INFORMATION:
STIFF PERSON SYNDROME, GAD-65, IVIg |
| Definition
of Stiff Person Syndrome Prepared by: Office of Communications and Public Liaison National Institute of Neurological Disorders and Stroke National Institutes of Health Bethesda, MD 20892 |
| More
than just a definition
of Stiff Person Syndrome Nancy Theresa Rodgers-Neame, MD, Assistant Professor, Department of Molecular Pharmacology and Physiology, University of South Florida; Director, Florida Comprehensive Epilepsy and Seizure Disorders Program From WebMD |
| GAD
Antibodies Commentary 2008 Excerpt: SPS does respond to immunotherapies such as intravenous Immunoglobulin (Dalakas et al., 2001) and other drugs (see Dalakas, 2008) |
| Intravenous
Immunoglobulin (IVIg) in Autoimmune Neuromuscular Diseases Excerpt: Among the new immunotherapies, intravenous Immunoglobulin (IVIg) has emerged as a major force, providing safe and effective long-term therapy and relieving previously untreatable conditions. |
| The
Stiff-Person Syndrome: An Autoimmune Disorder Affecting
Neurotransmission Excerpt: "Drugs that enhance GABA neurotransmission, such as diazepam, vigabatrin, and Baclofen, provide mild to modest relief of clinical symptoms. Immunomodulatory agents, such as steroids, plasmapheresis, and intravenous Immunoglobulin, seem to offer substantial improvement." |
CONCLUSIONS: SPS is: 1) frequently misdiagnosed due to multifaceted presentations and asymmetric signs, 2) disabling if untreated, and 3) associated with other autoimmune conditions. |
CONCLUSION: Based on a controlled study, IVIg is a safe and effective therapy for SPS in patients unresponsive to other agents. |
CONCLUSIONS: Intravenous immune globulin is a well-tolerated and effective, albeit costly, therapy for patients with stiff-person syndrome and anti-GAD65 antibodies. |
| Treatment
Options for Stiff Person Syndrome Excerpt: At present, GABA-enhancing agents, such as benzodiazepines, valproate, vigabatrin, tiagabine, gabapentin, and Baclofen, provide symptomatic relief. Plasmapheresis, steroids, and periodic intravenous Immunoglobulin infusions provide additional and lasting benefit. |
| Stiff
Person Syndrome-associated Autoantibodies to Amphiphysin Mediate Reduced GABAergic Inhibition Excerpt: induces stiff person syndrome-like symptoms in rats, including stiffness and muscle spasms. |
A search of ClinicalTrials.gov found only the following three trials, all of which have been completed. There are no current trials in the United States for treatment or determining the cause of Stiff Person Syndrome. |
Board certified in neurology and psychiatry, Dr. Dalakas is a Fellow of the American Academy of Neurology, as well as a member of the American Neurological Association, the European Neurological Society and the World Muscle Society. He is also a Diplomate of American Board of Psychiatry and Neurology. |