Brain Function in Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis
| Status: | Completed |
|---|---|
| Conditions: | Neurology, Neurology, Neurology |
| Therapuetic Areas: | Neurology |
| Healthy: | No |
| Age Range: | 18 - Any |
| Updated: | 12/27/2018 |
| Start Date: | June 6, 2006 |
| End Date: | August 3, 2015 |
Cortical 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.
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.
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.
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.
- PRIMARY LATERAL SCLEROSIS INCLUSION CRITERIA:
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:
- Insidious onset in adulthood, progressive course
- No family history
- Disease duration greater than 3 years without lower motor neuron clinical signs
- Clinical signs restricted to corticospinal/corticobulbar tract dysfunction
Imaging:
- Brain MRI normal (except cortical atrophy)
- Normal cervical spine
- Negative chest X-ray, negative mammograms in women
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.
- Probable ALS: Upper and Lower motor neuron signs are present in more than two regions,
but some UMN signs must be rostral to LMN signs.
- Definite ALS: Upper and Lower motor neuron signs are present in more than three
regions.
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.
- Pacemakers or other implanted electrical devices, brain stimulators, dental implants,
aneurysm clips (metal clips on the wall of a large artery), metallic prostheses
(including metal pins and rods, heart valves, and cochlear implants), permanent
eyeliner, implanted delivery pumps, or shrapnel fragments.
- Occupational exposure to metal fragments, as in welders and metal workers, which may
lead to small fragments in the eye.
- Pregnancy. Women of childbearing potential will undergo urine pregnancy testing before
MRI scanning.
- Fear of confined spaces.
We found this trial at
1
site
9000 Rockville Pike
Bethesda, Maryland 20892
Bethesda, Maryland 20892
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