Beh$et's disease is a multisystem vasculitis of unknown origin. It is especially common in Middle Eastern and Mediterranean countries. CNS involvement has been described in 4-49% of cases . The parenchymal distribution of lesions, especially at the mesodiencephalic junction (46%) supports small vessel vasculitis involving both the arterial and venous systems; mainly venules. The lesions are occasionally reversible on MRI, which mainly represents vasogenic edema, which is why DW imaging is useful in distinguishing them from infarction (Fig. 7.7). The treatment is usually a combination of cytotoxic agents and steroids. In other types of collagen diseases, such as scleroderma or rheumatoid arthritis, involvement of the CNS is very rare.
Churg-Strauss disease in a 65-year-old man with seizures. A T2-weighted image shows multiple hyperintense lesions in both corona radiata and right parieto-oc-cipital area, the latter with a he-mosiderin rim, representing old hemorrhage (arrow). b DW image shows a hyperintense lesion (arrow) in the left corona radiata, representing an acute infarct. An old hemorrhage shows hypointensity (arrowhead) on DW image. MR and DS angiography revealed no abnormalities (not shown). (From )
Neuro-Beh<;et's disease in a 24-year-old male. a T2-weighted image shows a hyperintense lesion in the left midbrain extending into the left temporal lobe, with enlargement of the left cerebral peduncle (arrow). b Gadolinium-enhanced T1 -weighted image shows enhancement in this lesion (arrow). c DW image shows a hyperintense lesion with increased signal intensity in the left cerebral peduncle, probably representing vasogenic edema (arrow). d ADC map shows increased ADC in this lesion (arrow). (From )
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