçet’s disease (ICBD) [4].

In the absence of sufficient clinical criteria and additional paraclinical examinations, diagnostic confirmation remains difficult.

Neurological manifestation in BD varies from 5, 4 to 59% of cases [5] and they are characterized by their clinical polymorphism, affecting the central nervous system (CNS) in 98% of cases [6].

Neurological disorders can precede other systemic manifestations in more than 3% of cases [4], causing diagnostic and therapeutic delay and constitute an element of the severity of the disease [7,8].

Considering that Tunisia is an endemic country of this pathology [9], a study of the neurological manifestations would be interesting.

The objective of our work was to analyze epidemiological, clinical and paraclinical characteristics of Tunisian BD patients with neurological involvement based on the new classifications.

Patients and Methods

We performed a retrospective study of 150 patients diagnosed as BD monitored in the Neurology and Internal Medicine departments of the Military Instruction Hospital of Tunis over a period of 23 years, from January 1997 to December 2019.

Diagnosis of BD was made according to the criteria of the international study group (ISG) of 1990 and those of the International Criteria for Behçet’s disease (ICBD).

Neuro-Behcet’s (NB) diagnosis was retained according to international consensus recommendation (ICR) criteria.

Patients without any evidence of objective neurological involvement including those with isolated headache, those who did not show any abnormality upon neurological examination, cerebrospinal fluid (CSF) analysis or neuro-radiological examinations, were excluded, as well patients affected by diseases known to induce neurological involvement.

Epidemiological parameters (age, gender), family history of BD, clinical presentation, diagnostic criteria, investigations, complications and treatment were analyzed.

The onset of NB was defined as the time when the first neurological symptoms attributed to the disease occurred.

Neurological symptoms were subdivided into central and peripheral involvement.

Magnetic resonance imaging (MRI) was performed on all of our patients to explore different regions of the brain, spinal cord and cerebral vessels. A lumbar puncture was done with chemical and cytological study of the CSF. Electro-neuro-myogram (ENMG) was performed in front of signs of peripheral involvement. The typing of the specificity of HLA-B51 was carried out by the serological technique of micro-lymphotoxicity.

The treatment modalities consisted of immunosuppressive agents in combination with oral high doses or intravenous pulses of glucocorticosteroids.

The course of treatment was described using the modified Rankin score (MRs).

Our data were analyzed using the Statistical Package for the Social Sciences (SPSS version 20). Results in all groups and subgroups were compared by Pearson’s chi-squared test. A value of p <0.05 was regarded as statistically significant.


We collected 35 patients of NB from a population of 150 patients with BD. They were 30 males and 5 females (M/F ratio = 6). The mean age at diagnosis was de 34±1, 92 (range 27-71 years). Average diagnostic duration was 2.9 years with extremes ranging from one week to 13 years. Two of our patients had a family history of BD.

The distribution of the extra-neurological manifestations: cutaneous-mucous, articular, ocular and vascular is summarized in Table 1.