Protocol Number: 04-N-0017

Title:

Movement Related Cortical Potentials in Primary Lateral Sclerosis

Number:

04-N-0017

Summary:

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.

Sponsoring Institute:

National Institute of Neurological Disorders and Stroke (NINDS)

Recruitment Detail

Type: Active Accrual Of New Subjects

Gender: Male & Female

Referral Letter Required: Yes

Population Exclusion(s): Children

Eligibility Criteria:

INCLUSION CRITERIA:

Patients

1. Ages 40-75

2. Fulfill the proposed diagnostic criteria for PLS of Pringle 1992.

3. Have an ascending course of symptom progression.

4. Slow hand movements, as determined by measuring finger-tapping speeds of 20-65 taps in 15 s.

5. Absent motor evoked potentials in hand muscles with 100% output of the MagStim200 transcranial magnetic stimulator.

Normal Volunteers

Healthy adult volunteers ages 40-75 who are willing to participate.

EXCLUSION CRITERIA:

1. Neurological diseases or conditions other than PLS.

2. Implanted devices, such as pumps, pacemakers, or metal fragments in the skull or eye.

3. Pregnancy as determined by urine pregnancy test within 48 hours of MRI study in women of childbearing

Special Instructions: Currently Not Provided

Keywords:

EEG

Voluntary Movement

Motor Cortex

Reaction Time

Corticospinal

Primary Lateral Sclerosis

PLS

Healthy Volunteer

HV

Recruitment Keywords:

None

Conditions:

Motor Neuron Disease

Investigational Drug(s):

None

Investigational Device(s):

None

Contacts:

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citations:

Rowland LP. Primary lateral sclerosis: disease, syndrome, both or neither? J Neurol Sci. 1999 Nov 15;170(1):1-4. Review.

Swash M, Desai J, Misra VP. What is primary lateral sclerosis?

J Neurol Sci. 1999 Nov 15;170(1):5-10. Review.

Sasaki S, Iwata M. Ultrastructural study of Betz cells in the primary motor cortex of the human brain. J Anat. 2001 Dec;199(Pt 6):699-708.

• Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC.
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