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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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Neuro-Behcet's Disease

Unlike Takayasu disease, Behcet’s syndrome is more common in men. There is a triad of oral and genital ulcers with uveitis. There can be CNS involvement in up to 30% of cases which can include small vessel vasculitis and meningovascular inflammatory cell deposition. Neuro-Behcet’s disease can be either acute or chronic progressive [77]. The acute form can be set off by cyclosporine A. The acute form is associated with a meningoencephalitis with focal lesions seen on T2-weighted and FLAIR MRI [78]. This form tends to be very steroid responsive and self-limited.

The chronic form is characterized by a slowly progressive neurological impairment with dementia, ataxia, and dysarthria [79]. There is an elevation of cerebrospinal fluid interleukin-6 activity [54]. High-dose methylprednisolone tends to be the first line of therapy with well-recognized resistance to such therapy along with cyclophosphamide and azathioprine. However, low-dose methotrexate may be of particular benefit for disease suppression in the chronic progressive form [80].

 
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