Myelopathy in Behçet's disease: The Bagel Sign

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Excerpt

Behçet's disease (BD) was first described with oro‐genital ulceration and uveitis known as the “triple‐symptom complex.”1 BD is a chronic, multisystemic, vascular‐inflammatory disease of unknown origin. According to the International Study Group (ISG) criteria, BD diagnosis requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test.2
Neurological involvement, referred to as neuro‐Behçet's Syndrome (NBS), occurs in approximately 5% to 10% of all patients with BD.3 Two major forms of NBS are identified: cerebral venous sinus thrombosis (VST) and parenchymal NBS (p‐NBS) (70–80% of all NBS).4
The p‐NBS predominantly affects the telencephalic/diencephalic junction and the brainstem, but less commonly the cerebrum and the spinal cord.5 Brainstem lesions are generally located within the brainstem with a tendency to extend to the diencephalon.6 Anatomically, the p‐NBS lesions seem to localize to areas with a relative paucity of radial and longitudinal anastomotic channels, akin to a venous watershed concept.6
Single or multiple spinal cord lesions with or without atrophy, and gadolinium (Gd) enhancement, while uncommon, have previously been reported in BD.7 Here, we report on a case series of myelopathy associated with BD and describe the distinctive magnetic resonance imaging (MRI) features.
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