PRISM Registry: Pseudobulbar Affect Registry Series
Alzheimer's DiseaseAmyotrophic Lateral Sclerosis (ALS)4 morePBA is a neurologic condition that is estimated to impact over a million patients and their families in the United States. PBA occurs secondary to an otherwise unrelated neurologic disease or injury, and manifests as involuntary, frequent, and disruptive outbursts of crying and/or laughing. Progress has been made in better understanding this debilitating condition, but much more needs to be done. That's why a new PBA patient registry, PRISM (Pseudobulbar Affect RegIstry Series), has been initiated. The goal of PRISM is to establish the prevalence and quality of life (QOL) impact of PBA in patients with underlying neurologic conditions including Alzheimer's disease Amyotrophic lateral sclerosis Multiple sclerosis Parkinson's disease Stroke Traumatic brain injury Because this is an observational registry, it doesn't require you to intervene with any specific treatment or procedure. Your participation allows the PRISM registry to collect and analyze data from your site and also compare it to national numbers captured in the PRISM registry about PBA across all of the major at-risk neurologic populations.
Collection of Blood Samples for DNA in Motor Neuron Disease
Motor Neuron DiseasesThis study will collect blood samples from patients with primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) to be used for research on genetic causes of motor neuron diseases and other neurological disorders. Patients 18 years of age and older with PLS or ALS may be eligible for this study. Candidates are screened with a medical history, physical examination and diagnostic tests. Participants provide a blood sample. The sample, along with masked (anonymous) medical and family history information are sent to the NINDS Respository at the Coriell Cell Repositories in Camden, NJ. This facility collects, stores and distributes medical research information and cell cultures and DNA samples to researchers at hospitals, universities and commercial organizations. The blood sample has an identification number that is unrelated to any identifying information for the patient and cannot be tracked back to the patient.
Nuclear Magnetic Spectroscopy Imaging to Evaluate Primary Lateral Sclerosis, Hereditary Spastic...
Primary Lateral SclerosisHereditary Spastic Paraplegia1 moreThis study will use a magnetic resonance imaging technique called nuclear magnetic spectroscopy (H-MRS) to define the pathology and progression of primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis and assess the usefulness of this technique in evaluating patients' response to therapy. H-MRS will be used to examine metabolic changes in the parts of the brain and spinal cord (motor cortex and corticospinal tract) involved in movement. Normal volunteers and patients with primary lateral sclerosis, hereditary spastic paraplegia or amyotrophic lateral sclerosis between 21 and 65 years of age may be eligible for this study. Participants will have up to five H-MRS studies, including baseline and follow-up tests. For this procedure, the subject lies on a stretcher that is moved into a strong magnetic field. Earplugs are worn to muffle the loud knocking noise that occurs during switching of radio frequencies. The subject will be asked to lie still during each scan, for 1 to 8 minutes at a time. Total scanning time varies from 20 minutes to 2 hours, with most examinations lasting between 45 and 90 minutes. Communication with the medical staff is possible at all times during the scan.
Amyotrophic Lateral Sclerosis (ALS) Gulf War Study
Amyotrophic Lateral SclerosisRecently, concern has arisen regarding a possible elevated occurrence of ALS among veterans who served in the Persian Gulf during Operations Desert Shield (August 2, 1990 - January 15, 1991), Desert Storm (January 16, 1991 - February 28, 1991) and Clean-up (March 1, 1991 - July 31, 1991). This study involves an epidemiologic investigation into the occurrence of ALS among veterans of the Gulf War. This study will further define the epidemiology of this neurological disease among younger individuals while determining whether there is a higher than expected occurrence. It will also ascertain the etiologic importance of deployment to the Persian Gulf and exposure to specific environmental factors in that geographic area. VA is leading this joint federal government epidemiologic study that also involves DoD, HHS, CDC, and academic centers of excellence in neurology, with advice from the ALS Association.
Intermediate Expanded Access Protocol for ALS
Amyotrophic Lateral SclerosisThis is a single-center intermediate expanded access program to provide access to the investigational product, CNM-Au8, up to 40 participants diagnosed with ALS.
Venous Disease Thromboembolic and Amyotrophic Lateral Sclerosis
Lateral Amyotrophic SclerosisVenous Thrombo-embolic DeseaseVenous thrombo-embolic (VTE) rates could be high in patients with amyotrophic lateral sclerosis (ALS). Indeed, the rate of VTE in this specific population could be 7-fold higher in this population. Predictiv factors of VTE in patients with ALS are mobility reduction and neurological paralysis. However, to our knowledge, medical littérature is poor concerning VTE and ALS association. Our first aim is to define annual rate of VTE in ALS population.Then we aim to identify predictiv factors of VTE in this specific population. The studied population is Brest universitary hospital cohort of ALS patient included between 2000 and 2019.
Delineating Swallowing Impairment and Decline in ALS
Amyotrophic Lateral SclerosisThe purpose of this study is to 1) evaluate the discriminant ability of simple clinical markers to detect swallowing impairment in individuals with ALS, 2) develop and validate a minimally invasive clinical screening tool for use at multidisciplinary ALS clinics, and 3) determine the natural history of swallowing impairment and decline in ALS.
Predictive Factors for the Diagnosis of Early Noninvasive Ventilation Equipment
Amyotrophic Lateral SclerosisTo compensate for insufficiency of diagnostic tools, the present study propose to look for the predictive factors of an early fitting by noninvasive ventilation.
Quantitative Neuromuscular Ultrasonography in Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic Lateral SclerosisAmyotrophic Lateral Sclerosis (ALS) is a progressive and fatal neurological disease. Nonspecific symptoms lead to a delay in the diagnosis, only confirmed by the electrophysiologic study. Objectives. To establish the diagnostic value of ultrasonography in ALS. To evaluate the rate of muscle and nerve degeneration by ultrasonography in patients with ALS. To check the relationship between ultrasound, clinical variables and functional tests in patients with ALS. Methods. A longitudinal observational study in a consecutive sample of patients diagnosed with ALS will be realized. All the patients will be examined 3 times during 6 months and capabilities associated with ALS and muscle strength will be assessed. Bilateral and cross sectional ultrasonography of several muscles and also median and tibial nerves will be performed. All the images will be processed and analyzed for obtaining morphometric variables (muscle thickness and nerve area) and textural ones (echogenic variation, entropy, homogeneity, textural contrast and correlation). Frequency of twitches will be also recorded. After longitudinal study, a survival study will be performed in relation to functional and sonographic variables.
In-situ Cytokines Expression in the CNS in Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic Lateral SclerosisThe investigators aim at exploring the differential/topographical in-situ expression of cytokines in the central nervous system (CNS) of patients who died with amyotrophic lateral sclerosis (ALS), using archived histopathology slides and residual paraffin blocks from autopsied cases. Previous studies from the investigators and other groups showed that inflammatory cytokines are implicated in several neurological affections, particularly neurodegenerative conditions. However, in-situ cytokine expression has never been studied so far in ALS. The investigators wanted to see if these neuro-mediators are involved in the neuromolecular chain/cascade underlying ALS.