Sensorimotor, Visual, and Auditory Cortical Atrophy in Unverricht-Lundborg Disease Mapped with Cortical Thickness Analysis

Koskenkorva, Päivi; Niskanen, Eini; Hyppönen, Jelena; Könönen, Mervi; Mervaala, Esa; Soininen, H.; Kälviäinen, Reetta; Vanninen, Ritva

doi:10.3174/ajnr.a2882

Cited by 26 publications

(30 citation statements)

References 38 publications

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“…Heinzel et al 2013 have recently reported that up to 41 % of the variance in the HHb signal and up to 18 % of the variance in the O 2 Hb signal in frontal regions can be explained by individual grey matter volume; moreover, differences in the distance between the scalp and cortex may affect the sensitivity of fNIRS (Haeussinger et al 2011). As in previous studies of EPM1 patients (Koskenkorva et al , 2012, we found that motor area CT was moderately reduced in our patients, and correlated with the reduction in the hemodynamic signals.…”

Section: Movement-related Eeg Changes and Hemodynamicsmentioning

confidence: 80%

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

et al. 2014

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Multimodal human brain mapping has been proposed as an integrated approach capable of improving the recognition of the cortical correlates of specific neurological functions. We used simultaneous EEG-fMRI (functional magnetic resonance imaging) and EEG-TDfNIRS (time domain functional near-infrared spectroscopy) recordings to compare different hemodynamic methods with changes in EEG in ten patients with progressive myoclonic epilepsy and 12 healthy controls. We evaluated O 2 Hb, HHb and Blood oxygen level-dependent (BOLD) changes and event-related desynchronization/synchronization (ERD/ERS) in the a and b bands of all of the subjects while they performed a simple motor task. The general linear model was used to obtain comparable fMRI and TDfNIRS activation maps. We also analyzed cortical thickness in order to evaluate any structural changes. In the patients, the TD-NIRS and fMRI data significantly correlated and showed a significant lessening of the increase in O 2 Hb and the decrease in BOLD. The post-movement b rebound was minimal or absent in patients. Cortical thickness was moderately reduced in the motor area of the patients and correlated with the reduction in the hemodynamic signals. The fMRI and TD-NIRS results were consistent, significantly correlated and showed smaller hemodynamic changes in the patients. This finding may be partially attributable to mild cortical thickening. However, cortical hyperexcitability, which is known to generate myoclonic jerks and probably accounts for the lack of EEG b-ERS, did not reflect any increased energy requirement. We hypothesize that this is due to a loss of inhibitory neuronal components that typically fire at high frequencies.

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Section: Movement-related Eeg Changes and Hemodynamicsmentioning

confidence: 80%

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

et al. 2014

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“…However, changes in MRI, such as mild to moderate cerebral and/or cerebellar atrophy, loss of neuronal volume in the brainstem and high intensity signal changes in the basal ganglia have been reported as well [7], [10]–[12]. Recently modern group level MRI analysis methods have revealed loss of gray matter volume in cortical motor areas (voxel-based morphometry, VBM), and atrophy of the sensorimotor, visual and auditory cortices (cortical thickness analysis, CTH) in EPM1 patients [13], [14] while abnormal findings could not be detected in visual assessments. Loss of gray matter volume in the thalamus has also been reported in one VBM study [13], paralleling a PET study indicating dopamine depletion in the thalamostriatal area in four EPM1 patients [15].…”

Section: Introductionmentioning

confidence: 99%

3D Texture Analysis Reveals Imperceptible MRI Textural Alterations in the Thalamus and Putamen in Progressive Myoclonic Epilepsy Type 1, EPM1

et al. 2013

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Progressive myoclonic epilepsy type 1 (EPM1) is an autosomal recessively inherited neurodegenerative disorder characterized by young onset age, myoclonus and tonic-clonic epileptic seizures. At the time of diagnosis, the visual assessment of the brain MRI is usually normal, with no major changes found later. Therefore, we utilized texture analysis (TA) to characterize and classify the underlying properties of the affected brain tissue by means of 3D texture features. Sixteen genetically verified patients with EPM1 and 16 healthy controls were included in the study. TA was performed upon 3D volumes of interest that were placed bilaterally in the thalamus, amygdala, hippocampus, caudate nucleus and putamen. Compared to the healthy controls, EPM1 patients had significant textural differences especially in the thalamus and right putamen. The most significantly differing texture features included parameters that measure the complexity and heterogeneity of the tissue, such as the co-occurrence matrix-based entropy and angular second moment, and also the run-length matrix-based parameters of gray-level non-uniformity, short run emphasis and long run emphasis. This study demonstrates the usability of 3D TA for extracting additional information from MR images. Textural alterations which suggest complex, coarse and heterogeneous appearance were found bilaterally in the thalamus, supporting the previous literature on thalamic pathology in EPM1. The observed putamenal involvement is a novel finding. Our results encourage further studies on the clinical applications, feasibility, reproducibility and reliability of 3D TA.

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“…A voxelbased morphometry (VBM) analysis revealed atrophy in primary, premotor, and supplementary motor cortices bilaterally . Furthermore, a cortical thickness analysis found that EPM1 patients had thinner sensorimotor, visual and auditory cortices than controls (Koskenkorva et al, 2012). Cortical thickness was shown to correlate negatively with age, duration of EPM1, and clinical severity of myoclonus.…”

Section: Introductionmentioning

confidence: 98%

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

et al. 2015

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Sensorimotor, Visual, and Auditory Cortical Atrophy in Unverricht-Lundborg Disease Mapped with Cortical Thickness Analysis

Abstract: Cortical thinning in the sensorimotor areas in EPM1 correlated significantly with the degree of the severity of the myoclonus and is most likely related to the widespread stimulus sensitivity in EPM1.

Cited by 26 publications

References 38 publications

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

3D Texture Analysis Reveals Imperceptible MRI Textural Alterations in the Thalamus and Putamen in Progressive Myoclonic Epilepsy Type 1, EPM1

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

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