What is the risk of developing neuro-Behçet's (Neurological manifestations of Behçet's disease) in patients with Behçet's disease and retinal vasculitis?

Risk of Neuro-Behçet's in Patients with Retinal Vasculitis

Patients with Behçet's disease who have retinal vasculitis are at significant risk for developing neurological manifestations, with neurological involvement occurring in approximately 5.3% to 50% of all Behçet's patients. 1

Epidemiology and Risk Factors

• Neurological manifestations represent a major cause of morbidity and mortality in Behçet's disease 2
• Neurological involvement can be divided into two major forms:
  • Parenchymal lesions (mainly meningoencephalitis)
  • Extra-parenchymal lesions (cerebral venous thrombosis and arterial aneurysms) 1
• In approximately one-third of cases, neurological manifestations can be the presenting features of Behçet's disease, making early recognition challenging 1

Clinical Presentation of Neuro-Behçet's

• Common neurological manifestations include:
  • Headache and pyramidal signs, particularly in young men 1
  • Brainstem syndrome
  • Multiple sclerosis-like presentations
  • Movement disorders
  • Meningoencephalitic syndrome
  • Myelopathic syndrome
  • Cerebral venous sinus thrombosis
  • Intracranial hypertension 3

Diagnostic Features

• MRI findings in acute neuro-Behçet's typically show mesodiencephalic lesions 3
• The pattern of extension from thalamus to midbrain provides a characteristic "cascade sign" 3
• Cerebrospinal fluid (CSF) generally exhibits pleocytosis, increased protein, and normal glucose in parenchymal neuro-Behçet's 3
• In cerebral venous sinus thrombosis, CSF pressure is increased but content is usually normal 3

Treatment Recommendations

• High-dose corticosteroids remain the cornerstone for managing acute phase neurological involvement in Behçet's disease 2
• For parenchymal involvement, agents to be tried may include:
  • Corticosteroids
  • Interferon-alpha
  • Azathioprine
  • Cyclophosphamide
  • Methotrexate
  • TNF-alpha antagonists 4, 5
• For dural sinus thrombosis, corticosteroids are recommended 4, 5
• Cyclosporine A should not be used in Behçet's patients with central nervous system involvement unless necessary for intraocular inflammation 4, 5

Management of Retinal Vasculitis

• Any patient with Behçet's disease and inflammatory eye disease affecting the posterior segment should be on a treatment regimen that includes azathioprine and systemic corticosteroids 4
• For severe eye disease (defined as >2 lines drop in visual acuity and/or retinal disease including retinal vasculitis), it is recommended to use either cyclosporine A or infliximab in combination with azathioprine and corticosteroids; alternatively, interferon-alpha with or without corticosteroids could be used 4
• Corticosteroids should never be used alone in patients with posterior uveitis 4

Prevention and Monitoring

• Regular ophthalmologic examinations are essential for patients with ocular involvement 5
• Young men with early disease onset have a higher risk of severe disease and may benefit from early systemic immunosuppression with azathioprine 5
• Monitoring should include assessment of clinical symptoms and inflammatory markers (ESR, CRP) regularly, with consideration of repeat imaging for neurological involvement 5

Prognosis

• Ocular lesions may improve with immunosuppressive therapy but are usually not fully reversible and generally progress over time 6
• The prognosis of anterior uveitis is usually good, but patients with posterior lesions tend to have some degree of visual loss, even with adequate treatment 6
• Early recognition and timely treatment of neurological involvement are crucial to prevent long-term disability 2