Widespread subcortical grey matter degeneration in primary lateral sclerosis: a multimodal imaging study with genetic profiling

灰质 壳核 萎缩 基于体素的形态计量学 肌萎缩侧索硬化 神经科学 基底神经节 尾状核 神经影像学 心理学 病理 海马硬化 海马结构 医学 白质 磁共振成像 颞叶 疾病 中枢神经系统 放射科 癫痫
作者
Eoin Finegan,Stacey Li Hi Shing,Rangariroyashe H. Chipika,Mark A. Doherty,Jennifer C. Hengeveld,Alice Vajda,Colette Donaghy,Niall Pender,Russell L. McLaughlin,Orla Hardiman,Peter Bede
出处
期刊:NeuroImage: Clinical [Elsevier BV]
卷期号:24: 102089-102089 被引量:58
标识
DOI:10.1016/j.nicl.2019.102089
摘要

Primary lateral sclerosis (PLS) is a low incidence motor neuron disease which carries a markedly better prognosis than amyotrophic lateral sclerosis (ALS). Despite sporadic reports of extra-motor symptoms, PLS is widely regarded as a pure upper motor neuron disorder. The post mortem literature of PLS is strikingly sparse and very little is known of subcortical grey matter pathology in this condition. A prospective imaging study was undertaken with 33 PLS patients, 117 healthy controls and 100 ALS patients to specifically assess the integrity of subcortical grey matter structures and determine whether PLS and ALS have divergent thalamic, hippocampal and basal ganglia signatures. Volumetric, morphometric, segmentation and vertex-wise analyses were carried out in the three study groups to evaluate the integrity of thalamus, hippocampus, caudate, amygdala, pallidum, putamen and accumbens nucleus in each hemisphere. The hippocampus was further parcellated to characterise the involvement of specific subfields. Considerable thalamic, caudate, and hippocampal atrophy was detected in PLS based on both volumetric and vertex analyses. Significant volume reductions were also detected in the accumbens nuclei. Hippocampal atrophy in PLS was dominated by dentate gyrus, hippocampal tail and CA4 subfield volume reductions. The morphometric comparison of ALS and PLS cohorts revealed preferential medial bi-thalamic pathology in PLS compared to the predominant putaminal degeneration detected in ALS. Another distinguishing feature between ALS and PLS was the preferential atrophy of the amygdala in ALS. PLS is associated with considerable subcortical grey matter degeneration and due to the extensive extra-motor involvement, it should no longer be regarded a pure upper motor neuron disorder. Given its unique pathological features and a clinical course which differs considerably from ALS, dedicated research studies and disease-specific therapeutic strategies are urgently required in PLS.

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