Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper Arm
We recently investigated the role of the cerebellum during development, reporting that children with genetic slow-progressive ataxia (SlowP) show worse postural control during quiet stance and gait initiation compared to healthy children (H). Instead, children with genetic non-progressive ataxia (No...
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MDPI AG
2023-04-01
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| Series: | Brain Sciences |
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| Online Access: | https://www.mdpi.com/2076-3425/13/4/620 |
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| author | Silvia Maria Marchese Roberto Esposti Veronica Farinelli Claudia Ciaccio Arianna De Laurentiis Stefano D’Arrigo Paolo Cavallari |
| author_facet | Silvia Maria Marchese Roberto Esposti Veronica Farinelli Claudia Ciaccio Arianna De Laurentiis Stefano D’Arrigo Paolo Cavallari |
| author_sort | Silvia Maria Marchese |
| collection | DOAJ |
| description | We recently investigated the role of the cerebellum during development, reporting that children with genetic slow-progressive ataxia (SlowP) show worse postural control during quiet stance and gait initiation compared to healthy children (H). Instead, children with genetic non-progressive ataxia (NonP) recalled the behavior of H. This may derive from compensatory networks, which are hindered by disease progression in SlowP while free to develop in NonP. In the aim of extending our findings to <i>intra-limb</i> postural control, we recorded, in 10 NonP, 10 SlowP and 10 H young patients, Anticipatory Postural Adjustments (APAs) in the proximal muscles of the upper-limb and preceding brisk index finger flexions. No significant differences in APA timing occurred between NonP and H, while APAs in SlowP were delayed. Indeed, the excitatory APA in Triceps Brachii was always present but significantly delayed with respect to both H and NonP. Moreover, the inhibitory APAs in the Biceps Brachii and Anterior Deltoid, which are normally followed by a late excitation, could not be detected in most SlowP children, as if inhibition was delayed to the extent where there was overlap with a late excitation. In conclusion, disease progression seems to be detrimental for <i>intra-limb</i> posture, supporting the idea that <i>inter-</i> and <i>intra-limb</i> postures seemingly share the same control mechanism. |
| first_indexed | 2024-03-11T05:10:53Z |
| format | Article |
| id | doaj.art-3bfc9d5f32de4a1596737fb8649d104e |
| institution | Directory Open Access Journal |
| issn | 2076-3425 |
| language | English |
| last_indexed | 2024-03-11T05:10:53Z |
| publishDate | 2023-04-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Brain Sciences |
| spelling | doaj.art-3bfc9d5f32de4a1596737fb8649d104e2023-11-17T18:32:45ZengMDPI AGBrain Sciences2076-34252023-04-0113462010.3390/brainsci13040620Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper ArmSilvia Maria Marchese0Roberto Esposti1Veronica Farinelli2Claudia Ciaccio3Arianna De Laurentiis4Stefano D’Arrigo5Paolo Cavallari6Human Physiology Section of the DePT, Università degli Studi di Milano, 20133 Milan, ItalyHuman Physiology Section of the DePT, Università degli Studi di Milano, 20133 Milan, ItalyHuman Physiology Section of the DePT, Università degli Studi di Milano, 20133 Milan, ItalyDepartment of Pediatric Neuroscience, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, 20133 Milan, ItalyDepartment of Pediatric Neuroscience, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, 20133 Milan, ItalyDepartment of Pediatric Neuroscience, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, 20133 Milan, ItalyHuman Physiology Section of the DePT, Università degli Studi di Milano, 20133 Milan, ItalyWe recently investigated the role of the cerebellum during development, reporting that children with genetic slow-progressive ataxia (SlowP) show worse postural control during quiet stance and gait initiation compared to healthy children (H). Instead, children with genetic non-progressive ataxia (NonP) recalled the behavior of H. This may derive from compensatory networks, which are hindered by disease progression in SlowP while free to develop in NonP. In the aim of extending our findings to <i>intra-limb</i> postural control, we recorded, in 10 NonP, 10 SlowP and 10 H young patients, Anticipatory Postural Adjustments (APAs) in the proximal muscles of the upper-limb and preceding brisk index finger flexions. No significant differences in APA timing occurred between NonP and H, while APAs in SlowP were delayed. Indeed, the excitatory APA in Triceps Brachii was always present but significantly delayed with respect to both H and NonP. Moreover, the inhibitory APAs in the Biceps Brachii and Anterior Deltoid, which are normally followed by a late excitation, could not be detected in most SlowP children, as if inhibition was delayed to the extent where there was overlap with a late excitation. In conclusion, disease progression seems to be detrimental for <i>intra-limb</i> posture, supporting the idea that <i>inter-</i> and <i>intra-limb</i> postures seemingly share the same control mechanism.https://www.mdpi.com/2076-3425/13/4/620genetic ataxiageneralized cerebellar atrophycerebellar vermis hypoplasiapostural controlcompensatory strategies |
| spellingShingle | Silvia Maria Marchese Roberto Esposti Veronica Farinelli Claudia Ciaccio Arianna De Laurentiis Stefano D’Arrigo Paolo Cavallari Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper Arm Brain Sciences genetic ataxia generalized cerebellar atrophy cerebellar vermis hypoplasia postural control compensatory strategies |
| title | Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper Arm |
| title_full | Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper Arm |
| title_fullStr | Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper Arm |
| title_full_unstemmed | Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper Arm |
| title_short | Pediatric Slow-Progressive, but Not Non-Progressive Cerebellar Ataxia Delays Intra-Limb Anticipatory Postural Adjustments in the Upper Arm |
| title_sort | pediatric slow progressive but not non progressive cerebellar ataxia delays intra limb anticipatory postural adjustments in the upper arm |
| topic | genetic ataxia generalized cerebellar atrophy cerebellar vermis hypoplasia postural control compensatory strategies |
| url | https://www.mdpi.com/2076-3425/13/4/620 |
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