Protocol Number: 05-HG-0236
Four patients with HIBM will be admitted to this study at the NIH Clinical Center for evaluation and IG treatment. The evaluation lasts about 1 month. After completing baseline studies (see below), patients receive two intravenous doses of immune globulin (on days 6 and 7), followed by measurement of muscle strength 2 days later (day 9). They receive additional IG infusions on days 13, 20, and 27. A final set of tests is performed on day 29. Patients may leave the hospital on pass when no studies are being done. A patient's initial evaluation includes: -History and physical examination, neurological examination, eye examination -24-hour urine collection -Blood tests on two separate days -Photographs showing the extent of muscle affected -Chest x-ray, electrocardiogram (EKG), and echocardiogram -Two muscle biopsies, one before and one after the IG treatments. For this procedure, a small sample of muscle tissue is surgically removed for examination under the microscope. -Muscle strength and endurance testing, including the following: The patient uses pulleys attached to machines that measure the strength of 24 different muscle groups The patient walks for 6 minutes and performs exercises To evaluate swallowing, the patient swallows a thick substance called barium The patient's tongue strength is measured using a specialized instrument. -Magnetic resonance imaging (MRI) of the muscles of the thigh or calf: MRI uses a magnetic field and radio waves to produce detailed pictures of organs and tissues. During the scan, the subject lies on a table in a narrow cylinder containing a magnetic field, wearing ear plugs to muffle loud noises that occur with electrical switching of the magnetic fields. He or she can speak with a staff member via an intercom system at all times during the procedure. The neurological and muscle strength and endurance evaluations are repeated on study days 9 and 29.
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