Moyamoya-like vascular abnormality in pulmonary sarcoidosis.

Cigarette smoking, alcohol ingestion and any regular medications including birth control pills were denied. Family history of stroke was also denied. Physical and neurological examination revealed no abnormal signs except for bilaterally decreased superfi cial sensation in the distribution from the right 2nd thoracic and left 8th cervical to bilateral 2nd lumbar segments. Laboratory results revealed decreased platelet count (125 ! 10 3 /mm 3 ) and an elevated serum angiotensin-converting enzyme level (67.8 U/l, normal range 8–52 U/l). Electrocardiogram was normal. Chest X-ray and CT scan showed bilateral hilar and mediastinal lymphadenopathy. Specimens obtained from transbronchial lung biopsy revealed noncaseating granulomas, which was consistent with sarcoidosis. Transfemoral cerebral angiography showed bilateral occlusion of the internal carotid arteries (ICAs) at the terminal portion ( fi g. 2 ). There were no defi nite basal moyamoya vessels. Collateral circulation was provided by the vertebrobasilar system through posterior communicating arteries and by external carotid arteries through transdural anastomosis via meningeal arteries. Brain SPECT disclosed perfusion defect on the right frontal cortex and decreased perfusion reserve in the anterior cerebral artery (ACA) and middle cerebral artery (MCA) territory bilaterally. MRIs of the cervical and thoracic spine showed no signifi cant abnormalities. CSF examination results were within normal limits. Needle EMG examination showed fi brillation potentials and positive sharp waves in 2–8 left thoracic and 3–7 right paraspinal muscles, indicating bilateral thoracic radiculopathy. Cerebrovasc Dis 2006;22:71–73 DOI: 10.1159/000092924