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134 active trials for Amyotrophic Lateral Sclerosis

Explore Neuroprotective Effect of Lipoic Acid in Amyotrophic Lateral Sclerosis

In this proposed study, the investigators will evaluate the safety and efficacy of lipoic acid in treatment of Amyotrophic lateral sclerosis (ALS). The study will recruit 150 AD patients, and then these patients will be randomized to lipoic acid group or control group (75 patients per arm) for 6 courses for about 5 months. Clinical assessment will be done at screen/baseline, 3th course and 6th course. The specific aims are to compare lipoic acid versus control on: motor function and disease progression. During the study period, clinical effect index will be recorded, including bulbar function, motor function, respiratory function, and safety index including blood and urine routine, liver and kidney function, coagulation function.

Start: September 2020

Presymptomatic Neuromuscular Junction Defects and Compensatory Mechanisms in ALS

Denervation of neuromuscular junctions (NMJs) and initial compensatory reinnervation is the earliest pathological event in various motor neuron disease models, occurring far before motor symptom onset. In patients harboring genetic mutations responsible for Amyotrophic Lateral Sclerosis (ALS), identification of early, pre-symptomatic, NMJ pathological events and compensatory mechanisms could lead to the development of new treatments to prevent motor functional impairment. The aims of our study are thus: To investigate and characterize early, presymptomatic, defects of NMJ morphology in pre-manifest C9ORF72 or SOD1 mutation carriers; To investigate and quantify reinnervation at the level of NMJs in these subjects; To identify muscle molecular dysregulated pathways involved in the development of NMJ alterations and the development / maintenance of compensatory collateral reinnervation.

Start: April 2019

A Study of IPL344 in the Treatment of ALS Patients

This is a prospective, open-label, phase 1/2a study, dose escalation, to evaluate tolerability, safety, and PK of I.V. administered IPL344 in participants with Amyotrophic Lateral Sclerosis (ALS).

Start: August 2018

Fecal Microbiota Transplantation Effect on Amyotrophic Lateral Sclerosis Patients

Given the role of adaptive immunity in ALS, the pathogenicity of some clostridial strains on motorneurons, the putative role of cyanobacteria in ALS development, and the increasing interest for microbiota in neurodegenerative disorders, the modification of intestinal microbiota might affect ALS at its core. This interventional study aims at evaluating the biological and disease-modifying effects of Fecal Microbiota Transplant (FMT) in patients affected by Amyotrophic Lateral Sclerosis. As a primary aim of the study, the investigators postulate ALS patients treated with FMT compared to the control arm will display increased Tregs number, which is a favourable biomarker of disease activity and progression. Clinical outcomes as disease progression measured by ALS Functional Rating Scale Revised (ALSFRS-R) score, survival, respiratory function and quality of life will be assessed during the whole treatment and follow-up period. Moreover, biological activity of FMT will be evaluated in different biomatrices, together with FMT safety and tolerability in a cohort of ALS patients.

Start: July 2020

Optimizing BCI-FIT: Brain Computer Interface - Functional Implementation Toolkit

This project adds to non-invasive BCIs for communication for adults with severe speech and physical impairments due to neurodegenerative diseases. Researchers will optimize & adapt BCI signal acquisition, signal processing, natural language processing, & clinical implementation. BCI-FIT relies on active inference and transfer learning to customize a completely adaptive intent estimation classifier to each user's multi-modality signals simultaneously. 3 specific aims are: 1. develop & evaluate methods for on-line & robust adaptation of multi-modal signal models to infer user intent; 2. develop & evaluate methods for efficient user intent inference through active querying, and 3. integrate partner & environment-supported language interaction & letter/word supplementation as input modality. The same 4 dependent variables are measured in each SA: typing speed, typing accuracy, information transfer rate (ITR), & user experience (UX) feedback. Four alternating-treatments single case experimental research designs will test hypotheses about optimizing user performance and technology performance for each aim.Tasks include copy-spelling with BCI-FIT to explore the effects of multi-modal access method configurations (SA1.4a), adaptive signal modeling (SA1.4b), & active querying (SA2.2), and story retell to examine the effects of language model enhancements. Five people with SSPI will be recruited for each study. Healthy control participants will be recruited for experiments in SA2.2 and SA3.4. Study hypotheses are: (SA1.4a) A customized BCI-FIT configuration based on multi-modal input and personal metadata will improve typing accuracy on a copy-spelling task compared to a user's existing AAC access method. (SA1.4b) Adaptive signal modeling will mitigate the effects of changes in user state on typing accuracy during a copy-spelling task with BCI-FIT. (SA2.2) Either of two methods of adaptive querying will improve BCI-FIT typing accuracy for users with mediocre AUC scores. (SA3.4) Language model enhancements, including a combination of partner and environmental input and word completion during typing, will improve typing performance with BCI-FIT, as measured by ITR during a story-retell task. Optimized recommendations for a multi-modal BCI for each end user will be established, based on an innovative combination of clinical expertise, user feedback, customized multi-modal sensor fusion, and reinforcement learning.

Start: July 2021

A Systematic Investigation of Phonetic Complexity Effects on Articulatory Motor Performance in Progressive Dysarthria

The goal is to improve the fundamental knowledge about articulatory motor performance in people with Lou Gehrig's disease (also known as ALS) and Parkinson's disease (PD), in order to develop more sensitive assessments for progressive speech loss, which may lead to the improved timing of speech therapies.

Start: July 2017

Safety and Tolerability of Perampanel in Amyotrophic Lateral Sclerosis Patients

Amyotrophic lateral sclerosis (ALS), the most common motor neuron disease, is a fatal progressive neurodegenerative disease affecting motor cortex, brainstem and spinal cord leading to motor neuron death. It is a devastating disease of the anterior and lateral corticospinal tracts with approximately 3 years mean duration from symptoms onset to death, one-fifth survival at 5 years and only 10% may make it to 10 years. Among the neuronal death pathways, excitotoxicity mechanism is considered to be the foremost-involved mechanism. AMPA receptors are thought to be the prime mediator of the fast excitation in spinal motor neurons, where they are expressed ubiquitously. AMPA receptor antagonist was able to prevent this acute degeneration in previous animal studies. The investigators aim to study the tolerability and safety of the novel AMPA antagonist, perampanel, in patients diagnosed with ALS. Perampanel [2-(2-oxo-1-phenyl-5- pyridin-2-yl-1,2-dihydropyridin-3-yl) benzonitrile] with its selective non-competitive AMPA antagonism, was recently approved for epilepsy. Various long-term trials studying perampanel in epilepsy showed favorable tolerability profile and most common side effects were mainly: dizziness, headache and somnolence. All patients presenting to Neurology clinics at AUBMC diagnosed with Amyotrophic Lateral Sclerosis, will be considered for the study. Investigators will obtain informed consents from all patients who agree to be enrolled in this study in accordance with institutional review board (IRB) requirements. Patients of both genders and over 18 years old who meet the El Escorial criteria for possible, probable or definite ALS and fit the inclusion criteria will be recruited. Subjects should not be started on riluzole for the past 30 days or stable on a dose of riluzole for at least 30 days prior to the screening process. In titration phase, perampanel dose will be increase by 2mg/day increments every one week to reach a maximum dose of 8 mg/day; reaching the maximum dose in four weeks. Treatment phase will be followed by washout period during which, dose will be tapered by 2mg/day every 5 days (over total of 15 days).

Start: December 2017

Safety and Tolerability of High Dose Biotin in Patients With Amyotrophic Lateral Sclerosis

This is a randomized double blinded randomized 2:1 study. The duration of the study is 6 month. The safety and tolerability of high doses of biotin (300 mg/ day) will be compared to placebo in patients with amyotrophic lateral sclerosis. Patients will be evaluated at baseline, 3, and 6 month. The primary outcome will be any adverse effects recorded. The secondary outcomes will be motor disability measured by ALS-FRS, change in Pulmonary function test parameters (FEV1- FVC), change in subject weight (in kg).

Start: January 2018

Study of Predictive Factors of Progression of Motor Neurone Disease

Amyotrophic lateral sclerosis (ALS) is a complex polymorph and devastating neurodegenerative disease. Although the pathophysiological mechanisms underlying the development of ALS remain to be fully elucidated, there have been significant advances in the understanding of ALS pathogenesis, with evidence emerging of a complex interaction between genetic factors and dysfunction of vital molecular pathways. However, the numerous randomized clinical trials (RCT) for ALS have failed to generate improved drug treatments. Biomarkers able to bring prognostic value and to distinguish the different endophenotypes of this polymorphic disease could help to better select clusters of patients in order to improve the RCT outcomes. However, little progress has been made in the development of viable diagnostic, prognostic and monitoring markers. This could be explained by common shortcomings, such as relatively small sample sizes, statistically underpowered study designs, lack of disease controls and poorly characterized patient cohorts. It is yet crucial that the investigators further develop and validate ALS biomarkers and incorporate these biomarkers into the drug development pipeline for ALS. The aim of the present study is therefore to determine the clinical, biological, imaging, and electrophysiological biomarkers of prognosis of survival without events (i.e. severe comorbidity, 24 hours of non invasive ventilation, tracheotomy). This is a prospective observational multicentric French study of a cohort of 1000 ALS patients. This large multimodal database will be open for international fruitful scientific collaborations.

Start: January 2015

Natural History and Clinical Features of Amyotrophic Lateral Sclerosis (ALS)

Describe the distribution of ALS in mainland China, to explore the differences in the number of ALS in different times, regions and populations in order to further explore the causes affecting the distribution of ALS; To investigate the cause of ALS in mainland China in the crowd disease development process and the corresponding characteristics change ; To explore the effect of prognosis of ALS;

Start: November 2019

Development and Test of a Headset for BCI Until Obtaining an Efficient and Comfortable System That Can be Used in Daily Practice by ALS People

"Brain-computer interfaces (BCIs) are computer-based systems that acquire brain signals, analyze them, and translate them into commands that are relayed to an output device to carry out a desired action. BCIs represent a very active and promising field of research among devices for people with severe motor disabilities. As the currently available systems correspond to research prototypes, they are not adapted to daily live situations. On the other hand, some systems have recently been commercialized, principally for video games but they are not satisfactory for use as a substitute technology in disability. A BCI's prototype for alternative communication using a virtual keyboard, the P300 Speller, has been developed by the National Institute for Research in Digital Science and Technology (Athena team - Nice University). This prototype includes an EEG-cap with gel based active electrodes. A recent study conducted on 20 patients with ALS (University Hospital, Nice) demonstrated the usability of the system and the patient satisfaction concerning the ease of use and utility. To achieve a system that can be used in daily live in severely disabled patients, technical developments are necessary. The investigators have conceptualized and developed an ergonomic, comfortable, headset, including dry electrodes to allow a prolonged use of the system. The purpose of the study conducted all along the development of the headset is to improve the developed system until a successful system is achieved. This study is a monocentric usability study conducted on ALS people.

Start: July 2020

Italian Validation of the Beaumont Behavioural Inventory (BBI)

Up to 50% of patients affected with amyotrophic lateral sclerosis (ALS) can show behavioral dysfunctions within the spectrum of frontotemporal degenerations (FTD) - namely, apathy, disinhibition, loss of sympathy/empathy, perseverative and stereotyped behaviours, dietary changes [Strong et al., 2017]. The Beaumont Behavioural Inventory (BBI) [Elamin et al., 2017] is a questionnaire designed for detecting FTD-spectrum behavioural changes in ALS patients. The present study aims at both validating the BBI in an Italian ALS population and determining normative cut-off values of the instrument.

Start: September 2019

A Clinical Trial of Pimozide in Patients With Amyotrophic Lateral Sclerosis (ALS)

This study will look at whether Pimozide may help to slow the progression of Amyotrophic Lateral Sclerosis. 100 people from several Canadian centres with ALS who have provided their consent will be randomly assigned into one of 2 groups. The first group will receive a dose of up to 2mg of Pimozide per day and the second group will receive placebo (lactose tablets). Subjects will be assigned randomly (like by a flip of a coin) to receive either Pimozide 2 mg per day or placebo tablets. There will be a fifty-fifty chance of receiving Pimozide or placebo. Participants will be on study medication up to 22 weeks, and on study up to 26 weeks. There are 8 clinic visits and 1 phone visit over the course of the Treatment Phase of the study. The second phase which is Observational, is optional with follow-up for up to 5 years from the end of the Treatment Phase.

Start: October 2017

Breath Stacking Technique Associated With Expiratory Muscle Training in Amyotrophic Lateral Sclerosis Patients

it will be conducted a randomized parallel controlled trial with patients diagnosed with Amyotrophic Lateral Sclerosis (ALS) to compare two techniques to lung recruitment and cough augmentation, to assess their effects on pulmonary function, global functionally, swallowing and ability to speech in these population.

Start: January 2020

Registration Study of Amyotrophic Lateral Sclerosis (ALS) in Mainland China

A multi-center registration study of natural history and clinical characteristics of ALS in mainland China

Start: March 2020

Efficacy of Cannabinoids in Amyotrophic Lateral Sclerosis or Motor Neurone Disease

This is a randomised, double-blind, placebo controlled study on a cannabis-based medicine extract (MediCabilis CBD Oil), in patients with Amyotrophic Lateral Sclerosis or Motor Neurone Disease. Participants will be randomised in a 1:1 ratio to receive MediCabilis CBD Oil or placebo oil. The treatment duration is 6 months with one-month safety follow up. Participants will be checked every month either face to face or via telephone and will be assessed to collect data for study objectives such as ALSFRS-R, Forced Vital Capacity, pain and spasticity score, and quality of life. Thirty (30) participants will be randomised.

Start: January 2019

Intraspinal Transplantation of Autologous ADRC in ALS Patients

The goal of our nonrandomized, open label study is to investigate the safety and efficacy of autologous adipose derived mesenchymal regenerative cells (ADRC) transplantation into the individuals with diagnosed amyotrophic lateral sclerosis (ALS). All enrolled patients will have a documented at least 3-months clinical and electrophisiological observation of ALS disease course prior to study enrollment. Each patient will recive 3 injections of ADRC every 3 months: an intraspinal injection followed by 2 subsequent intrathecal infusions. Safety, adverse events and efficacy will be confirmed by clinical, elecrophisiological ( EMG, MUNIX), neuroimmaging and spirometry together with functional (ALSFRS-R) and objective motor assesment (MRC and dynamometer).

Start: October 2015

The E-health Application To Modify ORal Energy Intake and Measure Outcomes REmotely in ALS Clinical Trial (EAT MORE2)

This is phase IIa feasibility and tolerability study of a mobile health (mHealth) application designed to study the effects of remote dietary counseling on disease progression and quality of life. The study will consist of two phases: Part I will consist of building and beta-testing the ALS Nutrition app and Part II will consist of enrolling a larger cohort of users into the app.

Start: March 2020

Treatment Continuation Study for Patients With ALS/MND Who Completed Study CMD-2019-001

Provides up to six months treatment with CuATSM for subjects who have successfully completed study CMD-2019-001

Start: March 2020

Colchicine for Amyotrophic Lateral Sclerosis

The study evaluates the effects of two different Colchicine doses (0.01mg/kg/day or 0.005 mg/kg/day) compared to placebo in Amyotrophic Lateral Sclerosis (ALS) patients. Disease progression as defined by changes in ALSFRS-r is the primary outcome measure. Other measures of clinical progression and survival, together with safety and tolerability of Colchicine in ALS patients will be assessed.

Start: April 2019