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
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.
GAD Antibodies Commentary 2008
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
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
"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."
GAD 65 and Stiff Person Syndrome
CONCLUSIONS: SPS is: 1) frequently misdiagnosed due to multifaceted presentations and asymmetric signs,
2) disabling if untreated, and 3) associated with other autoimmune conditions.
IVIg and GAD 65
CONCLUSION: Based on a controlled study, IVIg is a safe and 
effective therapy for SPS in patients unresponsive to other agents.
IVIg for Stiff Person Syndrome
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
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
Here we show that intrathecal application of purified anti-amphiphysin immunoglobulin G antibodies
induces stiff person syndrome-like symptoms in rats, including stiffness and muscle spasms. Search
A search of 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.
About Marinos C. Dalakas, MD
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.