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https://www.readbyqxmd.com/read/27913627/aberrant-interhemispheric-homotopic-functional-and-structural-connectivity-in-amyotrophic-lateral-sclerosis
#1
Jiuquan Zhang, Bing Ji, Jun Hu, Chaoyang Zhou, Longchuan Li, Zhihao Li, Xuequan Huang, Xiaoping Hu
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is an idiopathic and fatal neurodegenerative disease of the human motor system. While microstructural alterations in corpus callosum (CC) have been identified as a consistent feature of ALS, studies directly examining interhemispheric neural connectivity are still lacking. To shed more light on the pathophysiology of ALS, the present study aims to examine alterations of interhemispheric structural and functional connectivity in individuals with ALS...
December 2, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27907080/development-of-an-automated-mri-based-diagnostic-protocol-for-amyotrophic-lateral-sclerosis-using-disease-specific-pathognomonic-features-a-quantitative-disease-state-classification-study
#2
Christina Schuster, Orla Hardiman, Peter Bede
BACKGROUND: Despite significant advances in quantitative neuroimaging, the diagnosis of ALS remains clinical and MRI-based biomarkers are not currently used to aid the diagnosis. The objective of this study is to develop a robust, disease-specific, multimodal classification protocol and validate its diagnostic accuracy in independent, early-stage and follow-up data sets. METHODS: 147 participants (81 ALS patients and 66 healthy controls) were divided into a training sample and a validation sample...
2016: PloS One
https://www.readbyqxmd.com/read/27796080/white-matter-structural-network-abnormalities-underlie-executive-dysfunction-in-amyotrophic-lateral-sclerosis
#3
Dennis Dimond, Abdullah Ishaque, Sneha Chenji, Dennell Mah, Zhang Chen, Peter Seres, Christian Beaulieu, Sanjay Kalra
Research in amyotrophic lateral sclerosis (ALS) suggests that executive dysfunction, a prevalent cognitive feature of the disease, is associated with abnormal structural connectivity and white matter integrity. In this exploratory study, we investigated the white matter constructs of executive dysfunction, and attempted to detect structural abnormalities specific to cognitively impaired ALS patients. About 18 ALS patients and 22 age and education matched healthy controls underwent magnetic resonance imaging on a 4...
October 31, 2016: Human Brain Mapping
https://www.readbyqxmd.com/read/27784774/safety-of-intrathecal-autologous-adipose-derived-mesenchymal-stromal-cells-in-patients-with-als
#4
Nathan P Staff, Nicolas N Madigan, Jonathan Morris, Mark Jentoft, Eric J Sorenson, Greg Butler, Dennis Gastineau, Allan Dietz, Anthony J Windebank
OBJECTIVE: To determine the safety of intrathecal autologous adipose-derived mesenchymal stromal cell treatment for amyotrophic lateral sclerosis (ALS). METHODS: Participants with ALS were enrolled and treated in this phase I dose-escalation safety trial, ranging from 1 × 10(7) (single dose) to 1 × 10(8) cells (2 monthly doses). After intrathecal treatments, participants underwent standardized follow-up, which included clinical examinations, revised ALS Functional Rating Scale (ALSFRS-R) questionnaire, blood and CSF sampling, and MRI of the neuroaxis...
November 22, 2016: Neurology
https://www.readbyqxmd.com/read/27760655/-research-of-upper-arms-muscle-mri-in-patients-with-kennedy-s-disease
#5
M Lu, D S Fan
Objective: To investigate whether or not the involvement degree of extensor and flexor muscles in patients with Kennedy's Disease (KD) was different by measuring the diameter of biceps and triceps brachii muscles using Magnetic Resonance Imaging (MRI). Methods: All the subjects were outpatients or hospitalized patients from Department of Neurology, the Third Hospital of Peking University, 2011-2015. Subjects included 30 KD patients and 30 amyotrophic lateral sclerosis (ALS) patients. The right upper arms of all subjects were examined by muscle MRI...
October 11, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27756805/widespread-structural-brain-involvement-in-als-is-not-limited-to-the-c9orf72-repeat-expansion
#6
Henk-Jan Westeneng, Renée Walhout, Milou Straathof, Ruben Schmidt, Jeroen Hendrikse, Jan H Veldink, Martijn P van den Heuvel, Leonard H van den Berg
BACKGROUND: In patients with a C9orf72 repeat expansion (C9+), a neuroimaging phenotype with widespread structural cerebral changes has been found. We aimed to investigate the specificity of this neuroimaging phenotype in patients with amyotrophic lateral sclerosis (ALS). METHODS: 156 C9- and 14 C9+ patients with ALS underwent high-resolution T1-weighted MRI; a subset (n=126) underwent diffusion-weighted imaging. Cortical thickness, subcortical volumes and white matter integrity were compared between C9+ and C9- patients...
October 18, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27741285/perception-of-emotional-facial-expressions-in-amyotrophic-lateral-sclerosis-als-at-behavioural-and-brain-metabolic-level
#7
Helena E A Aho-Özhan, Jürgen Keller, Johanna Heimrath, Ingo Uttner, Jan Kassubek, Niels Birbaumer, Albert C Ludolph, Dorothée Lulé
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) primarily impairs motor abilities but also affects cognition and emotional processing. We hypothesise that subjective ratings of emotional stimuli depicting social interactions and facial expressions is changed in ALS. It was found that recognition of negative emotions and ability to mentalize other's intentions is reduced. METHODS: Processing of emotions in faces was investigated. A behavioural test of Ekman faces expressing six basic emotions was presented to 30 ALS patients and 29 age-, gender and education matched healthy controls...
2016: PloS One
https://www.readbyqxmd.com/read/27671242/mitochondrial-membrane-protein-associated-neurodegeneration-mimicking-juvenile-amyotrophic-lateral%C3%A2-sclerosis
#8
Jiyeon Kim Md PhD, Yu-Hsien Liao, Cristian Ionita, Allen E Bale, Basil Darras, Gyula Acsadi
BACKGROUND: Mitochondrial membrane protein associated neurodegeneration (MPAN) is the third most common subtype of neurodegeneration with brain iron accumulation (NBIA) and caused by mutations of the orphan gene C19ORF12 encoding a transmembrane mitochondrial protein. Like other NBIA disorders, the hallmark of neuropathology is iron deposition in the basal ganglia, but the clinical presentation is highly variable. METHODS: We present the relevant clinical history, neurological examination, electrophysiological and neuroimaging tests of a currently ten-year-old patient are presented...
August 24, 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27663988/fully-integrated-framework-for-the-segmentation-and-registration-of-the-spinal-cord-white-and-gray-matter
#9
Sara M Dupont, Benjamin De Leener, Manuel Taso, Arnaud Le Troter, Nikola Stikov, Virginie Callot, Julien Cohen-Adad
: The spinal cord white and gray matter can be affected by various pathologies such as multiple sclerosis, amyotrophic lateral sclerosis or trauma. Being able to precisely segment the white and gray matter could help with MR image analysis and hence be useful in further understanding these pathologies, and helping with diagnosis/prognosis and drug development. Up to date, white/gray matter segmentation has mostly been done manually, which is time consuming, induces a bias related to the rater and prevents large-scale multi-center studies...
September 20, 2016: NeuroImage
https://www.readbyqxmd.com/read/27655303/mri-shows-thickening-and-altered-diffusion-in-the-median-and-ulnar-nerves-in-multifocal-motor-neuropathy
#10
Wieke Haakma, Bas A Jongbloed, Martijn Froeling, H Stephan Goedee, Clemens Bos, Alexander Leemans, Leonard H van den Berg, Jeroen Hendrikse, W Ludo van der Pol
OBJECTIVES: To study disease mechanisms in multifocal motor neuropathy (MMN) with magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) of the median and ulnar nerves. METHODS: We enrolled ten MMN patients, ten patients with amyotrophic lateral sclerosis (ALS) and ten healthy controls (HCs). Patients underwent MRI (in a prone position) and nerve conduction studies. DTI and fat-suppressed T2-weighted scans of the forearms were performed on a 3.0T MRI scanner...
September 21, 2016: European Radiology
https://www.readbyqxmd.com/read/27571543/comparative-study-of-peripheral-nerve-mri-and-ultrasound-in-multifocal-motor-neuropathy-and-amyotrophic-lateral-sclerosis
#11
Bas A Jongbloed, Wieke Haakma, H Stephan Goedee, Jeroen W Bos, Clemens Bos, Jeroen Hendrikse, Leonard H Van Den Berg, W Ludo Van Der Pol
INTRODUCTION: Differentiating multifocal motor neuropathy (MMN) from amyotrophic lateral sclerosis (ALS) is important, as MMN is a difficult, but treatable disorder. METHODS: We studied peripheral nerve imaging techniques in differentiating MMN from ALS by measuring the cross-sectional area (CSA) of the median and ulnar nerves in the forearms using high resolution ultrasound (HRUS) and MRI. RESULTS: HRUS CSA values of the median nerve in the forearm (P = 0...
December 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27567641/the-value-of-magnetic-resonance-imaging-as-a-biomarker-for-amyotrophic-lateral-sclerosis-a-systematic-review
#12
G Grolez, C Moreau, V Danel-Brunaud, C Delmaire, R Lopes, P F Pradat, M M El Mendili, L Defebvre, D Devos
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal, rapidly progressive neurodegenerative disease that mainly affects the motor system. A number of potentially neuroprotective and neurorestorative disease-modifying drugs are currently in clinical development. At present, the evaluation of a drug's clinical efficacy in ALS is based on the ALS Functional Rating Scale Revised, motor tests and survival. However, these endpoints are general, variable and late-stage measures of the ALS disease process and thus require the long-term assessment of large cohorts...
August 27, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27566977/traumatic-brain-injury-results-in-cellular-structural-and-functional-changes-resembling-motor-neuron-disease
#13
David K Wright, Shijie Liu, Chris van der Poel, Stuart J McDonald, Rhys D Brady, Lily Taylor, Li Yang, Andrew J Gardner, Roger Ordidge, Terence J O'Brien, Leigh A Johnston, Sandy R Shultz
Traumatic brain injury (TBI) has been suggested to increase the risk of amyotrophic lateral sclerosis (ALS). However, this link remains controversial and as such, here we performed experimental moderate TBI in rats and assessed for the presence of ALS-like pathological and functional abnormalities at both 1 and 12 weeks post-injury. Serial in-vivo magnetic resonance imaging (MRI) demonstrated that rats given a TBI had progressive atrophy of the motor cortices and degeneration of the corticospinal tracts compared with sham-injured rats...
August 27, 2016: Cerebral Cortex
https://www.readbyqxmd.com/read/27560686/comparison-of-diffusion-mri-acquisition-protocols-for-the-in-vivo-characterization-of-the-mouse-spinal-cord-variability-analysis-and-application-to-an-amyotrophic-lateral-sclerosis-model
#14
Matteo Figini, Alessandro Scotti, Stefania Marcuzzo, Silvia Bonanno, Francesco Padelli, Victoria Moreno-Manzano, José Manuel García-Verdugo, Pia Bernasconi, Renato Mantegazza, Maria Grazia Bruzzone, Ileana Zucca
Diffusion-weighted Magnetic Resonance Imaging (dMRI) has relevant applications in the microstructural characterization of the spinal cord, especially in neurodegenerative diseases. Animal models have a pivotal role in the study of such diseases; however, in vivo spinal dMRI of small animals entails additional challenges that require a systematical investigation of acquisition parameters. The purpose of this study is to compare three acquisition protocols and identify the scanning parameters allowing a robust estimation of the main diffusion quantities and a good sensitivity to neurodegeneration in the mouse spinal cord...
2016: PloS One
https://www.readbyqxmd.com/read/27514792/hypoparathyroidism-a-rare-mimicker-of-amyotrophic-lateral-sclerosis
#15
Haris Hakeem, Masood Uz Zaman, Sara Khan
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) carries a grim prognosis. Various ALS mimics have been reported and should be excluded before confirming this diagnosis. METHODS: We report the case of a 61-year-old man who presented with progressively worsening limb weakness and dysphagia. His examination showed mixed upper and lower motor neuron signs without sensory impairment. ALS was suspected, however atypical diffuse pain prompted diagnostic work-up to exclude other causes...
August 12, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27508110/omental-transplantation-in-a-patient-with-mild-als
#16
Hernando Rafael
To demonstrate that amyotrophic lateral sclerosis (ALS) is not a neurodegenerative disease. The patient, a 33-year-old man began with symptoms of the bulbar form of ALS, characterized by burning pain in both feet during two months and then, he presented right crural monoparesis, fasciculations, slight dysarthria and he walked with help of orthopedic devices. A preoperative MRI scans showed atherosclerosis at the V4 segment of the left vertebral artery. On May 2012, he received an omental transplantation on the anterior, left lateral and posterior surface of the medulla oblongata...
2016: American Journal of Neurodegenerative Disease
https://www.readbyqxmd.com/read/27423585/hyperintensity-of-the-corticospinal-tract-on-flair-a-simple-and-sensitive-objective-upper-motor-neuron-degeneration-marker-in-clinically-verified-amyotrophic-lateral-sclerosis
#17
Jiaoting Jin, Fangfang Hu, Qiuli Zhang, Rui Jia, Jingxia Dang
OBJECTIVE: The involvement of upper motor neuron (UMN) degeneration is crucial to the diagnosis of amyotrophic lateral sclerosis (ALS). However, it is difficult to detect in the early stages, and particularly with predominantly lower motor neuron (LMN) dysfunction. Thus, objective and sensitive UMN degeneration markers are needed for an accurate and early diagnosis. Several studies have investigated the abnormal signal changes in brain MRI for patients with ALS, so we hope to develop a neuroimaging diagnosis method in brain MRI that can evaluate UMN degeneration...
August 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27423254/effects-of-a-spaceflight-analog-environment-on-brain-connectivity-and-behavior
#18
Kaitlin Cassady, Vincent Koppelmans, Patricia Reuter-Lorenz, Yiri De Dios, Nichole Gadd, Scott Wood, Roy Riascos Castenada, Igor Kofman, Jacob Bloomberg, Ajitkumar Mulavara, Rachael Seidler
Sensorimotor functioning is adaptively altered following long-duration spaceflight. The question of whether microgravity affects other central nervous system functions such as brain network organization and its relationship with behavior is largely unknown, but of importance to the health and performance of astronauts both during and post-flight. In the present study, we investigate the effects of prolonged exposure to an established spaceflight analog on resting state brain functional connectivity and its association with behavioral changes in 17 male participants...
November 1, 2016: NeuroImage
https://www.readbyqxmd.com/read/27323612/-correlation-between-fractional-anisotropy-and-amyotrophic-lateral-sclerosis-and-effect-of-inte-grative-medical-treatment-on-als
#19
Mei-li Tang, Bao-xin Du, Zhi-yuan Chen, Xin Chen, Si-run Liu, Yu-pin Liu, Ru-hang Huang, Bo Liu
OBJECTIVE: To explore whether fractional anisotropy (FA) value could be taken as a quantitative indicator for tracing and reexamining amyotrophic lateral sclerosis (ALS), and to analyze the correlation between FA value and integrative medical treatment. METHODS: Totally 18 ALS patients were recruited in this study. All patients received diffusion tensor imaging (DTI) using 3. OT (Propeller HD) MRI twice. Six regions of interest (ROI) were selected to measure FA values...
April 2016: Chinese Journal of Integrated Traditional and Western Medicine
https://www.readbyqxmd.com/read/27322194/investigating-default-mode-and-sensorimotor-network-connectivity-in-amyotrophic-lateral-sclerosis
#20
Sneha Chenji, Shankar Jha, Dawon Lee, Matthew Brown, Peter Seres, Dennell Mah, Sanjay Kalra
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition characterized by degeneration of upper motor neurons (UMN) arising from the motor cortex in the brain and lower motor neurons (LMN) in the brainstem and spinal cord. Cerebral changes create differences in brain activity captured by functional magnetic resonance imaging (fMRI), including the spontaneous and simultaneous activity occurring between regions known as the resting state networks (RSNs). Progressive neurodegeneration as observed in ALS may lead to a disruption of RSNs which could provide insights into the disease process...
2016: PloS One
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