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Neuro-Behçet's disease: A review.

Nabil Belfeki1, Nouha Ghriss1, Montasar Fourati2

  • 1Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud Île de France, Melun, France.

La Revue De Medecine Interne
|June 27, 2024
PubMed
Summary

Behcet disease (BD) can affect the nervous system (NBD), presenting diagnostic challenges. Early recognition and treatment of neurological Behcet disease are crucial for better outcomes and preventing complications.

Keywords:
Behçet's diseaseDiagnosisManagementNeuro-Behçet diseaseNeuroimagingPrognosis

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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.