Neurophysiological and BOLD signal uncoupling of giant somatosensory evoked potentials in progressive myoclonic epilepsy: A case-series study

Silvia F. Storti, Alessandra Del Felice, Laura Canafoglia, Emanuela Formaggio, Francesco Brigo, Franco Alessandrini, Luigi G. Bongiovanni, Gloria Menegaz, Paolo Manganotti

Research output: Contribution to journalArticle

Abstract

In progressive myoclonic epilepsy (PME), a rare epileptic syndrome caused by a variety of genetic disorders, the combination of peripheral stimulation and functional magnetic resonance imaging (fMRI) can shed light on the mechanisms underlying cortical dysfunction. The aim of the study is to investigate sensorimotor network modifications in PME by assessing the relationship between neurophysiological findings and blood oxygen level dependent (BOLD) activation. Somatosensory-evoked potential (SSEP) obtained briefly before fMRI and BOLD activation during median-nerve electrical stimulation were recorded in four subjects with typical PME phenotype and compared with normative data. Giant scalp SSEPs with enlarger N20-P25 complex compared to normal data (mean amplitude of 26.2 ± 8.2 μV after right stimulation and 27.9 ± 3.7 μV after left stimulation) were detected. Statistical group analysis showed a reduced BOLD activation in response to median nerve stimulation in PMEs compared to controls over the sensorimotor (SM) areas and an increased response over subcortical regions (p < 0.01, Z > 2.3, corrected). PMEs show dissociation between neurophysiological and BOLD findings of SSEPs (giant SSEP with reduced BOLD activation over SM). A direct pathway connecting a highly restricted area of the somatosensory cortex with the thalamus can be hypothesized to support the higher excitability of these areas.

Original languageEnglish
Article number44664
JournalScientific Reports
Volume7
DOIs
Publication statusPublished - Mar 15 2017

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Progressive Myoclonic Epilepsy
Somatosensory Evoked Potentials
Oxygen
Median Nerve
Magnetic Resonance Imaging
Inborn Genetic Diseases
Somatosensory Cortex
Scalp
Thalamus
Electric Stimulation
Phenotype

ASJC Scopus subject areas

  • General

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Neurophysiological and BOLD signal uncoupling of giant somatosensory evoked potentials in progressive myoclonic epilepsy : A case-series study. / Storti, Silvia F.; Del Felice, Alessandra; Canafoglia, Laura; Formaggio, Emanuela; Brigo, Francesco; Alessandrini, Franco; Bongiovanni, Luigi G.; Menegaz, Gloria; Manganotti, Paolo.

In: Scientific Reports, Vol. 7, 44664, 15.03.2017.

Research output: Contribution to journalArticle

Storti, SF, Del Felice, A, Canafoglia, L, Formaggio, E, Brigo, F, Alessandrini, F, Bongiovanni, LG, Menegaz, G & Manganotti, P 2017, 'Neurophysiological and BOLD signal uncoupling of giant somatosensory evoked potentials in progressive myoclonic epilepsy: A case-series study', Scientific Reports, vol. 7, 44664. https://doi.org/10.1038/srep44664
Storti, Silvia F. ; Del Felice, Alessandra ; Canafoglia, Laura ; Formaggio, Emanuela ; Brigo, Francesco ; Alessandrini, Franco ; Bongiovanni, Luigi G. ; Menegaz, Gloria ; Manganotti, Paolo. / Neurophysiological and BOLD signal uncoupling of giant somatosensory evoked potentials in progressive myoclonic epilepsy : A case-series study. In: Scientific Reports. 2017 ; Vol. 7.
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