Screening and Natural History: Primary Lateral Sclerosis and Related Disorders

This study is ongoing, but not recruiting participants.
Sponsor:
Information provided by (Responsible Party):
National Institutes of Health Clinical Center (CC) ( National Institute of Neurological Disorders and Stroke (NINDS) )
ClinicalTrials.gov Identifier:
NCT00015444
First received: April 18, 2001
Last updated: July 12, 2016
Last verified: July 2016
  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.


Condition
Primary Lateral Sclerosis

Study Type: Observational
Official Title: Screening and Natural History: Primary Lateral Sclerosis and Related Disorders

Resource links provided by NLM:


Further study details as provided by National Institutes of Health Clinical Center (CC):

Primary Outcome Measures:
  • 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. [ Designated as safety issue: No ]

Enrollment: 189
Study Start Date: April 2001
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.

  Eligibility

Ages Eligible for Study:   18 Years and older   (Adult, Senior)
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria
  • 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
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00015444

Locations
United States, Maryland
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda, Maryland, United States, 20892
Sponsors and Collaborators
National Institute of Neurological Disorders and Stroke (NINDS)
Investigators
Principal Investigator: Mary Kay Floeter, M.D. National Institute of Neurological Disorders and Stroke (NINDS)
  More Information

Additional Information:
Publications:
Responsible Party: National Institute of Neurological Disorders and Stroke (NINDS)
ClinicalTrials.gov Identifier: NCT00015444     History of Changes
Other Study ID Numbers: 010145  01-N-0145 
Study First Received: April 18, 2001
Last Updated: July 12, 2016
Health Authority: United States: Federal Government

Keywords provided by National Institutes of Health Clinical Center (CC):
Apparently Sporadic Spastic Paraplegia
Spasticity
Motor Neuron Disease
Amyotrophic Lateral Sclerosis
Primary Lateral Sclerosis
Muscle Stiffness
Involuntary Muscle Contraction
Isaacs Syndrome

Additional relevant MeSH terms:
Sclerosis
Motor Neuron Disease
Pathologic Processes
Neurodegenerative Diseases
Nervous System Diseases
Neuromuscular Diseases

ClinicalTrials.gov processed this record on August 29, 2016