Data from subjects receiving intrathecal laronidase for cervical spinal stenosis due to mucopolysaccharidosis type I

Peer reviewe

The Structural Brain Correlates of Cognitive Deficits in Adults with Klinefelter’s Syndrome

Context: Adults with Klinefelter’s syndrome (KS) are known to present disturbances of language skills and delayed learning abilities. Objectives: The aim of this study was to assess brain morphometry in KS and to correlate eventual volumetric changes with performance on neuropsychological tests. Patients: Patients included 18 KS adults and 20 age-matched controls. Methods: All participants underwent prospectively double-spin-echo brain magnetic resonance imaging and neuropsychological testing of verbal and nonverbal domains. On the axial stack of magnetic resonance imaging slices, regional brain volumes were measured either by automated segmentation (full brain, total cerebrospinal fluid, and ventricular volume) or manual drawing with help of a neuroanatomy atlas (frontal, temporal, and parietal lobes, gray matter component of the lobes, cerebellar hemispheres, and hippocampal complexes)