Microstructural MRI Basis of the Cognitive Functions in Patients with Spinocerebellar Ataxia Type 2
•Cerebellar microstructural damage affects Middle and Superior Cerebellar Peduncle in SCA2 patients.•Diffusivity changes predominantly reflect the white matter degeneration typically observed in cerebellar atrophies.•Diffusivity changes may reflect a dysregulation of cerebello-cerebral interaction.•...
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| Vydáno v: | Neuroscience Ročník 366; s. 44 - 53 |
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| Jazyk: | angličtina |
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Elsevier Ltd
16.12.2017
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| ISSN: | 0306-4522, 1873-7544, 1873-7544 |
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| Abstract | •Cerebellar microstructural damage affects Middle and Superior Cerebellar Peduncle in SCA2 patients.•Diffusivity changes predominantly reflect the white matter degeneration typically observed in cerebellar atrophies.•Diffusivity changes may reflect a dysregulation of cerebello-cerebral interaction.•The pattern of cerebellar white matter damage is associated with impaired cognitive performances of patients.
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum. The particular atrophy pattern results in some typical clinical features mainly including motor deficits. In addition, the presence of cognitive impairments, involving language, visuospatial and executive functions, has been also shown in SCA2 patients and it is now widely accepted as a feature of the disease. The aim of the study is to investigate the microstructural patterns and the anatomo-functional substrate that could account for the cognitive symptomatology observed in SCA2 patients. In the present study, diffusion tensor imaging (DTI) based-tractography was performed to map the main cerebellar white matter (WM) bundles, such as Middle and Superior Cerebellar Peduncles, connecting cerebellum with higher order cerebral regions. Damage-related diffusivity measures were used to determine the pattern of pathological changes of cerebellar WM microstructure in patients affected by SCA2 and correlated with the patients’ cognitive scores. Our results provide the first evidence that WM diffusivity is altered in the presence of the cerebellar cortical degeneration associated with SCA2 thus resulting in a cerebello-cerebral dysregulation that may account for the specificity of cognitive symptomatology observed in patients. |
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| AbstractList | •Cerebellar microstructural damage affects Middle and Superior Cerebellar Peduncle in SCA2 patients.•Diffusivity changes predominantly reflect the white matter degeneration typically observed in cerebellar atrophies.•Diffusivity changes may reflect a dysregulation of cerebello-cerebral interaction.•The pattern of cerebellar white matter damage is associated with impaired cognitive performances of patients.
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum. The particular atrophy pattern results in some typical clinical features mainly including motor deficits. In addition, the presence of cognitive impairments, involving language, visuospatial and executive functions, has been also shown in SCA2 patients and it is now widely accepted as a feature of the disease. The aim of the study is to investigate the microstructural patterns and the anatomo-functional substrate that could account for the cognitive symptomatology observed in SCA2 patients. In the present study, diffusion tensor imaging (DTI) based-tractography was performed to map the main cerebellar white matter (WM) bundles, such as Middle and Superior Cerebellar Peduncles, connecting cerebellum with higher order cerebral regions. Damage-related diffusivity measures were used to determine the pattern of pathological changes of cerebellar WM microstructure in patients affected by SCA2 and correlated with the patients’ cognitive scores. Our results provide the first evidence that WM diffusivity is altered in the presence of the cerebellar cortical degeneration associated with SCA2 thus resulting in a cerebello-cerebral dysregulation that may account for the specificity of cognitive symptomatology observed in patients. Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum. The particular atrophy pattern results in some typical clinical features mainly including motor deficits. In addition, the presence of cognitive impairments, involving language, visuospatial and executive functions, has been also shown in SCA2 patients and it is now widely accepted as a feature of the disease. The aim of the study is to investigate the microstructural patterns and the anatomo-functional substrate that could account for the cognitive symptomatology observed in SCA2 patients. In the present study, diffusion tensor imaging (DTI) based-tractography was performed to map the main cerebellar white matter (WM) bundles, such as Middle and Superior Cerebellar Peduncles, connecting cerebellum with higher order cerebral regions. Damage-related diffusivity measures were used to determine the pattern of pathological changes of cerebellar WM microstructure in patients affected by SCA2 and correlated with the patients' cognitive scores. Our results provide the first evidence that WM diffusivity is altered in the presence of the cerebellar cortical degeneration associated with SCA2 thus resulting in a cerebello-cerebral dysregulation that may account for the specificity of cognitive symptomatology observed in patients. Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum. The particular atrophy pattern results in some typical clinical features mainly including motor deficits. In addition, the presence of cognitive impairments, involving language, visuospatial and executive functions, has been also shown in SCA2 patients and it is now widely accepted as a feature of the disease. The aim of the study is to investigate the microstructural patterns and the anatomo-functional substrate that could account for the cognitive symptomatology observed in SCA2 patients. In the present study, diffusion tensor imaging (DTI) based-tractography was performed to map the main cerebellar white matter (WM) bundles, such as Middle and Superior Cerebellar Peduncles, connecting cerebellum with higher order cerebral regions. Damage-related diffusivity measures were used to determine the pattern of pathological changes of cerebellar WM microstructure in patients affected by SCA2 and correlated with the patients' cognitive scores. Our results provide the first evidence that WM diffusivity is altered in the presence of the cerebellar cortical degeneration associated with SCA2 thus resulting in a cerebello-cerebral dysregulation that may account for the specificity of cognitive symptomatology observed in patients.Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum. The particular atrophy pattern results in some typical clinical features mainly including motor deficits. In addition, the presence of cognitive impairments, involving language, visuospatial and executive functions, has been also shown in SCA2 patients and it is now widely accepted as a feature of the disease. The aim of the study is to investigate the microstructural patterns and the anatomo-functional substrate that could account for the cognitive symptomatology observed in SCA2 patients. In the present study, diffusion tensor imaging (DTI) based-tractography was performed to map the main cerebellar white matter (WM) bundles, such as Middle and Superior Cerebellar Peduncles, connecting cerebellum with higher order cerebral regions. Damage-related diffusivity measures were used to determine the pattern of pathological changes of cerebellar WM microstructure in patients affected by SCA2 and correlated with the patients' cognitive scores. Our results provide the first evidence that WM diffusivity is altered in the presence of the cerebellar cortical degeneration associated with SCA2 thus resulting in a cerebello-cerebral dysregulation that may account for the specificity of cognitive symptomatology observed in patients. |
| Author | Leggio, M. Romano, S. Cercignani, M. Masciullo, M. Bozzali, M. Olivito, G. Clausi, S. Molinari, M. Iacobacci, C. Lupo, M. |
| Author_xml | – sequence: 1 givenname: G. surname: Olivito fullname: Olivito, G. email: g.olivito@hsantalucia.it organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 2 givenname: M. surname: Lupo fullname: Lupo, M. organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 3 givenname: C. surname: Iacobacci fullname: Iacobacci, C. organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 4 givenname: S. surname: Clausi fullname: Clausi, S. organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 5 givenname: S. surname: Romano fullname: Romano, S. organization: Department of Neurosciences, Mental Health and Sensory Organs (NESMOS), “Sapienza” University of Rome – Sant’Andrea Hospital, Rome, Italy – sequence: 6 givenname: M. surname: Masciullo fullname: Masciullo, M. organization: SPInalREhabilitation Lab, IRCCS Fondazione Santa Lucia, Rome, Italy – sequence: 7 givenname: M. surname: Molinari fullname: Molinari, M. organization: Neurorehabilitation 1 and Spinal Center, Robotic Neurorehabilitation Lab, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 8 givenname: M. surname: Cercignani fullname: Cercignani, M. organization: Neuroimaging Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 9 givenname: M. surname: Bozzali fullname: Bozzali, M. organization: Neuroimaging Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 10 givenname: M. surname: Leggio fullname: Leggio, M. organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/29031602$$D View this record in MEDLINE/PubMed |
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| Snippet | •Cerebellar microstructural damage affects Middle and Superior Cerebellar Peduncle in SCA2 patients.•Diffusivity changes predominantly reflect the white matter... Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum. The particular atrophy pattern results in... |
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| SubjectTerms | Adult Aged cerebellar peduncles cerebellum Cerebellum - diagnostic imaging Cerebellum - pathology Cognition Diffusion Magnetic Resonance Imaging Diffusion Tensor Imaging DTI Female Humans Male Middle Aged Neuropsychological Tests Spinocerebellar Ataxias - diagnostic imaging Spinocerebellar Ataxias - pathology Spinocerebellar Ataxias - psychology tractography white matter White Matter - diagnostic imaging White Matter - pathology |
| Title | Microstructural MRI Basis of the Cognitive Functions in Patients with Spinocerebellar Ataxia Type 2 |
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