Clinical Trials Logo

Clinical Trial Summary

Background:

- Primary lateral sclerosis (PLS) is a disorder in which nerve cells in the brain that control movement degenerate. The cause of PLS is not known, but some research has suggested that environmental factors that produce oxidative stress trigger PLS in people who carry certain genes. Oxidative stress is caused when the body makes chemicals called "free radicals" faster than its natural systems can break them down. Oxidative stress can be triggered by exposures to chemicals related to the bodily effects of lead, smoking, alcohol consumption, physical activity, and psychological stress. Chemicals produced by the body during oxidative stress can be measured in the blood and urine. Researchers are interested in studying the physical, neurological, and chemical effects of PLS to better understand the effects of oxidative stress on the disorder.

Objectives:

- To study the relation of oxidative stress to the diagnosis and progression of motor neuron disease.

Eligibility:

- Individuals 20 years of age or older who have been diagnosed with PLS, and have had symptoms of PLS for at least 5 but not more than 8 years and been previously enrolled in 01-N-0145 Screening: Neurologic Disorders with Muscle Stiffness

Design:

- Participants will have an initial study visit and three follow-up visits. Each visit will require approximately 3 days of testing at the National Institutes of Health Clinical Center.

- As part of this study, participants will have the following tests and procedures:

- Neurological examination to test muscle strength, sensation, coordination, and reflexes, as well as clarity of speech

- Tests of memory, attention, concentration, and thinking

- Surveys on oxidative stress, including questions on life, mood, jobs held, and habit

- Electromyography to record the electrical activity of muscles

- Transcranial magnetic stimulation to measure electrical activity translated from their brain to the muscles

- Blood, urine, and skin biopsy samples for testing and sample collection

- After the initial visit, participants will have three more visits, once each in the following 3 years.


Clinical Trial Description

Objective

Primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) are motor neuron disorders with different phenotypes that progress at very different rates. ALS is a rapidly progressive disease with a median survival less than 5 years. Patients with PLS have a slowly progressive course with a normal lifespan. One hypothesis is that oxidative stress affects the way in which different motor neuron disorders progress. To test this hypothesis, exposures to putative triggers of oxidative stress and biomarkers that may reflect oxidative stress will be assessed in patients with motor neuron disorders. A multicenter effort (the COSMOS study) has been initiated to accumulate sufficient numbers of ALS patients to address this hypothesis. As an add-on study, PLS patients will also be assessed in the multicenter effort. The objective of this protocol is to enroll PLS patients in this multicenter effort. The goal is to assess environmental factors and markers of oxidative stress in patients with established PLS.

Study Population

15 adult patients with PLS who have symptoms of pure upper motor neuron dysfunction for at least 5 but not more than 15 years.

Design

Patients will undergo a standard battery of clinical, physiological, and cognitive screening tests at enrollment, with scheduled follow-up evaluation visits every 12 months for 36 months. Blood and urine samples will be sent to collaborators at Columbia University for analysis of markers of oxidative injury and genetic risk factors. Patients will complete a self-administered nutritional survey and will be interviewed by phone by Columbia University investigators using questionnaires to assess environmental, occupational, lifestyle and psychosocial factors thought to be triggers of oxidative stress.

Outcome Measures

The Columbia University collaborators will combine data from several centers in a regression model correlating the slope of decline of the ALS-FRS score with an index of oxidative stress. ;


Study Design


Related Conditions & MeSH terms


NCT number NCT01143428
Study type Observational
Source National Institutes of Health Clinical Center (CC)
Contact
Status Completed
Phase
Start date May 13, 2010
Completion date August 3, 2015

See also
  Status Clinical Trial Phase
Terminated NCT04579666 - MERIDIAN: A Study to Evaluate the Efficacy and Safety of Pegcetacoplan in Adults With Amyotrophic Lateral Sclerosis (ALS) Phase 2
Completed NCT03706391 - Study of ALS Reversals 4: LifeTime Exposures
Active, not recruiting NCT03067857 - Autologous Bone Marrow-Derived Stem Cell Therapy for Motor Neuron Disease Phase 1/Phase 2
Completed NCT02464748 - Telehealth in Motor Neurone Disease N/A
Completed NCT00956488 - Supported Treadmill Ambulation Training (STAT) for Patients Diagnosed With Amyotrophic Lateral Aclerosis Phase 1/Phase 2
Terminated NCT00537446 - Effect of Noninvasive Ventilation on Lung Function in Amyotrophic Lateral Sclerosis N/A
Recruiting NCT04944940 - Clinical, Molecular and Imaging Biomarkers in Spinal and Bulbar Muscular Atrophy (SBMA)
Recruiting NCT03362658 - Novel MRI Biomarkers for Monitoring Disease Progression in ALS
Completed NCT00714636 - Cerebrospinal Fluid Repository N/A
Completed NCT00071435 - Brain Function in Primary Lateral Sclerosis N/A
Completed NCT00076687 - Safety Study of Botulinum Toxin Type A in Post-Upper Limb Stroke Patients With Reduced Lung Function Phase 2
Completed NCT00001220 - Ultrasound and Videofluoroscopy for Diagnosing Swallowing Disorders N/A
Completed NCT03487263 - Dose-Escalation, Safety and Pharmacokinetic Study of IC14 in Motor Neurone Disease Phase 1
Enrolling by invitation NCT05725759 - Rehabilitation in SOD1 ALS Treated With Tofersen
Active, not recruiting NCT03811301 - [BrainConnexion] - Neurodevice Phase I Trial N/A
Completed NCT02469896 - A Trial of Tocilizumab in ALS Subjects Phase 2
Completed NCT02011204 - Study of Electrical Impedance Myography (EIM) in ALS N/A
Completed NCT01495390 - A Longitudinal Study of Amyotrophic Lateral Sclerosis (ALS) Biomarkers N/A
Completed NCT02870634 - Phase 1 Dose Escalation and PK Study of Cu(II)ATSM in ALS/MND Phase 1
Recruiting NCT06201650 - Neurofilament Light Chain in Amyotrophic Lateral Sclerosis