Neural substrates of motor and cognitive dysfunctions in SCA2 patients: A network based statistics analysis
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiat...
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| Vydáno v: | NeuroImage clinical Ročník 14; číslo C; s. 719 - 725 |
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| Médium: | Journal Article |
| Jazyk: | angličtina |
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Elsevier Inc
01.01.2017
Elsevier |
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| ISSN: | 2213-1582, 2213-1582 |
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| Abstract | Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms.
The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients.
In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral “nodes” in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes.
•A cerebellar dysfunction affects long-distance cerebral regions in SCA2 patients.•Connectivity changes affect sensorimotor and cognitive cerebello-cortical nodes.•Cerebellar symptoms may be related to altered cerebello-cerebral connectivity. |
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| AbstractList | Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms. The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients. In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral "nodes" in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes. AbstractSpinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms. The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients. In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral “nodes” in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes. Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms. The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients. In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral “nodes” in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes. •A cerebellar dysfunction affects long-distance cerebral regions in SCA2 patients.•Connectivity changes affect sensorimotor and cognitive cerebello-cortical nodes.•Cerebellar symptoms may be related to altered cerebello-cerebral connectivity. Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms. The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients. In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral "nodes" in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes.Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms. The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients. In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral "nodes" in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes. Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms. The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients. In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral “nodes” in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes. • A cerebellar dysfunction affects long-distance cerebral regions in SCA2 patients. • Connectivity changes affect sensorimotor and cognitive cerebello-cortical nodes. • Cerebellar symptoms may be related to altered cerebello-cerebral connectivity. |
| Author | Leggio, M. Cercignani, M. Romano, S. Masciullo, M. Bozzali, M. Olivito, G. Clausi, S. Molinari, M. Iacobacci, C. Lupo, M. |
| AuthorAffiliation | b Neuroimaging Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy d Department of Psychology, Faculty of Medicine and Psychology, “Sapienza” University of Rome, Rome, Italy f SPInal REhabilitation Lab, IRCCS Fondazione Santa Lucia,Rome, Italy g Neurorehabilitation 1 and Spinal Center, Robotic Neurorehabilitation Lab, IRCCS Santa Lucia Foundation, Rome, Italy e Department of Neurosciences, Mental Health and Sensory Organs (NESMOS), “Sapienza” University of Rome–Sant'Andrea Hospital, Rome, Italy a Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy c Clinical Imaging Science Center, Brighton and Sussex Medical School, Brighton, UK |
| AuthorAffiliation_xml | – name: a Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – name: c Clinical Imaging Science Center, Brighton and Sussex Medical School, Brighton, UK – name: d Department of Psychology, Faculty of Medicine and Psychology, “Sapienza” University of Rome, Rome, Italy – name: b Neuroimaging Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – name: f SPInal REhabilitation Lab, IRCCS Fondazione Santa Lucia,Rome, Italy – name: g Neurorehabilitation 1 and Spinal Center, Robotic Neurorehabilitation Lab, IRCCS Santa Lucia Foundation, Rome, Italy – name: e Department of Neurosciences, Mental Health and Sensory Organs (NESMOS), “Sapienza” University of Rome–Sant'Andrea Hospital, Rome, Italy |
| 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: Cercignani fullname: Cercignani, M. organization: Neuroimaging Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 3 givenname: M. surname: Lupo fullname: Lupo, M. organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 4 givenname: C. surname: Iacobacci fullname: Iacobacci, C. organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 5 givenname: S. surname: Clausi fullname: Clausi, S. organization: Ataxia Laboratory, IRCCS Santa Lucia Foundation, Rome, Italy – sequence: 6 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: 7 givenname: M. surname: Masciullo fullname: Masciullo, M. organization: SPInal REhabilitation Lab, IRCCS Fondazione Santa Lucia,Rome, Italy – sequence: 8 givenname: M. surname: Molinari fullname: Molinari, M. organization: Neurorehabilitation 1 and Spinal Center, Robotic Neurorehabilitation Lab, 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/28393013$$D View this record in MEDLINE/PubMed |
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| Snippet | Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be... AbstractSpinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can... |
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| SubjectTerms | Adult Aged Brain Mapping Cerebellum Cerebellum - diagnostic imaging Cerebral cortex Cognition Disorders - diagnostic imaging Cognition Disorders - etiology Female Functional connectivity Humans Image Processing, Computer-Assisted Magnetic Resonance Imaging Male Middle Aged Motor Disorders - diagnostic imaging Motor Disorders - etiology Nerve Net - diagnostic imaging Nodes Oxygen - blood Radiology Regular Rest Resting-state fMRI Severity of Illness Index Spinocerebellar Ataxias - complications |
| Title | Neural substrates of motor and cognitive dysfunctions in SCA2 patients: A network based statistics analysis |
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