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Neuromuscular and nervous system

Stiff-person syndrome (SPS)

Stiff-person syndrome is a rare disorder of the central nervous system that causes progressive muscle rigidity and painful spasms, usually starting in the trunk and the muscles close to it. Most people with the classic form carry very high levels of antibodies against GAD65, an enzyme the body needs to make GABA, a calming signal in the nervous system, and researchers believe impaired GABA signaling leaves muscles overexcitable. A smaller share of cases are paraneoplastic, linked to an underlying cancer and antibodies such as amphiphysin. It is associated with other autoimmune conditions and can occur alongside type 1 diabetes.

Stiff-person syndrome is considered rare, and research describes it as affecting roughly two-thirds women among autoimmune and paraneoplastic cases, with the paraneoplastic form most often tied to breast or lung cancer.

Common signs

Stiffness and rigidity in the back, abdomen, and limbs, sudden painful muscle spasms that can be set off by noise, touch, or stress, a tendency to startle, difficulty walking, and anxiety that often surrounds the unpredictable spasms.

This is a plain-language summary for orientation, not a diagnosis. Mechanisms are described as current understanding, not settled fact. Work with your own clinician for your care.

Questions people ask

Is stiff-person syndrome fatal?+
This page doesn't address life expectancy or mortality directly. It describes a rare disorder of the central nervous system causing progressive muscle rigidity and painful spasms, usually starting in the trunk and the muscles close to it, along with a tendency to startle and anxiety that often surrounds the unpredictable spasms. Questions about long-term outlook are best directed to a neurologist.
What triggers stiff-person syndrome spasms?+
Sudden painful muscle spasms can be set off by noise, touch, or stress. People with the condition often develop a tendency to startle, and the unpredictable nature of the spasms is frequently accompanied by anxiety.
Is stiff-person syndrome linked to diabetes?+
Yes. Stiff-person syndrome is associated with other autoimmune conditions and can occur alongside type 1 diabetes. Most people with the classic form carry very high levels of antibodies against GAD65, an enzyme the body needs to make GABA, a calming signal in the nervous system, and researchers believe impaired GABA signaling leaves muscles overexcitable.

In a doctor’s words

“Although people being treated for SPS continue to experience daily challenges, many are not having new ones, and their quality of life is better overall.”

Scott Newsome, MD, Director, Johns Hopkins Stiff Person Syndrome Center; Associate Professor of Neurology, Johns Hopkins Medicine
Read more at Johns Hopkins Medicine (Dome) ↗

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Daniela Hess, MSEd, is a co-founder of Great Energy and an Autoimmune Educator and Functional Wellness Consultant who lives and thrives with Hashimoto’s. She is not a licensed medical provider.

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