Neuropsychology and MRI correlates of neurodegeneration in SPG11 hereditary spastic paraplegia.

ABSTRACT: SPG11-linked hereditary spastic paraplegia is characterized by multisystem neurodegeneration leading to a complex clinical and yet incurable phenotype of progressive spasticity and weakness. Severe cognitive symptoms are present in the majority of SPG11 patients, but a systematic and multidimensional analysis of the neuropsychological phenotype in a larger cohort is lacking. While thinning of the corpus callosum is a well-known structural hallmark observed in SPG11 patients, the neuroanatomical pattern of cortical degeneration is less understood. We here aimed to integrate neuropsychological and brain morphometric measures in SPG11. We examined the neuropsychological profile in 16 SPG11 patients using a defined neuropsychological testing battery. Long-term follow up testing was performed i

SUBMITTER: Utz KS 

PROVIDER: S-EPMC9336101 | biostudies-literature | 2022 Jul

REPOSITORIES: biostudies-literature