NIH Clinical Research Studies

Protocol Number: 02-N-0122

Active Accrual, Protocols Recruiting New Patients

Title:
Natural History and Immunopathogenesis of Stiff Person Syndrome (SPS)
Number:
02-N-0122
Summary:
This study will explore the role of various immune factors involved in producing the disease symptoms in stiff-person syndrome (SPS) and follow disease progression in patients. SPS is a progressive disease in which unexpected noises, touches or stressful events set off muscle spasms and stiffness. It is thought to be an autoimmune disease in which the body produces antibodies that attack certain healthy tissues. A better understanding of the disease may help researchers design new therapies.

Patients of any age with SPS may be eligible for this study, except those who:

- Lack of serum anti-GAD antibodies

- Have very advanced disease that precludes traveling

- Have severe cardiovascular, renal, or other end-organ-disease states

Candidates will be screened with a medical history and physical and neurological examinations to confirm the diagnosis of SPS.

After screening, those enrolled in the study will be followed at the NIH Clinical Center every 6 months for 2 years (months 6, 12, 18, and 24) to have the following tests and procedures:

- Physical and neurological examinations and review of symptoms (every visit)

- Blood draw for routine tests and for research studies (every visit)

- Stiffness assessment (every visit) - Patients are asked a series of questions about their stiffness, which physicians rate according to the number of stiff areas (e.g., 0-no stiff areas; 1-stiffness of the lower trunk; 2-stiffness of the upper trunk, etc.).

- Lymphapheresis (at the beginning of the study and at 12 months) - This is a procedure for collecting large quantities of white blood cells. A needle is placed in a vein in the arm. Blood flows from the vein through a plastic tube (catheter) into a machine that spins the blood, separating it into its components. The white blood cells (lymphocytes) are removed, and the rest of the blood-plasma, red cells and platelets-is returned to the body through a second needle placed in the other arm.

- Electrophysiologic studies - These studies include electromyography and nerve conduction testing. For electromyography, a small needle is inserted into a few muscles and the patient is asked to relax or to contract the muscles. The electrical activity of the muscle cells is recorded and analyzed by a computer. For nerve conduction testing, nerves are stimulated through small wire electrodes attached to the skin, and the response is recorded and analyzed.

- Lumbar puncture (at the beginning of the study and at 12 months) - This procedure is done to examine the cerebrospinal fluid (CSF), which bathes the brain and spinal cord. After a local anesthetic is administered, a needle is inserted in the space between the bones in the lower back where the CSF circulates below the spinal cord. About 2 tablespoons of fluid is collected through the needle.

Sponsoring Institute:
National Institute of Neurological Disorders and Stroke (NINDS)
Recruitment Detail
Type: Active Accrual Of New Subjects
Gender: Male & Female
Referral Letter Required: Yes
Population Exclusion(s): Children

Eligibility Criteria:
INCLUSION CRITERIA:

All patients who fulfill the recently revised clinical criteria for SPS.

EXCLUSION CRITERIA:

Lack of anti-GAD antibodies in the serum;

Very advanced disease state that precludes traveling;

Severe cardiovascular, renal, or other end-organ-disease states.

Special Instructions: Currently Not Provided
Keywords:
Antigenic Epitopes
Glutamic Acid Decarboxylase (GAD)
Auto-antibodies
Stiffness Index
SPS
Anti-GAD
MR Spectroscopy
GAD
Recruitment Keywords:
Stiff Person Syndrome
SPS
Conditions:
Stiff-Person Syndrome
Investigational Drug(s):
None
Investigational Device(s):
None

Contacts:
Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citations:
McEvoy KM Stiff-man syndrome Semin Neurol 1991 Sep;11(3):197-205 Review No abstract available PMID: 1947481

Levy LM, Dalakas MC, Floeter MK The stiff-person syndrome: an autoimmune disorder affecting neurotransmission ofgamma-aminobutyric acid Ann Intern Med 1999 Oct 5;131(7):522-30 Review PMID: 10507962

Dalakas MC, Fujii M, Li M, McElroy B The clinical spectrum of anti-GAD antibody-positive patients with stiff-personsyndrome Neurology 2000 Nov 28;55(10):1531-5PMID: 11094109

Active Accrual, Protocols Recruiting New Patients

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