On imaging patients have cerebellar hemisphere atrophy, beginning in the vermis and the lateral aspect of the cerebellar hemispheres. Parenchymal hemorrhages may occur as a result of intraparenchymal telangiectasias. Patients with pulmonary vascular malformations may develop cerebral infarcts due to emboli traversing these malformations. SWI imaging may demonstrate multiple small hemorrhages. MRS at a TE=144 shows reduction in all metabolites in the cerebellum. MRS of T2/FLAIR hyperintense lesions of the cerebral hemispheres shows low NAA and a large lipid peak
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