Home
Search
Study Topics
Glossary
|
|
|
|
|
|
Sponsored by: |
National Institute of Neurological Disorders and Stroke (NINDS) |
---|---|
Information provided by: | National Institutes of Health Clinical Center (CC) |
ClinicalTrials.gov Identifier: | NCT00334516 |
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:
Condition |
---|
Cerebral Cortex Dysfunction |
Study Type: | Observational |
Official Title: | Cortical Function in Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis |
Estimated Enrollment: | 90 |
Study Start Date: | June 2006 |
Objective:
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.
Study Population:
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.
Design:
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.
Outcome Measures:
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.
Ages Eligible for Study: | 18 Years and older |
Genders Eligible for Study: | Both |
Accepts Healthy Volunteers: | Yes |
Patients with PLS, aged 18 and older, must meet the diagnostic criteria proposed by Pringle (1992), incorporating Santa Clara (2004) consensus for pure PLS.
Clinical:
Imaging:
EMG after 3 years, but within last 3 years, showing no active denervation.
Normal serological studies for serum chemistry, Vitamin B12, Vitamin E levels, very long-chain fatty acids.
Negative serology for syphilis, Lyme disease, HTLV 1 and 2.
AMYOTROPHIC LATERAL SCLEROSIS INCLUSION CRITERIA:
Patients with ALS, aged 18 and older, must fulfill the revised El Escorial criteria for probable or definite ALS.
HEALTHY VOLUNTEERS INCLUSION CRITERIA:
Healthy adults, aged 18 and older, willing to participate, and able to give informed consent.
EXCLUSION CRITERIA:
Cognitive impairment of such severity that patients cannot provide assent to participate in the protocol. During the screening examination, we will question the patients to see if they understand that they are being evaluated, the types of tests we are using, and the response required. If they are unable to answer these questions, they will be excluded from the study.
History or evidence of co-existence of a second neurological disorder, such as stroke, epilepsy, Parkinson's disease, polio, ataxia or neuropathy.
History of traumatic brain injury, skull defects or neurosurgery.
Patients who fulfill the inclusion criteria for ALS and PLS but have the following conditions will be allowed to participate in cognitive studies and EEG studies, but will be excluded from MRI testing.
Contact: Patient Recruitment and Public Liaison Office | (800) 411-1222 | prpl@mail.cc.nih.gov |
Contact: TTY | 1-866-411-1010 |
United States, Maryland | |
National Institutes of Health Clinical Center, 9000 Rockville Pike | Recruiting |
Bethesda, Maryland, United States, 20892 |
Study ID Numbers: | 060174, 06-N-0174 |
Study First Received: | June 7, 2006 |
Last Updated: | October 1, 2008 |
ClinicalTrials.gov Identifier: | NCT00334516 |
Health Authority: | United States: Federal Government |
Motor Neuron Disease Cognitive Testing Movement-Related Potential MRI Neuropsychologic Testing Primary Lateral Sclerosis |
PLS Amyotrophic Lateral Sclerosis ALS Healthy Volunteer HV |
Amyotrophic lateral sclerosis Neuromuscular Diseases Spinal Cord Diseases Amyotrophic Lateral Sclerosis Central Nervous System Diseases Lou Gehrig's disease |
Sclerosis Degenerative motor system disease Healthy Neurodegenerative Diseases Motor neuron disease Motor Neuron Disease |
Pathologic Processes Nervous System Diseases |