Hunter Syndrome
21-year-old male, complaining of headache.
T2/FLAIR (Figure 1) shows non-specific hyperintensitivity in periventricular and deep white matter. Sagittal T2 (Figure 2) shows that the posterior (arrow) vascular filament of C2 causes moderate spinal stenosis. Axial T2WI (Figure 3) shows diffuse thickening of the bilateral retina and inverted eyeball. Sagittal T2WI (Figure 4
) shows a degenerative (arrow) of the left temporomandibular joint. Axial T2WI (Figure 5) shows bilateral widening of the subarachnoid space of the middle cranial fossa (arrows). Axial T2WI (Figure 6) shows frontal bone hyperplasia, and the right frontal sinus is not vaporized (arrow). The sagittal T2WI (Figure 7) shows an enlarged molar gap in the left jaw (arrow).
Hunter syndrome is a form of mucopolysaccharide disease.
Clinical manifestations: may be mild or severe. The main causes of morbidity and mortality are neurodegeneration, spinal cord compression, narrowing of the airways and heart failure.
Imaging features: cerebral atrophy, bilateral periventricular and deep white matter T2 hyperintensity, spinal stenosis due to vascular formation at the cranial-cervical junction, hydrocephalus, enlargement of the perivascular and subarachnoid space, large occipital hydrangea, characteristic bilateral thickening of the retina, inverted eyeball, optic nerve atrophy, premature closure of the cranial suture and frontal sinus inospiration, early degeneration of the temporomandibular joint, increased spacing between teeth.
