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