Behcet's and stroke

Behcet's is an inflammatory condition of unknown cause with the characteristic triad of recurrent oral apthous ulcers, genital ulcers, and relapsing uveitis. It is multiorgan and is most common in the Mediterranean, Middle East and Japan. NeuroBehcet's occurs in about a third , by far, most often with meningoencephalitis, beginning months or years after mucocutaneous symptoms and concomitantly with other systemic symptoms. Rarely, it can be inaugural. The meningoencephalitis begins in the brainstem with fluctuating headache, bilateral corticispinal spinal signs, cerebellar incoordination, CN palsies, dysarthria, pseudobulbar palsy, seizures,stupor, pleocytosis. CT/MR shows enhancing lesions in non vascular distributions. They mimic MS without usually being periventricular. Most commonly they affect the brainstem, hypothalamus, and basal ganglia. Angio is normal. Path shows small foci of softening with lymphocytic perivascular infiltration, diffuse microglial activity, and small areas of demyelination.

Cerebral venous thrombosis and other venous thrombosis, including vena cava and portal vein, occur in one third. CVT was long overlooked and is the main cause of the papilledema that occurs in twenty percent (misdiagnosed as pseudotumor cerebri). Differences from that condition may include abnormal CT or CSF. Also, greater than 50 % present with focal signs seizures, deficits. CVT occurs in any cerebral vein. CVT must be differentiated from neuroBehcet's , which more commonly occurs with extravascular nonneurologic symptoms, and has a worse prognosis. Immunosuppression is not indicated in CVT without neuroBehcet's; anticoagulation is.

Other rarer arterial vascular complications include aneurysms, single or multiple, that are "far" less frequent than systemic aneurysms, arteriovenous malformations, intracranial hemorrhages (may be related to htn), dissection, large artery occlusive disease (rare). Aneurysm rupture complicates the prognosis for patients with arterial disease, for whom the prognosis is worse than it is for venous disease. Actual arteritis is rare. Steroids and immunosuppressive medication is indicated.

Other neurologic manifestations includeaseptic meningitis, myelitis, ON, PN, myositis.