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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Objective The aim of the study was to systematize the disability condition related to amyotrophic lateral sclerosis patients using the International Classification of Functioning, Disability and Health (ICF) and narrative medicine approach as a common tool to identify a patient's functional problems. Once identified, this can be used as the basis for an individual rehabilitation project. Design This is an observational study on patients residing in a central region of Italy with a diagnosis of amyotrophic lateral sclerosis. The narrative approach involved listening to the patients' stories while guiding them with a se...
Source: American Journal of Physical Medicine and Rehabilitation - Category: Rehabilitation Tags: Original Research Articles Source Type: research
Authors: Taga A, Maragakis NJ Abstract INTRODUCTION: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder which results in progressive muscle weakness as a prominent feature. However, clinical and pathogenic heterogeneity with regard to anatomical sites of disease onset and progression over time are important features of the disease which have frustrated attempts to inform patients about their prognosis as well as limited the potential successes of therapeutic studies. Areas covered: This paper discusses: clinical biomarkers which can be measured by history, physical examination, and clinical scales of ...
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research
Publication date: Available online 25 September 2018Source: Egyptian Journal of AnaesthesiaAuthor(s): Bo-Ra Kim, Young-Bok Lee, Su-Jin Kim, Young-Wan KimAbstractAmyotrophic lateral sclerosis, which is also known as motor neuron disease, is a chronic neurodegenerative disease characterized by progressive muscular weakness, respiratory muscle disability, and eventual death. Previous epidemiologic studies have shown no association between cancer and amyotrophic lateral sclerosis. Colorectal cancer arising in patients with amyotrophic lateral sclerosis has rarely been reported. Here, we report a case involving rectal cancer ar...
Source: Egyptian Journal of Anaesthesia - Category: Anesthesiology Source Type: research
CONCLUSIONS: Research and new interventions should focus further on how to support the partners of plwALS. Future research with plwALS should place AAC strategies and technology in the context of wider communication and investigate how best to support the anticipation, preparation and adaptation to communication change. Specific areas of research need relating to this support are suggested. IMPLICATIONS FOR REHABILITATION People living with ALS (plwALS) are rarely involved in setting the research agenda in the areas of speech and augmentative and alternative communication (AAC). Enabling plwALS to contribute to research, e...
Source: Disability and Rehabilitation. Assistive Technology. - Category: Rehabilitation Authors: Tags: Disabil Rehabil Assist Technol Source Type: research
t PF Abstract OBJECTIVE: Objective of this study is the comprehensive characterisation of motor unit (MU) loss in type III and IV Spinal Muscular Atrophy (SMA) using motor unit number index (MUNIX), and evaluation of compensatory mechanisms based on MU size indices (MUSIX). METHODS: Nineteen type III and IV SMA patients and 16 gender- and age-matched healthy controls were recruited. Neuromuscular performance was evaluated by muscle strength testing and functional scales. Compound motor action potential (CMAP), MUNIX and MUSIX were studied in the abductor pollicis brevis (APB), abductor digiti minimi (ADM), de...
Source: Clinical Neurophysiology - Category: Neurology Authors: Tags: Clin Neurophysiol Source Type: research
Augmentative and alternative communication (AAC) is typically used by people with severe speech and physical disabilities and is one of the main application areas for the brain–computer interface (BCI) technology. The target population includes people with cerebral palsy, amyotrophic lateral sclerosis, and locked-in syndrome. Word-based AAC systems are mainly faster than letter-based counterparts and are usually supplemented by icons to aid the users. Those icon-based AAC systems that use binary signaling methods such as single click can convert into a single-input BCI system such as event-related potential (ERP) det...
Source: IEE Transactions on Neural Systems and Rehabilitation Engineering - Category: Neuroscience Source Type: research
We compared the functioning of two neurological patient groups, amyotrophic lateral sclerosis (ALS) and traumatic brain injury (TBI), using brief and validated International Classification of Functioning (ICF)-based tools. A 12-item World Health Organization Disability Assessment Schedule (WHODAS 2.0) questionnaire was mailed to ALS and TBI patients and their significant others 2 weeks before their appointment at an outpatient clinic of a university hospital. In addition, a neurologist filled in the ICF minimal generic set. Two years after diagnosis, no significant differences between the two diagnosis groups were found in...
Source: International Journal of Rehabilitation Research - Category: Rehabilitation Tags: Original articles Source Type: research
CONCLUSIONS: Performance on the ACE was comparable between patients and controls. Associations with motor function pose a challenge to accurate interpretation of ACE performance. It is likely that patients with poor cognition have greater disability, or that poor ACE performance reflects reduced motor ability to perform the task. This raises concern for the utility of the ACE as a screening tool in ALS patients, especially since recent versions of the ACE continue to include motor-based tasks. PMID: 30079844 [PubMed - as supplied by publisher]
Source: The Canadian Journal of Neurological Sciences - Category: Neurology Authors: Tags: Can J Neurol Sci 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
Katharina Linse, Elisa Aust, Markus Joos, Andreas Hermann
Source: Frontiers in Neurology - Category: Neurology Source Type: research
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