Neuro-Behçet's Disease Onset in the Context of Tuberculous Meningoencephalitis: A Case Report

Abstract

Behçet's disease (BD) is a systemic vasculitis that frequently presents with a relapsing-remitting pattern. CNS involvement (Neuro-Behçet) is rare, affecting approximately 10% of patients. Its etiological mechanisms are not yet fully understood. The most commonly accepted hypothesis is that of a systemic inflammatory reaction triggered by an infectious agent or by an autoantigen, such as heat shock protein, in genetically predisposed individuals. Mycobacterium tuberculosis is known to be closely interconnected with BD, both affecting cell-mediated immunity to a certain extent and probably sharing a common genetic background. We present the case of a 34-year-old Caucasian woman who had been diagnosed with tuberculous meningitis 15 months prior, with significant neurological deficits and lesional burden on MRI with repeated relapses whenever treatment withdrawal was attempted. These relapses were initially considered as reactivation of tuberculous meningoencephalitis, and symptoms improved after a combination of antituberculous treatment and corticosteroid therapy. After the second relapse, the diagnosis was reconsidered, as new information emerged about oral and genital aphthous lesions, making us suspect a BD diagnosis. HLA B51 testing was positive, antituberculous treatment was stopped, and the patient was started on high doses of oral Cortisone and Azathioprine. Consequently, the evolution was favorable, with no further relapses and slow improvements in neurological deficits. To our knowledge, this is the first report of Neuro-Behçet's disease onset precipitated by tuberculous meningitis. We include a review of the available literature on this subject. Our case reinforces the fact that Mycobacterium tuberculosis infection can precipitate BD in genetically predisposed patients, and we recommend HLA B51 screening in patients with prolonged or relapsing meningoencephalitis, even if an infectious agent is apparently involved.

Keywords: Behçet’s disease; HLA B51 screening; Neuro-Behcet’s disease; systemic vasculitis; tuberculous meningoencephalitis.

Figure 1

The first cerebral MRI examination at the onset of symptoms. (A–D) FLAIR images reveal disseminated infra- and supratentorial hyperintense lesions (yellow arrows). (E–H) DWI images show corresponding restriction of diffusion (red arrows) for the majority of the lesions visible on FLAIR. (I–L) Contrast-enhanced corresponding sections without any pathological enhancement.

Figure 2

Corresponding FLAIR images at different moments in time. (A–C) The first MRI examination, the same examination that is presented in Figure 1, with lesions affecting the brain stem, left capsule, and bilateral periventricular white matter. (D–F) MRI scan at 12 months from onset showing favorable evolution, with significant regression of lesions. (G–I) MRI scan 9 months later, during a severe relapse, with new pontine lesions and bilateral thalamic lesions extending to the left basal ganglia and the right periventricular white matter. Significant atrophy is visible comparatively with the previous examinations. (J–L) MRI scan at 3 years from onset showing, once again, lesion regression under treatment. Marked progression of atrophy is visible. A—anterior and P—posterior.

Figure 3

(A) Genital scarring from previous ulcerations on the right labia minora (yellow arrows). (B) Facial acne lesions, which had a tendency to disappear under corticotherapy.