Neuro-Behçet's disease: A review

Area of Science:

• Neurology
• Immunology
• Systemic Vasculitis

Background:

• Behcet disease (BD) is a multisystemic vasculitis with significant neurological involvement (NBD) in a substantial patient subset.
• Neurological manifestations in BD pose diagnostic challenges, particularly with initial neurological symptoms.
• NBD encompasses both parenchymal (meningoencephalitis) and extraparenchymal (vascular thrombosis) forms.

Purpose of the Study:

• To provide a comprehensive review of neurological presentations in Behcet disease.
• To emphasize diagnostic tools, prognosis, and therapeutic strategies for NBD.
• To highlight the importance of early diagnosis and management in improving patient outcomes.

Main Methods:

• Review of clinical, laboratory, and neuroimaging findings in NBD.
• Description of characteristic brain MRI findings (T2-FLAIR, T1-weighted images) in parenchymal NBD.
• Inclusion of diagnostic techniques like MR venography and CT angiography for extraparenchymal NBD.

Main Results:

• Parenchymal NBD involves immune-mediated meningoencephalitis affecting brainstem, basal ganglia, and thalamus.
• Extraparenchymal NBD includes cerebral venous thrombosis and arterial involvement.
• Neuroimaging reveals specific patterns such as ill-defined edema, swelling, and potential hemorrhages.

Conclusions:

• Early diagnosis of neurological Behcet disease is essential for timely and adequate therapy.
• Treatment strategies vary based on NBD subtype, ranging from glucocorticoids and immunosuppressants to cyclophosphamide or infliximab.
• Effective management aims to improve prognosis, reduce sequelae, and prevent mortality.