Primary Lateral SclerosisNCT00015444
Screening and Natural History: Primary Lateral Sclerosis and Related Disorders
An observational study of Primary Lateral Sclerosis, run by National Institute of Neurological Disorders and Stroke (NINDS) at 1 site in United States.
Trial 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… Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Primary Lateral Sclerosis
- How many people
- 189 participants
- Where
- 1 siteUnited States
- Study dates
- 1 May 2001to 24 Apr 2019
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Who may be able to join
- 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
Who cannot join
- 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
Trial locations and contacts
1 site in 1 country.
United States1 site
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda, Maryland, 20892
About this study
Objective:
The objectives of this protocol are:
Read more
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....
Read the detailed description
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.
- Conditions
- Primary Lateral Sclerosis
- Keywords
- Apparently Sporadic Spastic Paraplegia, Spasticity, Motor Neuron Disease, Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis, Muscle Stiffness, Involuntary Muscle Contraction, Isaacs Syndrome
- Medical subject headings
- Motor Neuron Disease, Muscle Spasticity, Amyotrophic Lateral Sclerosis, Dyskinesias, Isaacs Syndrome
What is being measured
The primary outcome is the main question the study is designed to answer.
The primary outcome of this protocol is to document the natural history of clinical progression in PLS, defined as the change in clinical measures of movement speed over time: finger tapping, timed gait, and time to read a standard passage.
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted19 Apr 2001 (estimated)
- Study start1 May 2001
- Study completion24 Apr 2019
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (4)Papers the sponsor has linked to this study.
- Other trials for Primary Lateral SclerosisStudies of the same condition, open and under way.
- More trials run by National Institute of Neurological Disorders and Stroke (NINDS)The sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Other
- Enrolment
- 189 participants (actual)
Sponsor and investigators
- Sponsor type
- US National Institutes of Health
- Responsible party
- Sponsor
- Investigators
- Mary Kay Floeter, M.D., Principal investigator, National Institute of Neurological Disorders and Stroke (NINDS)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00015444
- Sponsor's protocol ID
- 010145
- Other IDs
- 01-N-0145
- Status last verified
- 24 Apr 2019
- First submitted
- 18 Apr 2001
- Last updated
- 16 Dec 2019
- Results posted
- No
Publications
Leclerc KM, Landry FJ. Benign nocturnal leg cramps. Current controversies over use of quinine. Postgrad Med. 1996 Feb;99(2):177-8, 181-4.
PubMed 8632965Bentley S. Exercise-induced muscle cramp. Proposed mechanisms and management. Sports Med. 1996 Jun;21(6):409-20. doi: 10.2165/00007256-199621060-00003.
PubMed 8784961Auger RG. AAEM minimonograph #44: diseases associated with excess motor unit activity. Muscle Nerve. 1994 Nov;17(11):1250-63. doi: 10.1002/mus.880171103.
PubMed 7935547Clark MG, Smallwood Shoukry R, Huang CJ, Danielian LE, Bageac D, Floeter MK. Loss of functional connectivity is an early imaging marker in primary lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2018 Nov;19(7-8):562-569. doi: 10.1080/21678421.2018.1517180. Epub 2018 Oct 9.
PubMed 30299161
Study at a glance
From the registry record, last updated by the sponsor on 16 Dec 2019. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Screening and Natural History: Primary Lateral Sclerosis and Related Disorders
- ClinicalTrials.gov ID
- NCT00015444
- Acronym / sponsor ID
- 010145
- Conditions
- Primary Lateral Sclerosis
- Keywords
- Apparently Sporadic Spastic Paraplegia, Spasticity, Motor Neuron Disease, Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis, Muscle Stiffness, Involuntary Muscle Contraction, Isaacs Syndrome
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 189 participants (actual)
- Lead sponsor
- National Institute of Neurological Disorders and Stroke (NINDS)US National Institutes of Health
- Study start
- 1 May 2001
- Study completion
- 24 Apr 2019
- First posted
- 19 Apr 2001 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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....
Detailed description
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.
Study design
- Observational model
- Cohort
- Time perspective
- Other
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
Key inclusion criteria (4 listed)
- 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
Key exclusion criteria (7 listed)
- 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
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
The primary outcome of this protocol is to document the natural history of clinical progression in PLS, defined as the change in clinical measures of movement speed over time: finger tapping, timed gait, and time to read a standard passage.
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 16 Dec 2019.
- Background: PubMed 8632965Leclerc KM, Landry FJ. Benign nocturnal leg cramps. Current controversies over use of quinine. Postgrad Med. 1996 Feb;99(2):177-8, 181-4.
- Background: PubMed 8784961Bentley S. Exercise-induced muscle cramp. Proposed mechanisms and management. Sports Med. 1996 Jun;21(6):409-20. doi: 10.2165/00007256-199621060-00003.
- Background: PubMed 7935547Auger RG. AAEM minimonograph #44: diseases associated with excess motor unit activity. Muscle Nerve. 1994 Nov;17(11):1250-63. doi: 10.1002/mus.880171103.
- Derived from this study: PubMed 30299161Clark MG, Smallwood Shoukry R, Huang CJ, Danielian LE, Bageac D, Floeter MK. Loss of functional connectivity is an early imaging marker in primary lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2018 Nov;19(7-8):562-569. doi: 10.1080/21678421.2018.1517180. Epub 2018 Oct 9.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Mary Kay Floeter, M.D. principal investigator
National Institute of Neurological Disorders and Stroke (NINDS)
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Primary Lateral Sclerosis, run by National Institute of Neurological Disorders and Stroke (NINDS) at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 4 inclusion and 7 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 16 Dec 2019. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
The primary outcome of this protocol is to document the natural history of clinical progression in PLS, defined as the change in clinical measures of movement speed over time: finger tapping, timed gait, and time to read a standard passage.. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00015444, which also lists 4 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 16 Dec 2019.