Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration

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 foc...
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Autor*in:	Rangariroyashe H Chipika [verfasserIn] Grainne Mulkerrin [verfasserIn] Pierre-François Pradat [verfasserIn] Aizuri Murad [verfasserIn] Fabrice Ango [verfasserIn] Cédric Raoul [verfasserIn] Peter Bede [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2022

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

Übergeordnetes Werk:	In: Neural Regeneration Research - Wolters Kluwer Medknow Publications, 2014, 17(2022), 11, Seite 2335-2341
Übergeordnetes Werk:	volume:17 ; year:2022 ; number:11 ; pages:2335-2341

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.4103/1673-5374.336139

Katalog-ID:	DOAJ021617635

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spelling	10.4103/1673-5374.336139 doi (DE-627)DOAJ021617635 (DE-599)DOAJ0c124dd66dd9441d83293b5c996d1dba DE-627 ger DE-627 rakwb eng RC346-429 Rangariroyashe H Chipika verfasserin aut Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. amyotrophic lateral sclerosis; ataxia; cerebellum; magnetic resonance imaging; motor neuron disease; neuroimaging; neuroplasticity; pathology; primary lateral sclerosis; pseudobulbar affect Neurology. Diseases of the nervous system Grainne Mulkerrin verfasserin aut Pierre-François Pradat verfasserin aut Aizuri Murad verfasserin aut Fabrice Ango verfasserin aut Cédric Raoul verfasserin aut Peter Bede verfasserin aut In Neural Regeneration Research Wolters Kluwer Medknow Publications, 2014 17(2022), 11, Seite 2335-2341 (DE-627)545785499 (DE-600)2388460-5 18767958 nnns volume:17 year:2022 number:11 pages:2335-2341 https://doi.org/10.4103/1673-5374.336139 kostenfrei https://doaj.org/article/0c124dd66dd9441d83293b5c996d1dba kostenfrei http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=11;spage=2335;epage=2341;aulast=Chipika kostenfrei https://doaj.org/toc/1673-5374 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_374 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_2700 GBV_ILN_2817 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 17 2022 11 2335-2341
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author	Rangariroyashe H Chipika
spellingShingle	Rangariroyashe H Chipika misc RC346-429 misc amyotrophic lateral sclerosis; ataxia; cerebellum; magnetic resonance imaging; motor neuron disease; neuroimaging; neuroplasticity; pathology; primary lateral sclerosis; pseudobulbar affect misc Neurology. Diseases of the nervous system Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration
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topic_title	RC346-429 Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration amyotrophic lateral sclerosis; ataxia; cerebellum; magnetic resonance imaging; motor neuron disease; neuroimaging; neuroplasticity; pathology; primary lateral sclerosis; pseudobulbar affect
topic	misc RC346-429 misc amyotrophic lateral sclerosis; ataxia; cerebellum; magnetic resonance imaging; motor neuron disease; neuroimaging; neuroplasticity; pathology; primary lateral sclerosis; pseudobulbar affect misc Neurology. Diseases of the nervous system
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title	Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration
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title_sort	cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration
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title_auth	Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration
abstract	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.
abstractGer	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.
abstract_unstemmed	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.
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title_short	Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration
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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. 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Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration

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