View clinical trials related to Stiff-Person Syndrome.
Filter by:This study will test whether rituximab (RITUXAN (Trademark)) can relieve symptoms of stiff person syndrome (SPS), a progressive disease that causes stiffness of the muscles and muscle spasms induced by unexpected noises, touches, or stressful events. People with SPS may have certain proteins in their blood called anti-GAD antibodies that may cause some of the symptoms of the disease. Rituximab, a drug approved to treat lymphomas, targets certain white blood cells that produce antibodies. This study will see if rituximab can also be effective in patients with SPS who have high anti-GAD antibodies. Patients between 25 and 80 years of age with SPS may be eligible for this study. Candidates are screened with a medical history, physical examination, and blood tests. Participants undergo the following tests and procedures: - Rituximab or placebo treatment: Patients are randomly assigned to receive two infusions by vein of either rituximab or placebo (a look-alike solution with no active ingredient) 2 weeks apart. The infusions last from 3 to 4 hours, but may take as long as 16 hours if the rate must be slowed for any reason. Patients are followed monthly for up to 6 months and then every 2 months for up to 1 year after treatment. - Medical history and interview, physical and neurological examinations: Patients are questioned about their vaccination history, medical, surgical, and psychiatric history, exposure to environmental toxins or viruses, and family and social history, including habits and employment. - Blood drawing: Blood samples are collected before the two infusions and at all follow-up visits. - Apheresis: For this procedure, which is used to collect white blood cells, blood is collected through a needle in an arm vein, similar to donating blood. The blood flows from the vein through a catheter (plastic tube) into a machine that separates it into its components by centrifugation (spinning). The white cells are removed and the rest of the blood (red cells, plasma and platelets) is returned to the body through a second needle in the other arm. The procedure takes about 60 to 90 minutes. - Lumbar puncture (spinal tap): Lumbar puncture is done to sample a small amount of cerebrospinal fluid (CSF, the fluid that bathes the brain and spinal cord), for analysis. For this procedure, the patient is given a local anesthetic and a needle is inserted into the space between the bones in the lower back where the CSF circulates below the spinal cord. A small amount of fluid is withdrawn through the needle.
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.
Stiff-man Syndrome (SMS) is a chronic, progressive disorder of the nervous system. It is associated with painful muscle spasms and rigidity involving muscles of the limbs, trunk, and neck. The cause of the disease is unknown, but researchers believe it may be a result of an autoimmune process. Patients with Stiff-man Syndrome may produce antibodies that attack enzymes required for the normal function of the nervous system. Steroids, plasmapheresis, and intravenous immunoglobulin (IVIg) have been given to relieve some of the symptoms of Stiff-man Syndrome. However, none of these therapies have proven to be significantly effective. This study will attempt to determine the effectiveness of intravenous immunoglobulin (IVIg) for the treatment of Stiff-mann Syndrome. Patients participating in this study will be divided into two groups. Group one will receive 2 injections of IVIg once a month for three months. Group two will receive 2 injections of placebo "inactive sterile water" once a month for three months. Following the three months of treatment, group one will begin taking the placebo and group two will begin taking IVIg for an additional 3 months. The drug will be considered effective if patients receiving it experience a significant improvement in muscle function, mobility, and stiffness.