NeuroBehcet's pearls

multiple sources but NEJM CPC from May 28, 2009 was one.

1. Known as Silk Route disease due to occurrence along trading paths in Asia
2. Criteria include (major) oral ulcers occurring at least three times per year, genital ulcers or scars, ocular involvement (uveitis), skin (erythema nodosa, folliculitis, acne), positive skin pathergy test. Minor criteria are arthritis, DVT, superficial thrombophlebitis, epididymitis, positive family history, GI, CNS, or vascular involvement.
3. Neurologic involvement occurs in 5-10 %, with vascular involvement (venous sinus thrombosis or arterial thrombosis), or parenchymal infiltration. Brain stem and basal ganglia are common areas of predilection. The disease can be relapsing-remitting, chronic progressive, or secondary progressive.
4. Abnormal labs include high sed rate, CRP, complement, IL6,8 , TNF, and distinctive CSF neutrophilic pleocytosis.
5. Best treatment is not know, but in CPC authors suggested high dose pulsed steroid and cyclophosphamide, and maintenance immunoosuppression with azathioprine.