scholarly journals Cerebral atrophy in amyotrophic lateral sclerosis parallels the pathological distribution of TDP43

2020 ◽  
Vol 2 (2) ◽  
Author(s):  
Mahsa Dadar ◽  
Ana Laura Manera ◽  
Lorne Zinman ◽  
Lawrence Korngut ◽  
Angela Genge ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
White Matter ◽  
Motor Cortex ◽  
Grey Matter ◽  
Corticospinal Tract ◽  
Cerebral Atrophy ◽  
Precentral Gyrus ◽  
Cerebral Disease ◽  
Deformation Based Morphometry ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis is a neurodegenerative disease characterized by a preferential involvement of both upper and lower motor neurons. Evidence from neuroimaging and post-mortem studies confirms additional involvement of brain regions extending beyond the motor cortex. The aim of this study was to assess the extent of cerebral disease in amyotrophic lateral sclerosis cross-sectionally and longitudinally and to compare the findings with a recently proposed disease-staging model of amyotrophic lateral sclerosis pathology. Deformation-based morphometry was used to identify the patterns of brain atrophy associated with amyotrophic lateral sclerosis and to assess their relationship with clinical symptoms. Longitudinal T1-weighted MRI data and clinical measures were acquired at baseline, 4 months and 8 months, from 66 patients and 43 age-matched controls who participated in the Canadian Amyotrophic Lateral Sclerosis Neuroimaging Consortium study. Whole brain voxel-wise mixed-effects modelling analysis showed extensive atrophy patterns differentiating patients from the normal controls. Cerebral atrophy was present in the motor cortex and corticospinal tract, involving both grey matter and white matter, and to a lesser extent in non-motor regions. More specifically, the results showed significant bilateral atrophy in the motor cortex and corticospinal tract (including the internal capsule and brainstem) and ventricular enlargement, along with significant longitudinal atrophy in precentral gyrus, frontal and parietal white matter, accompanied by ventricular and sulcal enlargement. Atrophy in the precentral gyrus was significantly associated with greater disability as quantified with the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (P < 0.0001). The pattern of atrophy observed using deformation-based morphometry was consistent with the Brettschneider’s four-stage pathological model of the disease. Deformation-based morphometry provides a sensitive indicator of atrophy in Amyotrophic lateral sclerosis and has potential as a biomarker of disease burden, in both grey matter and white matter.

scholarly journals Cerebral Atrophy in Amyotrophic Lateral Sclerosis Parallels the Pathological Distribution of TDP43

2020 ◽  
Author(s):  
Mahsa Dadar ◽  
Ana Laura Manera ◽  
Lorne Zinman ◽  
Lawrence Korngut ◽  
Angela Genge ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
White Matter ◽  
Motor Cortex ◽  
Corticospinal Tract ◽  
Cerebral Atrophy ◽  
Precentral Gyrus ◽  
Cerebral Disease ◽  
Als Patients ◽  
Deformation Based Morphometry ◽  
Lateral Sclerosis

AbstractAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a preferential involvement of both upper and lower motor neurons. Evidence from neuroimaging and post-mortem studies confirms additional involvement of brain regions extending beyond the motor cortex. The aim of this study was to assess the extent of cerebral disease in ALS cross-sectionally and longitudinally, and to compare the findings with a recently proposed disease-staging model of ALS pathology. Deformation-based morphometry (DBM) was used to identify the patterns of brain atrophy associated with ALS and to assess their relationship with clinical symptoms. Longitudinal T1-weighted MRI data and clinical measures were acquired at baseline, 4 months, and 8 months, from 66 ALS patients and 43 age-matched controls who participated in the Canadian ALS Neuroimaging Consortium (CALSNIC) study. Whole brain voxel-wise mixed-effects modelling analysis showed extensive atrophy patterns differentiating ALS patients from the normal controls. Cerebral atrophy was present in the motor cortex and corticospinal tract, involving both GM and WM, and to a lesser extent in non-motor regions. More specifically, the results showed significant bilateral atrophy in the motor cortex, the corticospinal tract including the internal capsule and brainstem, with an overall pattern of ventricular enlargement; along with significant progressive longitudinal atrophy in the precentral gyrus, frontal and parietal white matter, accompanied by ventricular and sulcal enlargement. Atrophy in the precentral gyrus was significantly associated with greater disability as quantified with the ALS Functional Rating Scale-Revised (ALSFRS-R) (p<0.0001). The pattern of atrophy observed using DBM was consistent with the Brettschneider’s four stage pathological model of the disease. Deformation based morphometry provides a sensitive indicator of atrophy in ALS, and has potential as a biomarker of disease burden, in both gray and white matter.

Detection of motor cortex thinning and corticospinal tract involvement by quantitative MRI in amyotrophic lateral sclerosis

2009 ◽  
Vol 10 (1) ◽  
pp. 47-52 ◽  
Author(s):  
Luca Roccatagliata ◽  
Laura Bonzano ◽  
Gianluigi Mancardi ◽  
Cinzia Canepa ◽  
Claudia Caponnetto
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Cortex ◽  
Corticospinal Tract ◽  
Quantitative Mri ◽  
Tract Involvement ◽  
Lateral Sclerosis

scholarly journals Voxel-based morphometry in amyotrophic lateral sclerosis

2019 ◽  
Vol 99 (6) ◽  
pp. 287-294
Author(s):  
I. S. Bakulin ◽  
R. N. Konovalov ◽  
M. V. Krotenkova ◽  
N. A. Suponeva ◽  
M. N. Zakharova
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Grey Matter ◽  
Middle Part ◽  
Grey Matter Volume ◽  
Precentral Gyrus ◽  
Healthy Controls ◽  
Voxel Based Morphometry ◽  
Matter Volume ◽  
Occipital Lobes ◽  
Lateral Sclerosis

Objective:to investigate changes in grey matter volume in patients with classical amyotrophic lateral sclerosis (ALS) and lower motor neuron syndrome (LMNS) with voxel-based morphometry (VBM).Material and methods. 30 patients with classical ALS, 22 patients with LMNS and 23 age and gender matched healthy controls were enrolled in this study. All participants underwent a T1MPR (multiplanar reconstruction) magnetic resonance imaging with post-processing included spatial normalization, segmentation and smoothing. VBM was used to investigate changes in grey matter volume across the groups.Results. There was a significant decrease in grey matter volume of middle part of left pre- and postcentral gyri, middle part of right precentral gyrus, right and left occipital lobes in patients with classical ALS compared to healthy subjects. There was no difference in grey matter volume between patients with LMNS and healthy controls. Patients with classical ALS showed a significant decrease in grey matter volume of middle part of left preand postcentral gyri, upper part of left precentral gyrus, middle and upper parts of right precentral gyrus, right and left occipital lobes compared to patients with LMNS. There was no significant correlation between grey matter volume and clinical findings in patients with ALS and LMNS.Conclusion.VBM reveals a decrease in grey matter volume of motor and nonmotor brain regions in patients with classical ALS, but not in patients with LMNS.

scholarly journals Cross-sectional and longitudinal measures of chitinase proteins in amyotrophic lateral sclerosis and expression of CHI3L1 in activated astrocytes

2020 ◽  
Vol 91 (4) ◽  
pp. 350-358 ◽  
Author(s):  
Lucas Vu ◽  
Jiyan An ◽  
Tina Kovalik ◽  
Tania Gendron ◽  
Leonard Petrucelli ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
White Matter ◽  
Motor Cortex ◽  
Complex Disease ◽  
Cell Types ◽  
Cross Sectional ◽  
Tissue Sections ◽  
Disease Mechanisms ◽  
Homogeneous Subset ◽  
Lateral Sclerosis

ObjectiveAmyotrophic lateral sclerosis (ALS) is a complex disease with numerous pathological mechanisms resulting in a heterogeneous patient population. Using biomarkers for particular disease mechanisms may enrich a homogeneous subset of patients. In this study, we quantified chitotriosidase (Chit-1) and chitinase-3-like protein 1 (CHI3L1), markers of glial activation, in cerebrospinal fluid (CSF) and plasma and determined the cell types that express CHI3L1 in ALS.MethodsImmunoassays were used to quantify Chit-1, CHI3L1 and phosphorylated neurofilament heavy chain levels in longitudinal CSF and matching plasma samples from 118 patients with ALS, 17 disease controls (DCs), and 24 healthy controls (HCs). Immunostaining was performed to identify and quantify CHI3L1-positive cells in tissue sections from ALS, DCs and non-neurological DCs.ResultsCSF Chit-1 exhibited increased levels in ALS as compared with DCs and HCs. CSF CHI3L1 levels were increased in ALS and DCs compared with HCs. No quantitative differences were noted in plasma for either chitinase. Patients with ALS with fast-progressing disease exhibited higher levels of CSF Chit-1 and CHI3L1 than patients with slow-progressing disease. Increased numbers of CHI3L1-positive cells were observed in postmortem ALS motor cortex as compared with controls, and these cells were identified as a subset of activated astrocytes located predominately in the white matter of the motor cortex and the spinal cord.ConclusionsCSF Chit-1 and CHI3L1 are significantly increased in ALS, and CSF Chit-1 and CHI3L1 levels correlate to the rate of disease progression. CHI3L1 is expressed by a subset of activated astrocytes predominately located in white matter.

scholarly journals White matter structural network abnormalities underlie executive dysfunction in amyotrophic lateral sclerosis

2016 ◽  
Vol 38 (3) ◽  
pp. 1249-1268 ◽  
Author(s):  
Dennis Dimond ◽  
Abdullah Ishaque ◽  
Sneha Chenji ◽  
Dennell Mah ◽  
Zhang Chen ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
White Matter ◽  
Executive Dysfunction ◽  
Structural Network ◽  
Lateral Sclerosis

Intraoperative Motor Evoked Potential Alteration in Intracranial Tumor Surgery and Its Relation to Signal Alteration in Postoperative Magnetic Resonance Imaging

Neurosurgery ◽  
2010 ◽  
Vol 67 (2) ◽  
pp. 302-313 ◽  
Author(s):  
Andrea Szelényi ◽  
Elke Hattingen ◽  
Stefan Weidauer ◽  
Volker Seifert ◽  
Ulf Ziemann
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Motor Cortex ◽  
Evoked Potential ◽  
Corticospinal Tract ◽  
Motor Evoked Potential ◽  
Motor Deficit ◽  
Warning Sign ◽  
Precentral Gyrus ◽  
Resonance Imaging

Abstract OBJECTIVE To determine the degree to which the pattern of intraoperative isolated, unilateral alteration of motor evoked potential (MEP) in intracranial surgery was related to motor outcome and location of new postoperative signal alterations on magnetic resonance imaging (MRI). METHODS In 29 patients (age, 42.8 ± 18.2 years; 15 female patients; 25 supratentorial, 4 infratentorial procedures), intraoperative MEP alterations in isolation (without significant alteration in other evoked potential modalities) were classified as deterioration (&gt; 50% amplitude decrease and/or motor threshold increase) or loss, respectively, or reversible and irreversible. Postoperative MRI was described for the location and type of new signal alteration. RESULTS New motor deficit was present in all 5 patients with irreversible MEP loss, in 7 of 10 patients with irreversible MEP deterioration, in 1 of 6 patients with reversible MEP loss, and in 0 of 8 patients with reversible MEP deterioration. Irreversible compared with reversible MEP alteration was significantly more often correlated with postoperative motor deficit (P &lt; .0001). In 20 patients, 22 new signal alterations affected 29 various locations (precentral gyrus, n = 5; corticospinal tract, n = 19). Irreversible MEP alteration was more often associated with postoperative new signal alteration in MRI compared with reversible MEP alteration (P = .02). MEP loss was significantly more often associated with subcortically located new signal alteration (P = .006). MEP deterioration was significantly more often followed by new signal alterations located in the precentral gyrus (P = .04). CONCLUSION MEP loss bears a higher risk than MEP deterioration for postoperative motor deficit resulting from subcortical postoperative MR changes in the corticospinal tract. In contrast, MEP deterioration points to motor cortex lesion. Thus, even MEP deterioration should be considered a warning sign if surgery close to the motor cortex is performed.

Diffusion-Tensor MR Imaging of Corticospinal Tract in Amyotrophic Lateral Sclerosis and Progressive Muscular Atrophy

Radiology ◽  
2005 ◽  
Vol 237 (1) ◽  
pp. 258-264 ◽  
Author(s):  
Mirco Cosottini ◽  
Marco Giannelli ◽  
Gabriele Siciliano ◽  
Guido Lazzarotti ◽  
Maria Chiara Michelassi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mr Imaging ◽  
Corticospinal Tract ◽  
Diffusion Tensor ◽  
Muscular Atrophy ◽  
Diffusion Tensor Mr Imaging ◽  
Lateral Sclerosis ◽  
Progressive Muscular Atrophy
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