Pathologic and physiologic function in the subcortical band of double cortex

Briellmann, Regula S.; Little, T; Harvey, A. Simon; Abbott, David F.; Jacobs, Rani; Waites, Anthony B.; Jackson, Graeme D.

doi:10.1212/01.wnl.0000237554.39283.6b

Cited by 8 publications

(6 citation statements)

References 9 publications

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“…Functional connectivity analysis showed a functional involvement of laminar heterotopias in spontaneous, oscillatory cortical dynamics. The positive connectivity between laminar heterotopias and its corresponding migrated gray matter region matches previous evidence on DC syndrome obtained with task-fMRI investigations in which a simultaneously involvement with the corresponding overlying migrated cortex in sensorimotor and linguistic tasks was demonstrated ( Draganski et al, 2004 ; Keene et al, 2004 ; Briellmann et al, 2006 ; Jirsch et al, 2006 ). Additionally, in PNH patients ( Emiliano et al, 2012 ; Christodoulou et al, 2012 ) periventricular gray matter nodules showed synchronization with the overlying and surrounding cortex, as well as with the cerebellum bilaterally ( Emiliano et al, 2012 ).…”

Section: Discussionsupporting

confidence: 87%

“…Few cases of laminar PH displaying the MRI features of Double Cortex have been reported, without mutation of Double Cortin X ( Cappello et al, 2013 ), opening a debate about the specific link between the development of “double-cortex”-like laminar PH and Double Cortin X gene. No data on the genetic mutation characterizing these patients are available to date, while only a handful of studies have investigated possible alteration of FC MRI patterns exclusively in patients with classical PNH ( Archer et al, 2010 ; Christodoulou et al, 2012 , 2013 ; Emiliano et al, 2012 ; Shafi et al, 2015 ) and task fMRI studies, showing an integration of DC in the normal sensorimotor circuits ( Draganski et al, 2004 ; Keene et al, 2004 ; Jirsch et al, 2006 ; Briellmann et al, 2006 ).…”

Section: Introductionmentioning

confidence: 99%

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Functional Connectivity and Genetic Profile of a “Double-Cortex”-Like Malformation

Sprugnoli

Vatti

Rossi

et al. 2018

Front. Integr. Neurosci.

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Laminar heterotopia is a rare condition consisting in an extra layer of gray matter under properly migrated cortex; it configures an atypical presentation of periventricular nodular heterotopia (PNH) or a double cortex (DC) syndrome. We conducted an original functional MRI (fMRI) analysis in a drug-resistant epilepsy patient with “double-cortex”-like malformation to reveal her functional connectivity (FC) as well as a wide genetic analysis to identify possible genetic substrates. Heterotopias were segmented into region of interests (ROIs), whose voxel-wise FC was compared to that of (i) its normally migrated counterpart, (ii) its contralateral homologous, and (iii) those of 30 age-matched healthy controls. Extensive genetic analysis was conducted to screen cortical malformations-associated genes. Compared to healthy controls, both laminar heterotopias and the overlying cortex showed significant reduction of FC with the contralateral hemisphere. Two heterozygous variants of uncertain clinical significance were found, involving autosomal recessive disease-causing genes, FAT4 and COL18A1. This first FC analysis of a unique case of “double-cortex”-like malformation revealed a hemispheric connectivity segregation both in the laminar cortex as in the correctly migrated one, with a new pattern of genes’ mutations. Our study suggests the altered FC could have an electrophysiological and functional impact on large-scale brain networks, and the involvement of not yet identified genes in “double-cortex”-like malformation with a possible role of rare variants in recessive genes as pathogenic cofactors.

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Functional Connectivity and Genetic Profile of a “Double-Cortex”-Like Malformation

Sprugnoli

Vatti

Rossi

et al. 2018

Front. Integr. Neurosci.

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“…This ties in to broader questions, such as: Does activation in a region imply that it is necessary to support function ( Price et al, 1999 )? What is the clinical significance of activation in a region not typically activated in controls ( Anderson et al, 2002 ), or in abnormal/heterotopic tissue ( Briellmann et al, 2006b )? Does bilateral language activation in a region imply redundancy such that unilateral resection is safe?…”

Section: Discussionmentioning

confidence: 99%

The diminishing dominance of the dominant hemisphere: Language fMRI in focal epilepsy

Tailby

Abbott

Jackson

2017

NeuroImage: Clinical

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“Which is the dominant hemisphere?” is a question that arises frequently in patients considered for neurosurgery. The concept of the dominant hemisphere implies uniformity of language lateralisation throughout the brain. It is increasingly recognised that this is not the case in the healthy control brain, and it is especially not so in neurological diseases such as epilepsy.In the present work we adapt our published objective lateralisation method (based on the construction of laterality curves) for use with sub-lobar cortical, subcortical and cerebellar regions of interest (ROIs). We apply this method to investigate regional lateralisation of language activation in 12 healthy controls and 18 focal epilepsy patients, using three different block design language fMRI paradigms, each tapping different aspects of language processing. We compared lateralisation within each ROI across tasks, and investigated how the quantity of data collected affected the ability to robustly estimate laterality across ROIs.In controls, lateralisation was stronger, and the variance across individuals smaller, in cortical ROIs, particularly in the Inferior Frontal (Broca) region. Lateralisation within temporal ROIs was dependent on the nature of the language task employed. One of the healthy controls was left lateralised anteriorly and right lateralised posteriorly. Consistent with previous work, departures from normality occurred in ~ 15–50% of focal epilepsy patients across the different ROIs, with atypicality most common in the Lateral Temporal (Wernicke) region. Across tasks and ROIs the absolute magnitude of the laterality estimate increased and its across participant variance decreased as more cycles of task and rest were included, stabilising at ~ 4 cycles (~ 4 min of data collection).Our data highlight the importance of considering language as a complex task where lateralisation varies at the subhemispheric scale. This is especially important for presurgical planning for focal resections where the concept of ‘hemispheric dominance’ may be misleading. This is a precision medicine approach that enables objective evaluation of language dominance within specific brain regions and can reveal surprising and unexpected anomalies that may be clinically important for individual cases.

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“…Carbon fiber electrodes have been constructed at our institute and used in-house with our 3 T MRI scanner since the year 2000 with no adverse effects; for example, Ref. ( 7 – 14 ). We have also used carbon fiber cables and electrodes for intracranial EEG in sheep ( 15 ).…”

Section: Introductionmentioning

confidence: 99%

Constructing Carbon Fiber Motion-Detection Loops for Simultaneous EEGâ€“fMRI

et al. 2015

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One of the most significant impediments to high-quality EEG recorded in an MRI scanner is subject motion. Availability of motion artifact sensors can substantially improve the quality of the recorded EEG. In the study of epilepsy, it can also dramatically increase the confidence that one has in discriminating true epileptiform activity from artifact. This is due both to the reduction in artifact and the ability to visually inspect the motion sensor signals when reading the EEG, revealing whether or not head motion is present. We have previously described the use of carbon fiber loops for detecting and correcting artifact in EEG acquired simultaneously with MRI. The loops, attached to the subject’s head, are electrically insulated from the scalp. They provide a simple and direct measure of specific artifact that is contaminating the EEG, including both subject motion and residual artifact arising from magnetic field gradients applied during MRI. Our previous implementation was used together with a custom-built EEG–fMRI system that differs substantially from current commercially available EEG–fMRI systems. The present technical note extends this work, describing in more detail how to construct the carbon fiber motion-detection loops, and how to interface them with a commercially available simultaneous EEG–fMRI system. We hope that the information provided may help those wishing to utilize a motion-detection/correction solution to improve the quality of EEG recorded within an MRI scanner.

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Pathologic and physiologic function in the subcortical band of double cortex

Cited by 8 publications

References 9 publications

Functional Connectivity and Genetic Profile of a “Double-Cortex”-Like Malformation

Functional Connectivity and Genetic Profile of a “Double-Cortex”-Like Malformation

The diminishing dominance of the dominant hemisphere: Language fMRI in focal epilepsy

Constructing Carbon Fiber Motion-Detection Loops for Simultaneous EEGâ€“fMRI

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