作者
Eoin Finegan, Rangariroyashe H Chipika, Stacey Li Hi Shing, Mark A Doherty, Jennifer C Hengeveld, Alice Vajda, Colette Donaghy, Russell L McLaughlin, Niall Pender, Orla Hardiman, Peter Bede
发表日期
2019/7/19
期刊
Journal of Neurology
页码范围
1-16
出版商
Springer Berlin Heidelberg
简介
Background
Primary lateral sclerosis is a progressive upper-motor-neuron disorder associated with markedly longer survival than ALS. In contrast to ALS, the genetic susceptibility, histopathological profile and imaging signature of PLS are poorly characterised. Suspected PLS patients often face considerable diagnostic delay and prognostic uncertainty.
Objective
To characterise the distinguishing clinical, genetic and imaging features of PLS in contrast to ALS and healthy controls.
Methods
A prospective population-based study was conducted with 49 PLS patients, 100 ALS patients and 100 healthy controls using genetic profiling, standardised clinical assessments and neuroimaging. Whole-brain and region-of-interest analyses were undertaken to evaluate patterns of grey and white matter degeneration …
学术搜索中的文章
E Finegan, RH Chipika, S Li Hi Shing, MA Doherty… - Journal of neurology, 2019