What is the initial treatment for neuro-Behcet syndrome?

Initial Treatment for Neuro-Behçet Syndrome

For patients with neuro-Behçet syndrome, high-dose corticosteroids (intravenous methylprednisolone 1g/day for 5-7 days) should be the initial treatment, followed by oral tapering over 2-3 months. 1, 2, 3

Classification of Neuro-Behçet Syndrome

Neuro-Behçet syndrome can be classified into two main types:

1. Parenchymal involvement (most common, 61%):

   • Brainstem syndrome (72.9%)
   • Cerebral/hemispheric involvement
   • Spinal cord involvement (24.5%)
   • Meningoencephalitic manifestations

2. Non-parenchymal involvement (28.6%):

   • Cerebral venous sinus thrombosis
   • Intracranial hypertension
   • Arterial involvement

Treatment Algorithm

Acute Parenchymal Neuro-Behçet:

1. Initial treatment:

   • High-dose intravenous methylprednisolone (1g/day for 5-7 days) 2, 3
   • Follow with oral prednisone (1 mg/kg/day) tapering over 2-3 months 2

2. Risk stratification:

   • Low-risk patients (no poor prognostic factors):

     • Azathioprine or methotrexate with corticosteroids 4

   • High-risk patients (multifocal involvement, spinal presentations, >2 attacks/year, progressive course):

     • Intravenous cyclophosphamide with corticosteroids 4, 5

3. For refractory cases:

   • TNF-alpha inhibitors (infliximab or etanercept) 4, 5
   • Interferon-alpha 4, 5
   • Chlorambucil (last resort) 4

Non-parenchymal Neuro-Behçet (Cerebral Venous Sinus Thrombosis):

• Short-term corticosteroids
• Consider immunosuppressants
• Note: According to EULAR recommendations, anticoagulation is not recommended due to risk of pulmonary arterial aneurysm which might result in fatal bleeding 1

Monitoring and Follow-up

• Regular assessment of neurological status
• MRI monitoring (acute NBD typically shows mesodiencephalic lesions; chronic NBD shows brain/brainstem atrophy) 6
• CSF analysis (parenchymal NBD typically shows pleocytosis and increased protein) 6

Prognosis

• Approximately 58% of patients with acute onset parenchymal disease have only one attack 5
• Mortality is higher in parenchymal NBD compared to non-parenchymal NBD 5
• Poor prognostic factors include multifocal involvement, spinal presentations, frequent attacks, progressive course, and increased CSF cell count/protein 4

Important Considerations

• Early initiation of treatment is crucial to prevent irreversible neurological damage
• Long-term immunosuppression is often necessary to prevent relapses
• Cyclosporine A should be avoided in patients with CNS involvement unless necessary for intraocular inflammation 1
• Regular monitoring for medication side effects is essential
• The disease typically follows a relapsing and remitting course, with the goal of treatment being to promptly suppress inflammatory exacerbations 1

The management of neuro-Behçet syndrome requires a multidisciplinary approach involving neurologists, rheumatologists, and other specialists depending on other organ system involvement.