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Meningeal involvement in Behçet's disease: MRI
1Department of Radiology, Hospital Duran i Reynals (Ciutat Sanitària i Universitària de Bellvitge), L'Hospitalet de Llobregat, Spain.
Abstract:
Behçet's disease is a multisystem disease that involves the central nervous system up to half of cases. Presentation with neurologic symptoms occurs in 5% of cases and cerebral venous thrombosis is one of its major manifestations. A feature not previously reported is progressive meningeal thickening with involvement of both optic nerves. We report a patient with cerebral venous thrombosis, meningeal thickening and contrast enhancement on MRI. This patient had two other unusual features: positive antineutrophil cytoplasmic antibodies and later development of central diabetes insipidus.
Insights
Behçet's disease can affect the central nervous system, leading to rare symptoms like progressive meningeal thickening and optic nerve involvement. This case highlights unusual MRI findings and associated conditions in a patient with this multisystem disorder.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Behçet's disease is a multisystem inflammatory disorder affecting various organs, including the central nervous system (CNS).
- Neurologic involvement occurs in approximately 5% of cases, with cerebral venous thrombosis being a significant manifestation.
- Progressive meningeal thickening is an uncommon presentation of neuro-Behçet's disease.
Observation:
- A patient presented with cerebral venous thrombosis and unusual progressive meningeal thickening with optic nerve involvement.
- Magnetic resonance imaging (MRI) revealed meningeal thickening and contrast enhancement.
- The patient also exhibited positive antineutrophil cytoplasmic antibodies (ANCA) and developed central diabetes insipidus.
Findings:
- This case describes a previously unreported combination of progressive meningeal thickening and optic nerve involvement in Behçet's disease.
- The findings underscore the diverse and sometimes atypical neurological manifestations of Behçet's disease.
- The presence of ANCA and subsequent central diabetes insipidus represent further unusual features in this patient.
Implications:
- This report expands the spectrum of neuroimaging findings in Behçet's disease.
- It emphasizes the importance of considering Behçet's disease in patients with unexplained neurological and meningeal abnormalities.
- Further research may elucidate the mechanisms behind these rare presentations and guide management strategies.