Reduced cortical activation in inferior frontal junction in Unverricht-Lundborg disease (EPM1) - A motor fMRI study

Epilepsy Research
Mervi KönönenE Niskanen

Abstract

Unverricht-Lundborg disease (EPM1) is characterized by stimulus-sensitive and action-activated myoclonus, tonic-clonic seizures and ataxia. Several disease-related alterations in cortical structure and excitability have been associated with the motor symptoms of EPM1. This study aimed to elucidate possible alterations in cortical activation related to motor performance in EPM1. Fifteen EPM1-patients and 15 healthy volunteers matched for age and sex underwent motor functional MRI. Group differences in activations were evaluated in the primary and supplementary motor cortices and sensory cortical areas. Furthermore, in EPM1 patients, the quantitative fMRI parameters were correlated with the severity of the motor symptoms. The EPM1-patients exhibited decreased activation in the left inferior frontal junction (IFJ) during right hand voluntary motor task when compared with controls. In the quantitative analysis, EPM1-patients had significantly weaker activation than controls in the hand knob and supplementary motor areas (SMA). The volume of activation in M1 decreased with age and duration of disease in the patient group, whereas the volume increased with age in controls. Negative correlations were observed between fMRI parameters o...Continue Reading

References

Jun 2, 2005·Trends in Cognitive Sciences·Marcel BrassD Yves von Cramon
Dec 13, 2006·Epilepsy Research·Edoardo FerlazzoPierre Genton
Mar 8, 2008·Epilepsia·Reetta KälviäinenEsa Mervaala
Dec 9, 2010·Human Brain Mapping·Egbert HartstraMarcel Brass
Jun 11, 2011·Movement Disorders : Official Journal of the Movement Disorder Society·Nils DannerEsa Mervaala
Aug 9, 2013·Movement Disorders : Official Journal of the Movement Disorder Society·Nils DannerEsa Mervaala

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Citations

Jun 26, 2021·Epilepsy & Behavior : E&B·Marja ÄikiäReetta Kälviäinen

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