Nuclear Magnetic Spectroscopy Imaging to Evaluate Primary Lateral Sclerosis, Hereditary Spastic Paraplegia and Amyotrophic Lateral Sclerosis - Full Text View

Purpose

This study will use a magnetic resonance imaging technique called nuclear magnetic spectroscopy (H-MRS) to define the pathology and progression of primary lateral sclerosis, hereditary spastic paraplegia and amyotrophic lateral sclerosis and assess the usefulness of this technique in evaluating patients' response to therapy. H-MRS will be used to examine metabolic changes in the parts of the brain and spinal cord (motor cortex and corticospinal tract) involved in movement.

Normal volunteers and patients with primary lateral sclerosis, hereditary spastic paraplegia or amyotrophic lateral sclerosis between 21 and 65 years of age may be eligible for this study. Participants will have up to five H-MRS studies, including baseline and follow-up tests. For this procedure, the subject lies on a stretcher that is moved into a strong magnetic field. Earplugs are worn to muffle the loud knocking noise that occurs during switching of radio frequencies. The subject will be asked to lie still during each scan, for 1 to 8 minutes at a time. Total scanning time varies from 20 minutes to 2 hours, with most examinations lasting between 45 and 90 minutes. Communication with the medical staff is possible at all times during the scan.

Condition
Primary Lateral Sclerosis Hereditary Spastic Paraplegia Amyotrophic Lateral Sclerosis


Study Type:	Observational
Official Title:	Nuclear Magnetic Spectroscopy for the Evaluation of Primary Lateral Sclerosis, Hereditary Spastic Paraplegia and Amyotrophic Lateral Sclerosis

Resource links provided by NLM:

Genetics Home Reference related topics: Silver syndrome Troyer syndrome amyotrophic lateral sclerosis infantile-onset ascending hereditary spastic paralysis juvenile primary lateral sclerosis spastic paraplegia type 11 spastic paraplegia type 15 spastic paraplegia type 2 spastic paraplegia type 31 spastic paraplegia type 3A spastic paraplegia type 4 spastic paraplegia type 7 spastic paraplegia type 8

MedlinePlus related topics: Amyotrophic Lateral Sclerosis

Genetic and Rare Diseases Information Center resources: Amyotrophic Lateral Sclerosis Paraplegia Hereditary Spastic Paraplegia Infantile-onset Ascending Hereditary Spastic Paralysis Primary Lateral Sclerosis Spastic Paraparesis Charcot-Marie-Tooth Disease Hereditary Neuropathy With Liability to Pressure Palsy Roussy Levy Syndrome

U.S. FDA Resources

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


Estimated Enrollment:	55
Study Start Date:	August 2001
Estimated Study Completion Date:	August 2003

Detailed Description:

Standard MRI imaging of the brain shows no consistent abnormalities in many degenerative disorders with progressive upper motor neuron symptoms, including primary lateral sclerosis, hereditary spastic paraparesis, and amyotrophic lateral sclerosis. Nuclear magnetic resonance spectroscopy (H-MRS) is able to evaluate certain cerebral metabolites that reflect the functioning and integrity of neurons. The objective of this protocol is to study metabolic changes in the motor cortex and corticospinal tract using MRS in these disorders to characterize the pathology, the progression of the diseases, and assess the utility of MRS to follow the response to therapies.

Eligibility


Ages Eligible for Study:	Child, Adult, Senior
Sexes Eligible for Study:	All
Accepts Healthy Volunteers:	Yes

Criteria

INCLUSION CRITERIA:

Adult patients with PLS, HSP, and ALS already evaluated by protocol #01-N-0148.

Adult normal volunteers

EXCLUSION CRITERIA:

Pregnancy, implanted devices such as pacemakers, medication pumps or defibrillators, metal in the cranium except the mouth, intracardiac lines, history of eye shrapnel injury or any other condition/device that may contraindicate or prevent the acquisition of an MRI.

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: NCT00023075

Locations


United States, Maryland
National Institute of Neurological Disorders and Stroke (NINDS)
Bethesda, Maryland, United States, 20892

Sponsors and Collaborators

National Institute of Neurological Disorders and Stroke (NINDS)

More Information

Publications:

Bonneau D, Rozet JM, Bulteau C, Berthier M, Mettey R, Gil R, Munnich A, Le Merrer M. X linked spastic paraplegia (SPG2): clinical heterogeneity at a single gene locus. J Med Genet. 1993 May;30(5):381-4.

Chan S, Shungu DC, Douglas-Akinwande A, Lange DJ, Rowland LP. Motor neuron diseases: comparison of single-voxel proton MR spectroscopy of the motor cortex with MR imaging of the brain. Radiology. 1999 Sep;212(3):763-9.

Comi G, Rovaris M, Leocani L. Review neuroimaging in amyotrophic lateral sclerosis. Eur J Neurol. 1999 Nov;6(6):629-37. Review.


ClinicalTrials.gov Identifier:	NCT00023075 History of Changes
Other Study ID Numbers:	010241 01-N-0241
Study First Received:	August 22, 2001
Last Updated:	March 3, 2008

Keywords provided by National Institutes of Health Clinical Center (CC):


MRI Neuromuscular Disease N-Acetyl Aspartate Choline Creatine Spasms Spasticity	Primary Lateral Sclerosis Hereditary Spastic Paraplegia Spastic Paraparesis Healthy Volunteer HV Normal Control

Additional relevant MeSH terms:


Sclerosis Motor Neuron Disease Amyotrophic Lateral Sclerosis Muscle Spasticity Paraplegia Spastic Paraplegia, Hereditary Pathologic Processes Neurodegenerative Diseases Nervous System Diseases Neuromuscular Diseases Spinal Cord Diseases Central Nervous System Diseases TDP-43 Proteinopathies Proteostasis Deficiencies Metabolic Diseases	Muscular Diseases Musculoskeletal Diseases Muscle Hypertonia Neuromuscular Manifestations Neurologic Manifestations Signs and Symptoms Paralysis Hereditary Sensory and Motor Neuropathy Nervous System Malformations Heredodegenerative Disorders, Nervous System Polyneuropathies Peripheral Nervous System Diseases Congenital Abnormalities Genetic Diseases, Inborn

ClinicalTrials.gov processed this record on August 23, 2017