Imaging Cerebral Activity in Amyotrophic Lateral Sclerosis

Proudfoot, Malcolm J.; Bede, Peter; Turner, Martin R

doi:10.3389/fneur.2018.01148

Cited by 66 publications

(54 citation statements)

References 166 publications

(202 reference statements)

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“…The functional reorganization of motor control has been previously linked to the recruitment of structurally less affected areas in ALS [5,12]. We observed increased striato-cerebellar connectivity in our UMNp cohort during movement preparation.…”

Section: Discussionsupporting

confidence: 64%

“…Pre‐symptomatic studies of ALS‐associated mutation carriers have highlighted that considerable structural changes can be detected long before symptom onset . Functional imaging studies of ALS suggest that a number of compensatory processes take place in the face of progressive motor cortex degeneration and activation patterns shift from the precentral gyrus to supplementary motor, pre‐motor and cerebellar regions during movement execution . Despite the plethora of imaging studies in ALS , most studies admix heterogeneous patient cohorts without stratifying them based on their genotype, cognitive profile or upper motor neuron (UMN) versus lower motor neuron (LMN) predominance .…”

Section: Introductionmentioning

confidence: 99%

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Adaptive functional reorganization in amyotrophic lateral sclerosis: coexisting degenerative and compensatory changes

Abidi

Marco

Couillandre

et al. 2019

Euro J of Neurology

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Background and purpose Considerable functional reorganization takes place in amyotrophic lateral sclerosis (ALS) in face of relentless structural degeneration. This study evaluates functional adaptation in ALS patients with lower motor neuron predominant (LMNp) and upper motor neuron predominant (UMNp) dysfunction. Methods Seventeen LMNp ALS patients, 14 UMNp ALS patients and 14 controls participated in a functional magnetic resonance imaging study. Study‐group‐specific activation patterns were evaluated during preparation for a motor task. Connectivity analyses were carried out using the supplementary motor area (SMA), cerebellum and striatum as seed regions and correlations were explored with clinical measures. Results Increased cerebellar, decreased dorsolateral prefrontal cortex and decreased SMA activation were detected in UMNp patients compared to controls. Increased cerebellar activation was also detected in UMNp patients compared to LMNp patients. UMNp patients exhibit increased effective connectivity between the cerebellum and caudate, and decreased connectivity between the SMA and caudate and between the SMA and cerebellum when performing self‐initiated movement. In UMNp patients, a positive correlation was detected between clinical variables and striato‐cerebellar connectivity. Conclusions Our findings indicate that, despite the dysfunction of SMA–striatal and SMA–cerebellar networks, cerebello‐striatal connectivity increases in ALS indicative of compensatory processes. The coexistence of circuits with decreased and increased connectivity suggests concomitant neurodegenerative and adaptive changes in ALS.

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Section: Discussionsupporting

confidence: 64%

Section: Introductionmentioning

confidence: 99%

Adaptive functional reorganization in amyotrophic lateral sclerosis: coexisting degenerative and compensatory changes

Abidi

Marco

Couillandre

et al. 2019

Euro J of Neurology

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“…1 The evidence that has accumulated over recent decades of widespread involvement of the extramotor system has changed the conventional concept of ALS to a multisystem degenerative disease. [2][3][4][5] Up to 60% of patients with ALS exhibit some form of measurable cognitive impairment, and patients with ALS combined with frontotemporal dementia (FTD) are frequently encountered. 6 Moreover, the coexistence of extrapyramidal motor symptoms such as rigidity, bradykinesia, and postural instability is not uncommon in ALS.…”

Section: Introductionmentioning

confidence: 99%

Shape Analysis of the Subcortical Nuclei in Amyotrophic Lateral Sclerosis without Cognitive Impairment

et al. 2020

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Background and Purpose Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that mainly affects the pyramidal motor system. However, recent studies have suggested that degeneration of the extramotor system plays a role in the disability experienced by patients with ALS. We investigated the local shape changes and mean volumes of the subcortical nuclei in sporadic ALS patients with preserved cognition. Methods The participants comprised 32 patients with ALS and 43 age-and sex-matched healthy controls. Three-dimensional T1-weighted structural images were acquired. Surfacebased vertex analysis was performed with fully automated segmentation of both amygdalae, hippocampi, caudate nuclei, nuclei accumbens, putamina, pallida, and thalami, and the brainstem. The scalar distances from the mean surfaces of the individual subcortical nuclei were compared between groups, and correlations of the local shape distances with initial Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALS-FRS-R) scores and the delta FRS-R and with the disease duration were analyzed. Results ALS patients showed regional shape contractions on the lateral surfaces of both pallida, the lateroposterior surface of the right putamen, and the anterior basal surface of the right accumbens. Delta FRS-R scores were negatively correlated with local shape distances in the right hippocampus and the putamina. However, the initial ALS-FRS-R score and disease duration were not correlated with local shape distances. Conclusions Subcortical gray-matter structures are involved in the neurodegenerative process of ALS before cognitive impairment becomes evident.

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“…Functional MRI (fMRI) detects local variations in blood oxygenation level-dependent MR signal at rest and during activation paradigms (128). FMRI has been extensively applied to ALS cohorts to describe network changes and assess altered activation patterns when performing motor or cognitive tasks (129–131).…”

Section: Spinal Cord Imaging In Alsmentioning

confidence: 99%

Spinal Cord Imaging in Amyotrophic Lateral Sclerosis: Historical Concepts—Novel Techniques

et al. 2019

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Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neuron disease with no effective disease modifying therapies at present. Spinal cord degeneration is a hallmark feature of ALS, highlighted in the earliest descriptions of the disease by Lockhart Clarke and Jean-Martin Charcot. The anterior horns and corticospinal tracts are invariably affected in ALS, but up to recently it has been notoriously challenging to detect and characterize spinal pathology in vivo . With recent technological advances, spinal imaging now offers unique opportunities to appraise lower motor neuron degeneration, sensory involvement, metabolic alterations, and interneuron pathology in ALS. Quantitative spinal imaging in ALS has now been used in cross-sectional and longitudinal study designs, applied to presymptomatic mutation carriers, and utilized in machine learning applications. Despite its enormous clinical and academic potential, a number of physiological, technological, and methodological challenges limit the routine use of computational spinal imaging in ALS. In this review, we provide a comprehensive overview of emerging spinal cord imaging methods and discuss their advantages, drawbacks, and biomarker potential in clinical applications, clinical trial settings, monitoring, and prognostic roles.

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Imaging Cerebral Activity in Amyotrophic Lateral Sclerosis

Cited by 66 publications

References 166 publications

Adaptive functional reorganization in amyotrophic lateral sclerosis: coexisting degenerative and compensatory changes

Adaptive functional reorganization in amyotrophic lateral sclerosis: coexisting degenerative and compensatory changes

Shape Analysis of the Subcortical Nuclei in Amyotrophic Lateral Sclerosis without Cognitive Impairment

Spinal Cord Imaging in Amyotrophic Lateral Sclerosis: Historical Concepts—Novel Techniques

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