Magnetic Resonance Imaging

The MRI abnormalities found in MPS vary greatly in severity from absent or negligible to severe, with a marked variation among sibs. However, in themselves, the abnormalities are fairly homogeneous.

Over the years many patients develop white matter abnormalities. These consist of multiple small spotlike lesions dispersed in the white matter, with a predilection for the parietal and occipital white matter. The signal intensity follows the signal intensity of CSF, indicative of the cystic nature of the lesions. The cystic areas often have a radial orientation from the subependymal region toward the cortex (Figs. 13.1 and 13.2). Punched-out cystic areas are often also present in the corpus callosum, best visualized on the sagittal images (Figs. 13.1 and 13.2). These cystic white matter lesions represent the perivascular lacunae seen on histopathological examination. In exceptional cases, the thalamus and basal ganglia have a honeycomb-like appearance, related to highly enlarged perivascular spaces in these areas. In addition, T2-weighted and FLAIR images may show multifocal smaller and larger hyperintense areas, the signal intensity of which does not follow that of CSF. These areas may become extensive and confluent, and probably reflect gliosis, which is also seen on histopatho-logical examination. The white matter abnormalities can be progressive on follow-up MRI. MRI and CT have demonstrated that white matter abnormalities may occur in all MPS variants. MRI may show a delay in myelination in young children.

Another frequent observation consists of ventricular enlargement, with or without accompanying enlargement of subarachnoid spaces. Enlargement of CSF spaces may occur in all MPS variants. The ven

Imaging Hurler
Fig. 13.1. A 3-year-old boy with Hurler syndrome (MPS I). The sagittal T1-weighted images show the radial stripes of enlarged perivascular spaces, most prominent in the parietal region and also seen in the corpus callosum.The T2-weighted

images confirm the enlarged perivascular spaces and show more extensive areas of signal abnormality in the region of the perivascular spaces. Courtesy of Dr. S. Blaser, Department of Diagnostic Imaging, Hospital for Sick Children,Toronto tricular enlargement is of variable severity and is in some cases progressive. Some of the patients appear to have enlarged CSF spaces on the basis of diffuse atrophy of the brain parenchyma. In many cases, however, the enlargement of the CSF spaces is caused by hydrocephalus as a consequence of disturbed CSF resorption. Signs of hydrocephalus are upward bulging

Fig. 13.2. A 4-year-old boy with Hunter syndrome (MPS II).The sagittal and axial T1-weighted images show the radial stripes of enlarged perivascular spaces, most prominent in the parietal region and the corpus callosum.The T2-weighted images confirm the enlarged perivascular spaces and show more extensive signal abnormalities in the region of the perivascular spaces. Courtesy of Dr. S. Blaser, Department of Diagnostic Imaging, Hospital for Sick Children,Toronto

Morquio Brain MriMaroteaux Lamy
Fig. 13.3. Spinal MRI of a 5-year-old boy with Maroteaux-Lamy syndrome (MPS VI) shows abnormalities of the cranio-cervical junction, with deposits of mucopolysaccharide material around the dens, narrowing the spinal canal. The T2-weighted images show no free CSF space around the cervical

spinal cord.The cervical vertebrae show platyspondyly. In the lower part of the vertebral column,T12,LI,L2,and L3 are dysmorphic with agenesis of the anterior parts of the vertebrae, resulting in local kyphosis

Morquio Syndrome Brain

Fig. 13.4. A 5-year-old boy with Morquio syndrome (MPS IV).The mid-sagittal images show the intradural mucopolysaccharide deposits, leading to severe narrowing of the arachnoid space at the level of the craniocervical junction with compression of the spinal cord. Courtesy of Dr. P.Tortori Donati, Department of Pediatric Neuroradiology, G.Gaslini Children's Hospital, Genoa, Italy

Fig. 13.4. A 5-year-old boy with Morquio syndrome (MPS IV).The mid-sagittal images show the intradural mucopolysaccharide deposits, leading to severe narrowing of the arachnoid space at the level of the craniocervical junction with compression of the spinal cord. Courtesy of Dr. P.Tortori Donati, Department of Pediatric Neuroradiology, G.Gaslini Children's Hospital, Genoa, Italy

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  • johanna
    What do csf abnormalities look like on mri?
    8 years ago

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