Extrapyramidal deficits in ALS: a combined biomechanical and neuroimaging study

AbstractIntroductionExtrapyramidal deficits are poorly characterised in amyotrophic lateral sclerosis (ALS) despite their contribution to functional disability, increased fall risk and their quality-of-life implications. Given the concomitant pyramidal and cerebellar degeneration in ALS, the clinical assessment of extrapyramidal features is particularly challenging.ObjectiveThe comprehensive characterisation  of postural instability in ALS using standardised clinical assessments, gait analyses and computational neuroimaging tools in a prospective study design.MethodsParameters of gait initiation in the anticipatory postural adjustment phase (APA) and execution phase (EP) were evaluated in ALS patients with and without postural instability and healthy controls. Clinical and gait analysis parameters were interpreted in the context of brain imaging findings.ResultsALS patients with postural instability exhibit impaired gait initiation with an altered APA phase, poor dynamic postural control and significantly decreased braking index. Consistent with their clinical profile, “unsteady” ALS patients have reduced caudate and brain stem volumes compared to “steady” ALS patients.InterpretationOur findings highlight that the ALS functional rating scale (ALSFRS-r) does not account for extrapyramidal deficits, which are major contributors to gait impairment in a subset of ALS patients. Basal ganglia degeneration in ALS does not only contribute to cognitive and...
Source: Journal of Neurology - Category: Neurology Source Type: research

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ConclusionThe findings suggested that bilateral motor cortex thinning presented in bulbar-onset ALS and extra-motor cortex thinning presented in spinal-onset ALS. The motor cortex thinning may be the intrinsic pathophysiological change that associated to the disease disability, and extra-motor cortex thinning may be secondary pathophysiological change that associated to disease duration.
Source: Chinese Medical Sciences Journal - Category: General Medicine Source Type: research
This study may serve disabled people to control assistive devices in natural, unobtrusive, speedy and reliable manner. Moreover, it is expected that GKP-based TMI could be a collaboration channel for traditional electroencephalography (EEG)-based brain computer interfaces which have significant inadequacies arisen from the EEG signals.
Source: Biocybernetics and Biomedical Engineering - Category: Biomedical Engineering Source Type: research
ConclusionThe findings suggested that bilateral motor cortex thinning presented in bulbar-onset ALS and extra-motor cortex thinning presented in spinal-onset ALS. The motor cortex thinning may be the intrinsic pathophysiological change that associated to the disease disability, and extra-motor cortex thinning may be secondary pathophysiological change that associated to disease duration.
Source: Chinese Medical Sciences Journal - Category: General Medicine Source Type: research
This study may serve disabled people to control assistive devices in natural, unobtrusive, speedy and reliable manner. Moreover, it is expected that GKP-based TMI could be a collaboration channel for traditional electroencephalography (EEG)-based brain computer interfaces which have significant inadequacies arisen from the EEG signals.
Source: Biocybernetics and Biomedical Engineering - Category: Biomedical Engineering Source Type: research
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motor neurons and other neuronal cells, leading to severe disability and eventually death from ventilatory failure. It has a prevalence of 5 in 100,000, with an incidence of 1.7 per 100,000, reflecting short average survival. The pathogenesis is incompletely understood, but defects of RNA processing and protein clearance may be fundamental. Repeat expansions in the chromosome 9 open reading frame 72 gene (C9orf72) are the most common known genetic cause of ALS and are seen in approximately 40% of patients with a family history and approximately 10% of those without.
Source: Mayo Clinic Proceedings - Category: Internal Medicine Authors: Tags: Review Source Type: research
This study may serve disabled people to control assistive devices in natural, unobtrusive, speedy and reliable manner. Moreover, it is expected that GKP-based TMI could be a collaboration channel for traditional electroencephalography (EEG)-based brain computer interfaces which have significant inadequacies arisen from the EEG signals.
Source: Biocybernetics and Biomedical Engineering - Category: Biomedical Engineering Source Type: research
AbstractAimTo investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis –frontotemporal dementia (ALS–FTD) spectrum of diseases.MethodsComprehensive assessment was taken on 21 patients with PLS and results were compared to patients diagnosed with pure motor ALS (n = 27) and ALS–FTD (n = 12). Clinical features, Addenbrooke’s Cognitive Examination (ACE) scores, Motor Neuron Disease Behaviour (Mind-B) scores, motor disability on the ALS functional rating scale (ALSFRS) and survival times were documented. Motor cortex excitabilit...
Source: Journal of Neurology - Category: Neurology Source Type: research
Authors: Dharmadasa T, Huynh W, Tsugawa J, Shimatani Y, Ma Y, Kiernan MC Abstract INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that causes progressive muscle weakness and disability, eventually leading to death. Heterogeneity of disease has become a major barrier to understanding key clinical questions such as prognosis and disease spread, and has disadvantaged clinical trials in search of therapeutic intervention. Patterns of disease have been explored through recent advances in neuroimaging, elucidating structural, molecular and functional changes. Unique brain signatures have eme...
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research
I woke up Wednesday morning to the news that Stephen Hawking had passed away. My first thought made me smile — that this incredible scientist who seemed to just will himself to stay alive against overwhelming odds, died on March 14th — Pi Day. Maybe that was his choice. Who knows? Stephen Hawking was a thinker — a brilliant scientist, professor and author who was known for his groundbreaking work in physics and cosmology. His books aimed to make science accessible to everyone. His more well-known works include A Brief History of Time, The Universe in a Nutshell, and A Briefer History of Time. At th...
Source: World of Psychology - Category: Psychiatry & Psychology Authors: Tags: Celebrities Grief and Loss Health-related Inspiration & Hope Memory and Perception Personal ALS Amyotrophic Lateral Sclerosis Depression Gratitude Lou Gehrig's Disease Motor Neuron Disease Stephen Hawking Source Type: blogs
(LONDON) — Stephen Hawking, whose brilliant mind ranged across time and space though his body was paralyzed by disease, died early Wednesday, a University of Cambridge spokesman said. He was 76 years old. Hawking died peacefully at his home in Cambridge, England. The best-known theoretical physicist of his time, Hawking wrote so lucidly of the mysteries of space, time and black holes that his book, “A Brief History of Time,” became an international best seller, making him one of science’s biggest celebrities since Albert Einstein. “He was a great scientist and an extraordinary man whose work a...
Source: TIME: Science - Category: Science Authors: Tags: Uncategorized onetime overnight remembrance Source Type: news
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