FNIRS evaluation during a phonemic verbal task reveals prefrontal hypometabolism in patients affected by myotonic dystrophy type 1

Pietro Caliandro, Gabriella Silvestri, Luca Padua, Maria Laura Ester Bianchi, Chiara Simbolotti, Giuseppina Russo, Marcella Masciullo, Paolo Maria Rossini

Research output: Contribution to journalArticle

Abstract

Objective: Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy in adults, is characterized by a multisystem involvement. Cognitive involvement predominantly affecting frono-temporal functions is an established clinical feature in this disorder. Brain imaging and metabolic studies showed a predominant involvement of fronto-temporal regions in DM1 patients, yet correlation studies among these findings and neuropsychological data gave contrasting results. In order to contribute to clarify the relationship between the metabolic changes documented in the frontal cortex of DM1 patients and a related cognitive task, we applied the functional near-infrared spectroscopy (fNIRS) during the execution of a phonemic verbal fluency task (pVFT). Methods: We enrolled 29 consecutive right-handed DM1 patients and 30 controls. A 2-channel fNIRS imaging system was used to investigate changes in oxygenated [O2Hb] and deoxygenated [HHb] hemoglobin concentrations in the prefrontal cortex (PFC) during a pVFT. [O2Hb] and [HHb] baseline-corrected activation values were calculated (respectively [O2Hb]c and [HHb]c). Results: In the control group [O2Hb] significantly increased and [HHb] significantly decreased during the pVFT, in the DM1 group no significant variation was found for both parameters revealing no activation of both PFCs during the task. On the other hand, in the DM1 sample, statistical analysis revealed a direct correlation between [O2Hb]c of the left PFC and the pVFT score, while no correlation was observed in the control group. Conclusions: Our study reveals that DM1 patients show prefrontal hypometabolism during a specific frontal cognitive task compared to controls. Moreover the rapid temporal discrimination of fNIRS allows revealing the correlation between the PFC hypometabolism and the cognitive performance in DM1 patients. Significance: fNIRS can be helpful to understand the functional correlates of the frontal cognitive impairment in DM1.

Original languageEnglish
Pages (from-to)2269-2276
Number of pages8
JournalClinical Neurophysiology
Volume124
Issue number11
DOIs
Publication statusPublished - Nov 2013

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Myotonic Dystrophy
Near-Infrared Spectroscopy
Prefrontal Cortex
Control Groups
Muscular Dystrophies
Frontal Lobe
Temporal Lobe
Neuroimaging
Hemoglobins

Keywords

  • FNIRS
  • Myotonic dystrophy
  • Prefrontal cortex

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology
  • Physiology (medical)
  • Sensory Systems

Cite this

FNIRS evaluation during a phonemic verbal task reveals prefrontal hypometabolism in patients affected by myotonic dystrophy type 1. / Caliandro, Pietro; Silvestri, Gabriella; Padua, Luca; Bianchi, Maria Laura Ester; Simbolotti, Chiara; Russo, Giuseppina; Masciullo, Marcella; Rossini, Paolo Maria.

In: Clinical Neurophysiology, Vol. 124, No. 11, 11.2013, p. 2269-2276.

Research output: Contribution to journalArticle

Caliandro, Pietro ; Silvestri, Gabriella ; Padua, Luca ; Bianchi, Maria Laura Ester ; Simbolotti, Chiara ; Russo, Giuseppina ; Masciullo, Marcella ; Rossini, Paolo Maria. / FNIRS evaluation during a phonemic verbal task reveals prefrontal hypometabolism in patients affected by myotonic dystrophy type 1. In: Clinical Neurophysiology. 2013 ; Vol. 124, No. 11. pp. 2269-2276.
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abstract = "Objective: Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy in adults, is characterized by a multisystem involvement. Cognitive involvement predominantly affecting frono-temporal functions is an established clinical feature in this disorder. Brain imaging and metabolic studies showed a predominant involvement of fronto-temporal regions in DM1 patients, yet correlation studies among these findings and neuropsychological data gave contrasting results. In order to contribute to clarify the relationship between the metabolic changes documented in the frontal cortex of DM1 patients and a related cognitive task, we applied the functional near-infrared spectroscopy (fNIRS) during the execution of a phonemic verbal fluency task (pVFT). Methods: We enrolled 29 consecutive right-handed DM1 patients and 30 controls. A 2-channel fNIRS imaging system was used to investigate changes in oxygenated [O2Hb] and deoxygenated [HHb] hemoglobin concentrations in the prefrontal cortex (PFC) during a pVFT. [O2Hb] and [HHb] baseline-corrected activation values were calculated (respectively [O2Hb]c and [HHb]c). Results: In the control group [O2Hb] significantly increased and [HHb] significantly decreased during the pVFT, in the DM1 group no significant variation was found for both parameters revealing no activation of both PFCs during the task. On the other hand, in the DM1 sample, statistical analysis revealed a direct correlation between [O2Hb]c of the left PFC and the pVFT score, while no correlation was observed in the control group. Conclusions: Our study reveals that DM1 patients show prefrontal hypometabolism during a specific frontal cognitive task compared to controls. Moreover the rapid temporal discrimination of fNIRS allows revealing the correlation between the PFC hypometabolism and the cognitive performance in DM1 patients. Significance: fNIRS can be helpful to understand the functional correlates of the frontal cognitive impairment in DM1.",
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AU - Caliandro, Pietro

AU - Silvestri, Gabriella

AU - Padua, Luca

AU - Bianchi, Maria Laura Ester

AU - Simbolotti, Chiara

AU - Russo, Giuseppina

AU - Masciullo, Marcella

AU - Rossini, Paolo Maria

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N2 - Objective: Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy in adults, is characterized by a multisystem involvement. Cognitive involvement predominantly affecting frono-temporal functions is an established clinical feature in this disorder. Brain imaging and metabolic studies showed a predominant involvement of fronto-temporal regions in DM1 patients, yet correlation studies among these findings and neuropsychological data gave contrasting results. In order to contribute to clarify the relationship between the metabolic changes documented in the frontal cortex of DM1 patients and a related cognitive task, we applied the functional near-infrared spectroscopy (fNIRS) during the execution of a phonemic verbal fluency task (pVFT). Methods: We enrolled 29 consecutive right-handed DM1 patients and 30 controls. A 2-channel fNIRS imaging system was used to investigate changes in oxygenated [O2Hb] and deoxygenated [HHb] hemoglobin concentrations in the prefrontal cortex (PFC) during a pVFT. [O2Hb] and [HHb] baseline-corrected activation values were calculated (respectively [O2Hb]c and [HHb]c). Results: In the control group [O2Hb] significantly increased and [HHb] significantly decreased during the pVFT, in the DM1 group no significant variation was found for both parameters revealing no activation of both PFCs during the task. On the other hand, in the DM1 sample, statistical analysis revealed a direct correlation between [O2Hb]c of the left PFC and the pVFT score, while no correlation was observed in the control group. Conclusions: Our study reveals that DM1 patients show prefrontal hypometabolism during a specific frontal cognitive task compared to controls. Moreover the rapid temporal discrimination of fNIRS allows revealing the correlation between the PFC hypometabolism and the cognitive performance in DM1 patients. Significance: fNIRS can be helpful to understand the functional correlates of the frontal cognitive impairment in DM1.

AB - Objective: Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy in adults, is characterized by a multisystem involvement. Cognitive involvement predominantly affecting frono-temporal functions is an established clinical feature in this disorder. Brain imaging and metabolic studies showed a predominant involvement of fronto-temporal regions in DM1 patients, yet correlation studies among these findings and neuropsychological data gave contrasting results. In order to contribute to clarify the relationship between the metabolic changes documented in the frontal cortex of DM1 patients and a related cognitive task, we applied the functional near-infrared spectroscopy (fNIRS) during the execution of a phonemic verbal fluency task (pVFT). Methods: We enrolled 29 consecutive right-handed DM1 patients and 30 controls. A 2-channel fNIRS imaging system was used to investigate changes in oxygenated [O2Hb] and deoxygenated [HHb] hemoglobin concentrations in the prefrontal cortex (PFC) during a pVFT. [O2Hb] and [HHb] baseline-corrected activation values were calculated (respectively [O2Hb]c and [HHb]c). Results: In the control group [O2Hb] significantly increased and [HHb] significantly decreased during the pVFT, in the DM1 group no significant variation was found for both parameters revealing no activation of both PFCs during the task. On the other hand, in the DM1 sample, statistical analysis revealed a direct correlation between [O2Hb]c of the left PFC and the pVFT score, while no correlation was observed in the control group. Conclusions: Our study reveals that DM1 patients show prefrontal hypometabolism during a specific frontal cognitive task compared to controls. Moreover the rapid temporal discrimination of fNIRS allows revealing the correlation between the PFC hypometabolism and the cognitive performance in DM1 patients. Significance: fNIRS can be helpful to understand the functional correlates of the frontal cognitive impairment in DM1.

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