Oxidative Stress in Motor Neuron Disease: COSMOS Add-On Study

NCT01143428 | Completed

• 2 other identifiers: 10012110-N-0121
• Study Type: observational
• Target: 10
• Locations: 1 country
• Sites: 1

Brief 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.

Conditions

Motor Neuron Disease; Primary Lateral Sclerosis

Keywords

Motor Neuron Disease; Primary Lateral Sclerosis; Oxidative Stress; Physiology; PLS

Outcome Measures

Primary Outcomes (1)

• Decline in ALSFRS score

Eligibility Criteria

• Age: 20 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Patients will be included if they:
• Are 20 years or older
• Have PLS, that is pure UMN dysfunction (spasticity, pathological hyperreflexia, pathological reflexes with or without motor weakness) of undetermined etiology
• Have been evaluated at NIH and are being willing to return for active follow-up for 3 years
• Had PLS symptom onset at least 5 years prior to the study enrollment but not more than 15 years
• Have normal nerve conduction studies and normal needle electrode examination performed within 12 months of the time of enrollment in this study
• Have no other definable diseases causing spasticity such as structural brain or spinal cord disease, metabolic diseases, paraneoplastic syndromes, hereditary diseases, infectious diseases, or other significant neurological abnormalities
• Have a reliable family caregiver who can assist in providing responses on telephone interviews and questionnaires if the proband has problems with speaking or writing
• Are fluent in English
• Ability to provide his/her own informed consent

You may not qualify if:

• Patients will be excluded if they:
• Have EMG evidence of active denervation or fasciculations in more than a few muscles with chronic neurogenic motor unit potentials at the time of enrollment
• Have only lower extremity involvement
• Have major medical diseases (e.g. active cancer, dialysis) that have required active medical treatment within the past 6 months
• Are participating in clinical treatment trials at the time of enrollment and acquisition of baseline biological samples (participation in clinical trials after the baseline visit is permitted)
• Are unwilling or unable to return for follow-up visits
• Have pacemakers or other implanted electrical devices, which might make TMS unsafe will be excluded from TMS testing

Sponsors & Collaborators

• National Institute of Neurological Disorders and Stroke (NINDS): lead

Study Sites (1)

National Institutes of Health Clinical Center, 9000 Rockville Pike

Bethesda, Maryland, 20892, United States

Location

Related Publications (3)

• Gordon PH, Cheng B, Katz IB, Mitsumoto H, Rowland LP. Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS. Neurology. 2009 Jun 2;72(22):1948-52. doi: 10.1212/WNL.0b013e3181a8269b.

  PMID: 19487653 BACKGROUND

• Strong MJ, Gordon PH. Primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis: discrete entities or spectrum? Amyotroph Lateral Scler Other Motor Neuron Disord. 2005 Mar;6(1):8-16. doi: 10.1080/14660820410021267.

  PMID: 16036421 BACKGROUND

• Tartaglia MC, Rowe A, Findlater K, Orange JB, Grace G, Strong MJ. Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up. Arch Neurol. 2007 Feb;64(2):232-6. doi: 10.1001/archneur.64.2.232.

  PMID: 17296839 BACKGROUND

MeSH Terms

Conditions

Motor Neuron Disease

Condition Hierarchy (Ancestors)

Neurodegenerative Diseases; Nervous System Diseases; Neuromuscular Diseases

Study Officials

• Mary Kay Floeter, M.D.

  National Institute of Neurological Disorders and Stroke (NINDS)

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Time Perspective: OTHER
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: June 11, 2010
• First Posted: June 14, 2010
• Study Start: May 13, 2010
• Study Completion: August 3, 2015
• Last Updated: November 5, 2019

Record last verified: 2015-08-03

Locations

US (1)