Premium
Selective Arterial Distribution of Cerebral Hyperperfusion in Fabry Disease
Author(s) -
Moore David F.,
Herscovitch Peter,
Schiffmann Raphael
Publication year - 2001
Publication title -
journal of neuroimaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.822
H-Index - 64
eISSN - 1552-6569
pISSN - 1051-2284
DOI - 10.1111/j.1552-6569.2001.tb00051.x
Subject(s) - statistical parametric mapping , cerebral blood flow , medicine , positron emission tomography , fabry disease , magnetic resonance imaging , emission computed tomography , hemodynamics , cardiology , blood flow , nuclear medicine , radiology , disease
Fabry disease is an X‐linked recessive deficiency of lysosomal α‐galactosidase A associated with an increased risk of early onset cerebrovascular disease. The disorder is reported to affect the posterior circulation predominantly. This hypothesis was investigated directly by the measurement of regional cerebral blood flow with positron emission tomography (PET). Resting regional cerebral blood flow (rCBF) in 26 hemizygous patients with Fabry disease and 10 control participants was examined using H 2 15 O and PET. Statistical parametric mapping (SPM{t}, SPM99) and PET images of patients and controls were produced. Significantly increased SPM{t} clusters were then color coded and blended with a coregistered T1 magnetic resonance imaging (MRI) template. Cerebral arterial territory maps were digitized and rescaled. Custom OpenGL and ImageVision Library C++ code was written to allow a first‐order affine transformation of the blended SPM{t} and MRI template onto the arterial territory map. The affine transformation was constrained by choosing corresponding cerebral landmark “tie points” between the SPM{t}∪ MRI template images and the cerebral arterial territory maps. The data demonstrated that the posterior circulation is the predominant arterial territory with a significantly increased rCBF in Fabry disease. No arterial distribution had a decreased rCBF.