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Cerebellar pathology in motor neuron disease:neuroplasticity and neurodegeneration

Neural Regeneration Research 2022年第11期17卷 2335-2341页

作者： Rangariroyashe H.Chipika grainne mulkerrin Pierre-François Pradat Aizuri Murad Fabrice Ango Cédric Raoul Peter Bede Computational Neuroimaging Group Biomedical Sciences InstituteTrinity College DublinDublinIreland Pitié-Salpêtrière University Hospital Sorbonne UniversityParisFrance The Neuroscience Institute of Montpellier(INM) INSERMCNRSMontpellierFrance

Amyotrophic lateral sclerosis is a relentlessly progressive multi-system *** clinical picture is dominated by upper and lower motor neuron degeneration,but extra-motor pathology is increasingly recognized,including cerebellar ***-mortem and neuroimaging studies primarily focus on the chara cterization of supratentorial disease,des pite emerging evidence of cerebellar degeneration in amyotrophic lateral *** 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 *** review summarizes in vivo and post mortem evidence for cerebellar degeneration in amyotrophic lateral scle *** imaging studies consistently capture cerebellar grey matter volume reductions,diffusivity studies readily detect both intra-cerebellar and cerebellar peduncle white matter alte rations and functional imaging studies commonly report increased functional connectivity with supratentorial *** functional connectivity is commonly interpreted as evidence of neuro plasticity representing compensatory processes despite the lack of post-mortem *** is a scarcity of post-mortem studies focusing on cerebellar alte rations,but these detect pTDP-43 in cerebellar *** rebellar pathology is an overloo ked facet of neurodegeneration in amyotrophic lateral sclerosis despite its contribution to a multitude of clinical symptoms,wides p read connectivity to spinal and supratentorial regions and putative role in compensating for the degeneration of primary motor regions.

关键词： amyotrophic lateral sclerosis ataxia cerebellum magnetic resonance imaging motor neuron disease neuroimaging neuroplasticity pathology primary lateral sclerosis pseudobulbar affect

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