Study Purpose:
Objective:The objectives of this protocol are:
to develop and maintain a repository of clinically characterized patients with primary lateral sclerosis for future research protocols,
to characterize the natural history of neurodegenerative disorders with corticospinal neuron degeneration,
to investigate proposed etiologies, risk factors, and biomarkers for the development of these disorders and for disease progression
Study Population:
240 patients with adult-onset progressive spasticity with a diagnosis of primary lateral sclerosis or related upper motor neuron disorder
Design:
Patients who have been referred by physicians for primary lateral sclerosis will undergo a screening evaluation at the first visit. The screening visit will include review of outside medical records, neurological examination, and diagnostic testing to determine possible causes of spasticity. Patients fulfilling the clinical criteria for primary lateral sclerosis by history or examination will be followed to determine the natural history of this disorder. Measures of motor and cognitive function will be made at baseline and follow-up visits to follow clinical progression. Magnetic resonance imaging will be carried out to determine if imaging changes occur over time. Patients identified in this protocol who are eligible for other research protocols will be invited to participate in additional protocols.
Outcome Measures:
Clinical progression will be documented by measures of finger-tapping, timed gait, speech. The association between clinical progression and MRI measures will be assessed as a secondary outcome....
Study Status:
Not recruiting
Disease:
Primary Lateral Sclerosis
Study Type:
Observational
Type of Intervention:
N/A
Intervention Name:
N/A
Placebo:
N/A
Phase:
N/A
Study Chair(s)/Principal Investigator(s):
Mary Kay Floeter, M.D., National Institute of Neurological Disorders and Stroke (NINDS)
Clinicaltrials.gov ID:
Neals Affiliated?
No
Coordinating Center Contact Information
National Institutes of Health Clinical Center
9000 Rockville Pike
Bethesda, Maryland, 20892 United States
Full Study Summary:
Objective:
The objectives of this protocol are:
- to develop and maintain a repository of clinically characterized patients with primary lateral sclerosis for future research protocols,
- to characterize the natural history of neurodegenerative disorders with corticospinal neuron degeneration,
- to investigate proposed etiologies, risk factors, and biomarkers for the development of these disorders and for disease progression
Study Population:
240 patients with adult-onset progressive spasticity with a diagnosis of primary lateral sclerosis or related upper motor neuron disorder
Design:
Patients who have been referred by physicians for primary lateral sclerosis will undergo a screening evaluation at the first visit. The screening visit will include review of outside medical records, neurological examination, and diagnostic testing to determine possible causes of spasticity. Patients fulfilling the clinical criteria for primary lateral sclerosis by history or examination will be followed to determine the natural history of this disorder. Measures of motor and cognitive function will be made at baseline and follow-up visits to follow clinical progression. Magnetic resonance imaging will be carried out to determine if imaging changes occur over time. Blood samples may be collected for measurement of potential etiologies of PLS, including risk factor genes. Patients identified in this protocol who are eligible for other research protocols will be invited to participate in additional protocols.
Outcome Measures:
Clinical progression will be documented by measures of finger-tapping, timed gait, speech. The association between clinical progression and MRI measures will be assessed as a secondary outcome.
The objectives of this protocol are:
- to develop and maintain a repository of clinically characterized patients with primary lateral sclerosis for future research protocols,
- to characterize the natural history of neurodegenerative disorders with corticospinal neuron degeneration,
- to investigate proposed etiologies, risk factors, and biomarkers for the development of these disorders and for disease progression
Study Population:
240 patients with adult-onset progressive spasticity with a diagnosis of primary lateral sclerosis or related upper motor neuron disorder
Design:
Patients who have been referred by physicians for primary lateral sclerosis will undergo a screening evaluation at the first visit. The screening visit will include review of outside medical records, neurological examination, and diagnostic testing to determine possible causes of spasticity. Patients fulfilling the clinical criteria for primary lateral sclerosis by history or examination will be followed to determine the natural history of this disorder. Measures of motor and cognitive function will be made at baseline and follow-up visits to follow clinical progression. Magnetic resonance imaging will be carried out to determine if imaging changes occur over time. Blood samples may be collected for measurement of potential etiologies of PLS, including risk factor genes. Patients identified in this protocol who are eligible for other research protocols will be invited to participate in additional protocols.
Outcome Measures:
Clinical progression will be documented by measures of finger-tapping, timed gait, speech. The association between clinical progression and MRI measures will be assessed as a secondary outcome.
Study Sponsor:
National Institute of Neurological Disorders and Stroke (NINDS)
Estimated Enrollment:
189
Estimated Study Start Date:
03 / 31 / 2001
Estimated Study Completion Date:
11 / 02 / 2016
Posting Last Modified Date:
12 / 16 / 2019
Date Study Added to neals.org:
04 / 19 / 2001
Minimum Age:
18 Years
Maximum Age:
N/A
- INCLUSION CRITERIA:- Age 18 years of age or older
- Adult onset of progressive spasticity
- No family history of a similar disorder
- Able to provide consent or with a legally-authorized representative who can provide consent
EXCLUSION CRITERIA:
- History of stroke, cerebral palsy, traumatic brain injury or other known etiology of spasticity
- Non-neurological disorders producing muscle stiffness, such as fasciitis or rheumatological conditions
- Disorders in which pain limits the ability to move muscles, such as fibromyalgia or complex regional pain syndromes
- Profound weakness of voluntary movement
- Inability to travel to NIH
- Anticoagulation will be an exclusion for needle EMG studies
- Implanted devices or metal fragments in the brain or spinal cord will be an exclusion for MRI scanning
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda, Maryland
20892
United States