Active clinical trials for "Amyotrophic Lateral Sclerosis"

Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular condition characterized by weakness, muscle wasting, fasciculations and increased reflexes. Depending on the site of onset, individuals with ALS progressively lose control of their skeletal muscles; bulbar or the extremities. As symptoms worsen and spread, muscle atrophy becomes apparent and upper motor neuron symptoms such as spasticity complicate gait (in lower limb involvement) and manual dexterity (in upper limb involvement). The patients progress to a state of profound disability and have great difficulty in communicating; some may even be entirely "locked in" to their bodies. The capacity for simple communication could greatly improve their quality of life. New technologies are giving people with disabilities alternate communication and control options. One such instrument is the EEG-based Brain-Computer Interface (BCI) which can provide both communication and control functions to those who have lost muscle control. By recording electroencephalographic (EEG) signals or brain waves from the scalp and then decoding them, the Wadsworth BCI allows people to make selections on a computer screen [i] In this study we will be investigating the feasibility of using EEG-based Brain-Computer Interface technology as a communication solution for individuals with ALS. The specific question addressed will be: Can individuals with ALS use the BCI for communication when they present with extreme loss of neuromuscular control and severe communication impairments? The goal of the project is to determine whether this device is a practical and realistic means for individuals with ALS to communicate. The study is intended to evaluate both the complexity of the system and the degree to which each participant will be able to communicate. Trials will consist of asking the subject to follow a series of simple instructions and complete certain tasks while using the BCI. This study design requires that the individual live in the Philadelphia region. Please contact the Wadsworth Center of the New York State Department of Health and State University of New York at Albany directly if you reside outside of this area.

This study will identify living veterans with amyotrophic lateral sclerosis (ALS) through a national registry that is being developed. Diagnosis of ALS will be verified by study neurologists via medical record review. Registry participation includes a bi-annual telephone interview to collect functional status data.

The primary objective of the study is to evaluate the feasibility of eliciting continuous narrative speech in different neurodegenerative and psychiatric indications, using remote, self-administered speech tasks, as measured by the average length of speech elicitation for each speech task during the first week of self-assessment. Secondary objectives include (1) evaluating the reliability of speech tasks in the remote self-administered setting, as measured by the intra- and inter-subject variance; (2) accessing the adherence of speech tasks in this setting, as measured by the subject average fraction of days during the first week, where at least one task response is submitted; (3) evaluating the feasibility of using speech tasks in the setting of a telemedicine videoconference, as measured by the average length of speech elicited in each group; (4) evaluate whether a set of acoustic and linguistic patterns can detect each indication, compare to either a control group or all other indications, as measured by the area under the receiver operating characteristic curve (AUC), sensitivity, specificity and Cohen's kappa of the relevant binary classifier; (5) evaluating how the performance of such algorithms can be impacted by speaker and environment covariates, as measured by the Kendall rank correlation coefficient of the AUC of each classifier and each of age group, gender and speech-to-reverberation modulation energy ratio.

Amyotrophic Lateral Sclerosis (ALS) is a degenerative neuromuscular disease, progressing inexorably to respiratory failure, the by involvement of respiratory muscles, the commitment with most impact on the prognosis of ALS. According to current knowledge, the clinical presentation of the disease is characterized by spinal or bulbar involvement, the latter being associated with a worse prognosis. There are multiple factors described in the aetiology of ALS, as the successive damage the motor neuron, which can happen in high-impact athletes, or exposure to heavy metals. Genetic mutations are also described, being associated to a higher prevalence of ALS. Data from retrospective studies with ALS populations reveal a prevalence of 4-8 cases per 100,000 persons. Research carried out in Trás-os-Montes e Alto Douro region (Northeast of Portugal) shows a high prevalence of ALS, with near 10 cases per 100,000 persons, with a recent increase in the bulbar involvement. The reasons for the high prevalence of ALS in this region are unknown.

This is a case-control study performed on a biological collection. The polymorphisms present on a pre-defined list of genes will be studied for 400 Amyotrophic Lateral Sclerosis (sporadic type) DNA samples and 400 control DNA samples.

This study proposes to identify acoustic and perceptual markers related to upper motor neuron (UMN) degeneration and lower motor neuron (LMN) degeneration in the dysarthria of patients with amyotrophic lateral sclerosis (ALS) which involves the degeneration of both systems. ALS patients will be gathered in clinical groups according to electromyogram (EMG) and clinical signs observed in the bulbar site. UMN signs are defined as jaw clonus, gag reflex and pseudobulbar features (lability). LMN signs are defined as lingual atrophy and fasciculations. The dysarthria will be compared to dysarthria of patients involving an exclusive UMN system degeneration (in primitive lateral sclerosis) and an exclusive LMN system degeneration (Kennedy's disease). Patients will be compared to the controls who permitted to establish the standards of the "MonPaGe" tool. MonPaGe is a computerized tool based on a multidimensional and quantified assessment of voice and speech, by a set of targeted acoustic and perceptual criteria.

This study is intended to obtain clinical information and establish a repository of DNA, RNA, peripheral blood monocyte, lymphocyte and skin tissue samples from people with ALS and related neurodegenerative motor neuron diseases, people with a family history of these conditions, and healthy people with no family history of these disorders. The samples will be used in future research to learn about how these disorders affect people, what causes these conditions, and how the investigators can tell when someone has this kind of disease. Future research may also include the generation of stem cells from stored blood cell and skin cell samples. Participants will not be paid for taking part in this study.

There is a great need for the development of sensitive outcomes that allow experimental drugs to be tested in human subjects more efficiently. If we could more precisely measure whether an experimental drug slows the progression of ALS or other neuromuscular diseases, this would allow more drugs to be tested quicker and at less expense. We have developed a new device that accurately measures isometric strength called: Accurate Test of Limb Isometric Strength (ATLIS). This device was designed to be portable, quick, and easy to use, while generating accurate and reliable, interval level data. This study will enable us to test the reliability and validity of ATLIS.

This study will examine whether the motor cortex (the part of the brain that controls movement) works properly in patients with primary lateral sclerosis (PLS), a disorder in which voluntary movements are very slow. Healthy volunteers between 40 and 75 years of age and patients with ascending PLS (a subset of PLS) may be eligible for this study. Patients with ascending PLS have a slowing of finger-tapping movements that corresponds to a particular abnormality of certain neuronal (nerve cell) activity. Participants perform a finger-tapping reaction time exercise while brain wave activity (electroencephalography, or EEG) and muscle activity (electroymogram, or EMG) are measured. The subject is seated in front of a computer screen. A signal appears on the screen and the subject taps a key as quickly as possible in response to the signal. For the EEG, brain activity is recorded by placing electrodes (small metal discs) on the scalp with an electrode cap or glue-like substance. A conductive gel is used to fill the space between the electrodes and the scalp to make sure there is good contact between them. The brain waves are recorded while the subject taps his or her fingers very slowly. For the surface EMG, electrodes filled with a conductive gel are taped to the skin. Participants also undergo magnetic resonance imaging (MRI). This test uses a strong magnetic field and radio waves to obtain images of the brain. During the procedure, the subject lies still on a table that can slide in and out of the scanner - a narrow metal cylinder. Scanning time varies from 20 minutes to 3 hours, with most scans lasting between 45 and 90 minutes. Subjects can communicate with the MRI staff at all times during the scan and can ask to be moved out of the machine at any time.

The purpose of this study is to find out if changes in speech can signal changes in the ability to think or remember. ALS patients with and without cognitive dysfunction will be followed for one year. Every three months, patients will undergo a series of cognitive and basic clinical outcomes tests. In addition, participants will take home a study-provided tablet on which they will complete weekly speech recording activities.