PMC8224401_01
PMC8224401_02
MPS I prior to stroke. (Left) MRI Head demonstrating imaging findings of macrocephaly, ventriculomegaly, small foramen magnum, and mild narrowing of cervical spine without cord compression. (Right) CT Head with contrast demonstrating bilateral internal carotid termini without stenosis.
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F1
HSCT, neuroimaging was obtained for macrocephaly and findings were consistent with MPS I (Figure 1). However, the patient showed no clinical evidence of neurological symptoms at this time.
PMC8224401_03
MPS I arteriopathy after left cerebral stroke. (Left) MR angiogram 8 weeks after stroke with left MCA occlusion and bilateral ICA termini steno-occlusive disease. (Right) New left arm weakness episodes 3 months after overt stroke with persisting bilateral steno-occlusive disease and adjacent irregular collaterals identified on CT angiogram (yellow arrow).
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F2
MRI revealed an acute left middle cerebral artery (MCA) territory infarct and right cerebral ischemia. MR and CT angiography demonstrated complete absence of blood flow into the distal left MCA segments and bilateral carotid terminus narrowing (Figure 2).
PMC8224401_04
(Upper) Initial MRI of left MCA overt stroke identified on T2, diffusion, and MRA. (Lower) Two years after right cerebral revascularization demonstrating no new ischemic or infarcted territories and increased right cerebral cortical vascularity.
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F3
A 2-year postoperative MRI demonstrated increased cortical vascularity without progressive ischemic changes (Figure 3, Lower panel).
PMC8224401_05
(Upper) Initial MRI of left MCA overt stroke identified on T2, diffusion, and MRA. (Lower) Two years after right cerebral revascularization demonstrating no new ischemic or infarcted territories and increased right cerebral cortical vascularity.
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F3
A 2-year postoperative MRI demonstrated increased cortical vascularity without progressive ischemic changes (Figure 3, Lower panel).