Brain Function in Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis
This study will test whether primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) affect parts of the brain responsible for thinking, planning, memory and emotion.
Healthy volunteers 18 years of age and older and patients with PLS and ALS may be eligible for this study.
Participants undergo the following procedures:
- Rating motor function: Subjects are asked to contract certain muscles in the face, arms and legs, to tap their finger on a keyboard rapidly, to walk 20 feet, and to read a paragraph out loud.
- Electroencephalography (EEG): The electrical activity of the brain (brain waves) is recorded while subjects tap their finger very slowly. For this test, electrodes are placed on the scalp using a cap or an adhesive substance. A conductive gel is used to fill the space between the electrodes and the scalp to ensure good contact.
- Surface electromyography (EMG): The electrical activity of the muscles is measured. Electrodes filled with a conductive gel are taped to the skin over the muscle tested.
- Neuropsychological testing: Testing may include questionnaires, pen-and-paper or computerized tests, and motor tasks.
- Magnetic resonance imaging (MRI): MRI uses a strong magnetic field and radio waves to produce images of the brain. The subject lies on a table that slides into the scanner. Scanning time varies from 20 minutes to 3 hours, with most scans lasting 45-90 minutes.
Cerebral Cortex Dysfunction
|Official Title:||Cortical Function in Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis|
|Study Start Date:||June 2006|
The goal of this study is to see whether patterns of cerebral cortex dysfunction differ in Primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS). The function of several regions of the cerebral cortex will be assessed by clinical, physiological, and neuropsychological tests. Magnetic resonance imaging will be carried out in a subset of patients to explore the correlation between functional and anatomical measures of these cortical areas.
30 patients with Primary lateral sclerosis who meet the diagnostic criteria for PLS proposed by Pringle and 30 patients with ALS who fulfill the revised El Escorial criteria for probable or definite ALS.
30 healthy volunteers will be studied to provide training and practice in using the rating scales and to provide age-matched controls for EEG and imaging studies.
A screening examination will be carried out under protocol 01-N-0145 to determine eligibility. Patients and caregivers will return for two or three visits for sessions of neuropsychological testing, a structured clinical neuropsychiatric evaluation, ratings of motor and clinical function, and measurement of movement-related cortical potentials with EEG. A subset of patients will undergo MRI scans for quantitative anatomical measures of cortical regions.
The primary outcome will be the classification of patients as normal or abnormal on neuropsychological testing of frontal cortex function. Correlations of the primary outcome with other variables - clinical measures of motor function, neuropsychiatric assessment will be examined. EEG measures of movement related cortical potentials and MR imaging findings will be analyzed in an exploratory fashion to determine whether these measures differ between PLS and ALS patients.
|United States, Maryland|
|National Institutes of Health Clinical Center, 9000 Rockville Pike|
|Bethesda, Maryland, United States, 20892|
|Principal Investigator:||Mary Kay Floeter, M.D.||National Institute of Neurological Disorders and Stroke (NINDS)|