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

ABSTRACT:

Background

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.

Methods

We examined the neuropsychological profile in 16 SPG11 patients using a defined neuropsychological testing battery. Long-term

SUBMITTER: Utz KS 

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

REPOSITORIES: biostudies-literature