Isolated vasculitis of the CMS

Vasculitis is a rare cause for disease of the CNS. The isolated vasculitis of the CNS is restricted to the CNS whereas other forms of vasculitis affec t various organs including the CNS. Headache, encephalopathy, focal deficit s and epileptic seizures are the major symptoms suggest ive for vasculitis. One major criterion of the isolated vasculitis of the CNS is the lack of e vidence for other vasculitis forms or for pathology of other organs. Angiog raphy displays multifocal segmental stenosis of intracranial vessels. MRI d emonstrates multiple lesions which in part show enhancement after gadoliniu m. A definite diagnosis can only be made on the grounds of biopsy from lept omeninges and parenchyma. Therapy consists of corticosteroids and cyclophos phamid.