Status:
COMPLETED
Brain Function in Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis
Lead Sponsor:
National Institute of Neurological Disorders and Stroke (NINDS)
Conditions:
Cerebral Cortex Dysfunction
Eligibility:
All Genders
18+ years
Brief Summary
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 voluntee...
Detailed Description
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 ...
Eligibility Criteria
Inclusion
- 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.
Exclusion
Key Trial Info
Start Date :
June 6 2006
Trial Type :
OBSERVATIONAL
Allocation :
ACTUAL
End Date :
August 3 2015
Estimated Enrollment :
77 Patients enrolled
Trial Details
Trial ID
NCT00334516
Start Date
June 6 2006
End Date
August 3 2015
Last Update
May 31 2019
Active Locations (1)
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1
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda, Maryland, United States, 20892