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...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2022-01-01
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| Series: | Neural Regeneration Research |
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| Online Access: | http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=11;spage=2335;epage=2341;aulast=Chipika |
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| author | Rangariroyashe H Chipika Grainne Mulkerrin Pierre-François Pradat Aizuri Murad Fabrice Ango Cédric Raoul Peter Bede |
| author_facet | Rangariroyashe H Chipika Grainne Mulkerrin Pierre-François Pradat Aizuri Murad Fabrice Ango Cédric Raoul Peter Bede |
| author_sort | Rangariroyashe H Chipika |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-12T14:32:06Z |
| format | Article |
| id | doaj.art-0c124dd66dd9441d83293b5c996d1dba |
| institution | Directory Open Access Journal |
| issn | 1673-5374 |
| language | English |
| last_indexed | 2024-04-12T14:32:06Z |
| publishDate | 2022-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Neural Regeneration Research |
| spelling | doaj.art-0c124dd66dd9441d83293b5c996d1dba2022-12-22T03:29:13ZengWolters Kluwer Medknow PublicationsNeural Regeneration Research1673-53742022-01-0117112335234110.4103/1673-5374.336139Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegenerationRangariroyashe H ChipikaGrainne MulkerrinPierre-François PradatAizuri MuradFabrice AngoCédric RaoulPeter BedeAmyotrophic 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.http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=11;spage=2335;epage=2341;aulast=Chipikaamyotrophic lateral sclerosis; ataxia; cerebellum; magnetic resonance imaging; motor neuron disease; neuroimaging; neuroplasticity; pathology; primary lateral sclerosis; pseudobulbar affect |
| spellingShingle | Rangariroyashe H Chipika Grainne Mulkerrin Pierre-François Pradat Aizuri Murad Fabrice Ango Cédric Raoul Peter Bede Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration Neural Regeneration Research amyotrophic lateral sclerosis; ataxia; cerebellum; magnetic resonance imaging; motor neuron disease; neuroimaging; neuroplasticity; pathology; primary lateral sclerosis; pseudobulbar affect |
| title | Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration |
| title_full | Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration |
| title_fullStr | Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration |
| title_full_unstemmed | Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration |
| title_short | Cerebellar pathology in motor neuron disease: neuroplasticity and neurodegeneration |
| title_sort | cerebellar pathology in motor neuron disease neuroplasticity and neurodegeneration |
| topic | amyotrophic lateral sclerosis; ataxia; cerebellum; magnetic resonance imaging; motor neuron disease; neuroimaging; neuroplasticity; pathology; primary lateral sclerosis; pseudobulbar affect |
| url | http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=11;spage=2335;epage=2341;aulast=Chipika |
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