|INFORMATION: STIFF PERSON SYNDROME, GAD-65, IVIg|
of Stiff Person Syndrome
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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
| Stiff Person
Syndrome Study (UK)
Shahrzad Hadavi; Alastair J Noyce; R David Leslie; Gavin Giovannoni
Posted: 10/28/2011; Pract Neurol. 2011;11(5):272-282. © 2011 BMJ Publishing Group
The severity of symptoms and lack of public awareness of the condition create anxiety and uncertainty for people with the disease
This review aims to raise awareness of SPS and to improve the likelihood of its earlier diagnosis and treatment.
Antibodies Commentary 2008
SPS does respond to immunotherapies such as intravenous Immunoglobulin
(Dalakas et al., 2001) and other drugs (see Dalakas, 2008)
Immunoglobulin (IVIg) in Autoimmune Neuromuscular Diseases
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.
Stiff-Person Syndrome: An Autoimmune Disorder Affecting
"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.
Options for Stiff Person Syndrome
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.
Person Syndrome-associated Autoantibodies
to Amphiphysin Mediate Reduced GABAergic Inhibition
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.