Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration
Rangariroyashe H.Chipika1
Grainne Mulkerrin1
Pierre-François Pradat2
Aizuri Murad1
Fabrice Ango3
Cédric Raoul3
Peter Bede4
1.Computational Neuroimaging Group,Biomedical Sciences Institute,Trinity College Dublin,Dublin,Ireland2.Pitié-Salpêtrière University Hospital,Sorbonne University,Paris,France3.The Neuroscience Institute of Montpellier(INM),INSERM,CNRS,Montpellier,France4.Computational Neuroimaging Group,Biomedical Sciences Institute,Trinity College Dublin,Dublin,Ireland;Pitié-Salpêtrière University Hospital,Sorbonne University,Paris,France
摘要：Amyotrophic lateral sclerosis is a relentlessly progressive multi-system condition. The clinical picture is dominated by upper and lower motor neuron degeneration, but extra-motor pathology is increasingly recognized, including cerebellar pathology. Post-mortem and neuroimaging studies primarily focus on the characterization of supratentorial disease, despite emerging evidence of cerebellar degeneration in amyotrophic lateral sclerosis. Cardinal clinical features of amyotrophic lateral sclerosis, such as dysarthria, dysphagia, cognitive and behavioral deficits, saccade abnormalities, gait impairment, respiratory weakness and pseudobulbar affect are likely to be exacerbated by co-existing cerebellar pathology. This review summarizes in vivo and post mortem evidence for cerebellar degeneration in amyotrophic lateral sclerosis. Structural imaging studies consistently capture cerebellar grey matter volume reductions, diffusivity studies readily detect both intra-cerebellar and cerebellar peduncle white matter alterations and functional imaging studies commonly report increased functional connectivity with supratentorial regions. Increased functional connectivity is commonly interpreted as evidence of neuroplasticity representing compensatory processes despite the lack of post-mortem validation. There is a scarcity of post-mortem studies focusing on cerebellar alterations, but these detect pTDP-43 in cerebellar nuclei. Cerebellar pathology is an overlooked facet of neurodegeneration in amyotrophic lateral sclerosis despite its contribution to a multitude of clinical symptoms, widespread connectivity to spinal and supratentorial regions and putative role in compensating for the degeneration of primary motor regions.
机标关键词：neurodneuronmotorcerebellardegenerationdiseaseneuroplasticitypathology
论文发表日期：2022-11-28
在线出版日期：2025-08-15（本平台首次上网日期，不代表文献的发表时间）
页数：7( 2335-2341 )
英文信息