Can serial cerebral MRIs predict the neuronopathic phenotype of MPS II?

ABSTRACT:

Objective

To advance the prediction of the neurocognitive development in MPS II patients by jointly analyzing MRI and neurocognitive data in mucopolysaccharidosis (MPS) II patients.

Methods

Cognitive ability scores (CAS) were obtained by neuropsychological testing. Cerebral MRIs were quantified using a disease-specific protocol. MRI sumscores were calculated for atrophy, white-matter abnormalities (WMA) and Virchow-Robin spaces (VRS). To distinguish between atrophy and hydrocephalus the Evans' index and the callosal angle (CA) were measured. A random effects repeated measurement model was used to correlate CAS with the three MRI sumscores.

Results

MRI (n = 47) and CAS scores (n = 78) of 19 male patients were analyzed. Ten patients were classified as neuronopathic and

SUBMITTER: Vollebregt AAM 

PROVIDER: S-EPMC8248480 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature