Unraveling ALS due to SOD1 mutation through the combination of brain and cervical cord MRI

Neurology ◽  
2018 ◽  
Vol 90 (8) ◽  
pp. e707-e716 ◽  
Author(s):  
Federica Agosta ◽  
Edoardo Gioele Spinelli ◽  
Ivan V. Marjanovic ◽  
Zorica Stevic ◽  
Elisabetta Pagani ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cortical Thickness ◽  
Brain Mri ◽  
Cross Sectional Area ◽  
Cervical Cord ◽  
Magnetization Transfer Ratio ◽  
Healthy Controls ◽  
Sectional Area ◽  
Cross Sectional ◽  
Sporadic Als

ObjectiveTo explore structural and functional changes of the brain and cervical cord in patients with amyotrophic lateral sclerosis (ALS) due to mutation in the superoxide dismutase (SOD1) gene compared with sporadic ALS.MethodsTwenty patients with SOD1 ALS, 11 with sporadic ALS, and 33 healthy controls underwent clinical evaluation and brain MRI. Cortical thickness analysis, diffusion tensor MRI of the corticospinal tracts (CST) and corpus callosum, and resting-state functional connectivity were performed. Patients with ALS also underwent cervical cord MRI to evaluate cord cross-sectional area and magnetization transfer ratio (MTR).ResultsPatients with SOD1 ALS showed longer disease duration and slower rate of functional decline relative to those with sporadic ALS. No cortical thickness abnormalities were found in patients with ALS compared with controls. Fractional anisotropy showed that sporadic ALS patients had significant CST damage relative to both healthy controls (p = 0.001−0.02) and SOD1-related ALS (p = 0.05), although the latter showed alterations that were intermediate between controls and sporadic ALS. Functional hyperconnectivity of the motor cortex in the sensorimotor network was observed in patients with sporadic ALS relative to controls. Conversely, patients with SOD1 ALS showed lower cord cross-sectional area along the whole cervical cord relative to those with sporadic ALS (p < 0.001). No cord MTR differences were found between patient groups.ConclusionsPatients with SOD1 ALS showed cervical cord atrophy relative to those with sporadic ALS and a relative preservation of brain motor structural and functional networks. Neurodegeneration in SOD1 ALS is likely to occur primarily in the spinal cord. An objective and accurate estimate of spinal cord damage has potential in the future assessment of preventive SOD1 ALS therapies.

scholarly journals Quantification of Cervical Cord Cross-Sectional Area: Which Acquisition, Vertebra Level, and Analysis Software? A Multicenter Repeatability Study on a Traveling Healthy Volunteer

2021 ◽  
Vol 12 ◽  
Author(s):  
Carsten Lukas ◽  
Barbara Bellenberg ◽  
Ferran Prados ◽  
Paola Valsasina ◽  
Katrin Parmar ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Healthy Volunteer ◽  
Brain Mri ◽  
Cross Sectional Area ◽  
Image Resolution ◽  
Cervical Cord ◽  
Surface Model ◽  
Sectional Area ◽  
Cross Sectional ◽  
Brain Images

Background: Considerable spinal cord (SC) atrophy occurs in multiple sclerosis (MS). While MRI-based techniques for SC cross-sectional area (CSA) quantification have improved over time, there is no common agreement on whether to measure at single vertebral levels or across larger regions and whether upper SC CSA can be reliably measured from brain images.Aim: To compare in a multicenter setting three CSA measurement methods in terms of repeatability at different anatomical levels. To analyze the agreement between measurements performed on the cervical cord and on brain MRI.Method: One healthy volunteer was scanned three times on the same day in six sites (three scanner vendors) using a 3T MRI protocol including sagittal 3D T1-weighted imaging of the brain (covering the upper cervical cord) and of the SC. Images were analyzed using two semiautomated methods [NeuroQLab (NQL) and the Active Surface Model (ASM)] and the fully automated Spinal Cord Toolbox (SCT) on different vertebral levels (C1–C2; C2/3) on SC and brain images and the entire cervical cord (C1–C7) on SC images only.Results: CSA estimates were significantly smaller using SCT compared to NQL and ASM (p &lt; 0.001), regardless of the cord level. Inter-scanner repeatability was best in C1–C7: coefficients of variation for NQL, ASM, and SCT: 0.4, 0.6, and 1.0%, respectively. CSAs estimated in brain MRI were slightly lower than in SC MRI (all p ≤ 0.006 at the C1–C2 level). Despite protocol harmonization between the centers with regard to image resolution and use of high-contrast 3D T1-weighted sequences, the variability of CSA was partly scanner dependent probably due to differences in scanner geometry, coil design, and details of the MRI parameter settings.Conclusion: For CSA quantification, dedicated isotropic SC MRI should be acquired, which yielded best repeatability in the entire cervical cord. In the upper part of the cervical cord, use of brain MRI scans entailed only a minor loss of CSA repeatability compared to SC MRI. Due to systematic differences between scanners and the CSA quantification software, both should be kept constant within a study. The MRI dataset of this study is available publicly to test new analysis approaches.

scholarly journals Quantitative spinal cord MRI in MOG-antibody disease, neuromyelitis optica and multiple sclerosis

Brain ◽  
2020 ◽  
Author(s):  
Romina Mariano ◽  
Silvia Messina ◽  
Adriana Roca-Fernandez ◽  
Maria I Leite ◽  
Yazhuo Kong ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Grey Matter ◽  
Magnetization Transfer ◽  
Cross Sectional Area ◽  
Cervical Cord ◽  
Magnetization Transfer Ratio ◽  
Sectional Area ◽  
Cross Sectional ◽  
Aqp4 Antibody

Abstract Spinal cord involvement is a hallmark feature of multiple sclerosis, neuromyelitis optica with AQP4 antibodies and MOG-antibody disease. In this cross-sectional study we use quantitative spinal cord MRI to better understand these conditions, differentiate them and associate with relevant clinical outcomes. Eighty participants (20 in each disease group and 20 matched healthy volunteers) underwent spinal cord MRI (cervical cord: 3D T1, 3D T2, diffusion tensor imaging and magnetization transfer ratio; thoracic cord: 3D T2), together with disability, pain and fatigue scoring. All participants had documented spinal cord involvement and were at least 6 months post an acute event. MRI scans were analysed using publicly available software. Those with AQP4-antibody disease showed a significant reduction in cervical cord cross-sectional area (P = 0.038), thoracic cord cross-sectional area (P = 0.043), cervical cord grey matter (P = 0.011), magnetization transfer ratio (P ≤ 0.001), fractional anisotropy (P = 0.004) and increased mean diffusivity (P = 0.008). Those with multiple sclerosis showed significantly increased mean diffusivity (P = 0.001) and reduced fractional anisotropy (P = 0.013), grey matter volume (P = 0.002) and magnetization transfer ratio (P = 0.011). In AQP4-antibody disease the damage was localized to areas of the cord involved in the acute attack. In multiple sclerosis this relationship with lesions was absent. MOG-antibody disease did not show significant differences to healthy volunteers in any modality. However, when considering only areas involved at the time of the acute attack, a reduction in grey matter volume was found (P = 0.023). This suggests a predominant central grey matter component to MOG-antibody myelitis, which we hypothesize could be partially responsible for the significant residual sphincter dysfunction. Those with relapsing MOG-antibody disease showed a reduction in cord cross-sectional area compared to those with monophasic disease, even when relapses occurred elsewhere (P = 0.012). This suggests that relapsing MOG-antibody disease is a more severe phenotype. We then applied a principle component analysis, followed by an orthogonal partial least squares analysis. MOG-antibody disease was discriminated from both AQP4-antibody disease and multiple sclerosis with moderate predictive values. Finally, we assessed the clinical relevance of these metrics using a multiple regression model. Cervical cord cross-sectional area associated with disability scores (B = −0.07, P = 0.0440, R2 = 0.20) and cervical cord spinothalamic tract fractional anisotropy associated with pain scores (B = −19.57, P = 0.016, R2 = 0.55). No spinal cord metric captured fatigue. This work contributes to our understanding of myelitis in these conditions and highlights the clinical relevance of quantitative spinal cord MRI.

scholarly journals Cervical Spinal Cord Atrophy can be Accurately Quantified Using Head Images

2022 ◽  
Vol 8 (1) ◽  
pp. 205521732110707
Author(s):  
Kamyar Taheri ◽  
Irene M Vavasour ◽  
Shawna Abel ◽  
Lisa Eunyoung Lee ◽  
Poljanka Johnson ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Brain Mri ◽  
Cross Sectional Area ◽  
Acquisition Time ◽  
Healthy Controls ◽  
Sectional Area ◽  
Spinal Cord Atrophy ◽  
Cross Sectional ◽  
Area Measure

Background Spinal cord atrophy provides a clinically relevant metric for monitoring MS. However, the spinal cord is imaged far less frequently than brain due to artefacts and acquisition time, whereas MRI of the brain is routinely performed. Objective To validate spinal cord cross-sectional area measurements from routine 3DT1 whole-brain MRI versus those from dedicated cord MRI in healthy controls and people with MS. Methods We calculated cross-sectional area at C1 and C2/3 using T2*-weighted spinal cord images and 3DT1 brain images, for 28 healthy controls and 73 people with MS. Correlations for both groups were assessed between: (1) C1 and C2/3 using cord images; (2) C1 from brain and C1 from cord; and (3) C1 from brain and C2/3 from cord. Results and Conclusion C1 and C2/3 from cord were strongly correlated in controls ( r = 0.94, p<0.0001) and MS ( r = 0.85, p<0.0001). There was strong agreement between C1 from brain and C2/3 from cord in controls ( r = 0.84, p<0.0001) and MS ( r = 0.81, p<0.0001). This supports the use of C1 cross-sectional area calculated from brain imaging as a surrogate for the traditional C2/3 cross-sectional area measure for spinal cord atrophy.

High resolution ultrasonography of peripheral nerves in diabetic patients to evaluate nerve cross sectional area with clinical profile

2021 ◽  
Vol 94 (1121) ◽  
pp. 20200173
Author(s):  
Shamrendra Narayan ◽  
Amit Goel ◽  
Ajai Kumar Singh ◽  
Anup Kumar Thacker ◽  
Neha Singh ◽  
...  
Keyword(s):  
Peripheral Nerves ◽  
Cross Sectional Area ◽  
Diabetic Patients ◽  
Tarsal Tunnel ◽  
Healthy Controls ◽  
Sectional Area ◽  
Cross Sectional ◽  
Early Therapy ◽  
Ultrasound Evaluation ◽  
Compressive Neuropathy

Objectives: The aim of this observational study was ultrasound evaluation of peripheral nerves cross-sectional area (CSA) in subjects with probable diabetic peripheral sensorimotor neuropathy (DPN). CSA was analyzed with reference to clinical and nerve conduction study’s (NCS) parameters for early diagnosis and pattern of involvement. Methods: A total of 50 patients with probable DPN due to Type 2 diabetes and 50 age-matched healthy controls underwent sonographic examinations of ulnar nerve at the lower arm, median nerve proximal to carpal tunnel, the common peroneal nerve proximal to fibular head, tibial nerve proximal to the tarsal tunnel, and sural nerve at lower third leg. Results: CSA was increased in cases of DPN as compared to healthy controls. Area changes were more marked with demyelinating pattern. Probable DPN cases with normal NCS had significantly higher number of peripheral nerves showing increased CSA as compared to healthy control. A cut-off of >4 nerve thickening showed a sensitivity of 86 %, and specificity of 56%. The neuropathy pattern in the lower limb was axonal, whereas in the upper limb, it was demyelinating with the majority showing sonographic feature of associated compressive neuropathy. Conclusion: There is an increase in CSA of peripheral nerve in diabetic patients. It can be used as a morphological marker for classifying DPN with changes being picked up earlier to NCS abnormality. Clinical neurological presentation in probable DPN can also be due to compressive neuropathy in early phases, and ultrasound can be a useful tool. Advances in knowledge: Early pick up of DPN cases shall be useful for early therapy and motivating the patients to actively participate in the treatment. Morphological changes on ultrasonography precedes the electrodiagnostic change in DPN. Symptoms of DPN is not only due to metabolic changes but also compressive neuropathy.

scholarly journals Dehydration affects spinal cord cross-sectional area measurement on MRI in healthy subjects

Spinal Cord ◽  
2014 ◽  
Vol 52 (8) ◽  
pp. 616-620 ◽  
Author(s):  
C Wang ◽  
R C Tam ◽  
E Mackie ◽  
D K B Li ◽  
A L Traboulsee
Keyword(s):  
Spinal Cord ◽  
Healthy Subjects ◽  
Cross Sectional Area ◽  
Area Measurement ◽  
Sectional Area ◽  
Cross Sectional

scholarly journals A comparison of minimal cross sectional areas, nasal volumes and peak nasal inspiratory flow between patients with obstructive sleep apnea and healthy controls

2016 ◽  
Vol 54 (4) ◽  
pp. 342-347
Author(s):  
M.H.S. Moxness ◽  
V. Bugten ◽  
W.M. Thorstensen ◽  
S. Nordgard ◽  
G. Bruskeland
Keyword(s):  
Nasal Cavity ◽  
Inspiratory Flow ◽  
Cross Sectional Area ◽  
Healthy Controls ◽  
Sectional Area ◽  
Cavity Volume ◽  
Cross Sectional ◽  
The Mean ◽  
And Function ◽  
Peak Nasal Inspiratory Flow

Background: The differences in nasal geometry and function between OSA patients and healthy individuals are not known. Our aim was to evaluate the differences in nasal geometry and function using acoustic rhinometry (AR) and peak nasal inspiratory flow (PNIF) between an OSA population and healthy controls. Methodology: The study was designed as a prospective case-control study. Ninety-three OSA patients and 92 controls were enrolled from 2010 to 2015. The minimal cross-sectional area (MCA) and the nasal cavity volume (NCV) in two parts of the nose (MCA0-3/NCV0-3 and MCA3-5.2/NCV3-5.2) and PNIF were measured at baseline and after decongestion. Results: The mean MCA0-3 in the OSA group was 0.49 cm2; compared to 0.55 cm2 in controls. The mean NCV0-3 correspondingly was 2.51 cm3 compared to 2.73 cm3 in controls. PNIF measured 105 litres/minute in the OSA group and 117 litres/minute in the controls. Conclusions: OSA patients have a lower minimum cross-sectional area, nasal cavity volume and peak inspiratory flow compared to controls. Our study supports the view that changes in the nasal cavity may contribute to development of OSA.

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