Stiff person syndrome is a rare, chronic neurological disorder.  The cause of this condition is unknown.  People with stiff person syndrome have antibodies that work against glutamic acid decarboxylase. 

Stiff person syndrome is a progressive disorder.  It is more common in females than in males.  It can happen to anyone.  It usually starts between the ages of 40-50 years old.  In extremely rare cases, stiff person syndrome can show up in children. 

Stiff person syndrome is associated with other autoimmune conditions like type 1 diabetes, thyroiditis, vitiligo, and pernicious anemia. 

 

Symptoms

The first symptom or sign of stiff person syndrome is muscle stiffness and painful spasms that come and go.  The symptoms may start as cramps or stiffness and then worsen from there.  In some cases other symptoms are present before the cramps, and muscle spasm. 

Painful muscle contractions and spasms most commonly occur in the legs, and back.  They can also occur in the abdomen, trunk, arms, neck, and face.  Spasms can be brought on from being startled, moving suddenly, cold temperatures, emotional stress, exercise, or touch. 

Other symptoms that you may also experience are difficulty walking, stiff or rigid posture, unsteadiness, falling due to spasms, shortness of breath, chronic pain, exaggerated curve in the lower back, anxiety, or agoraphobia.  Eye movement can also be a symptom such as double vision, speech issues, or lack of coordination.  

 

Types

There are different subtypes of stiff person syndrome. 

Classic stiff person syndrome is the most common.  It causes stiffness and spasms in the back and legs.  It can also cause a stiff gait, and pain constantly. 

Partial stiff person syndrome involves specific parts of your body being affected along with more prominent incoordination. 

Stiff person syndrome plus, is when you have common stiff person syndrome symptoms with additional symptoms signifying brainstem or cerebellar dysfunction. 

Progressive encephalomyelitis with rigidity and myoclonus is a severe and rare type of stiff person syndrome.  This type causes decreased consciousness, eye movement, ataxia, and autonomic dysfunction.  

 

 

Diagnosis

Diagnosing can be difficult.  Symptoms can be similar to other conditions.  Your doctor will want to do a physical exam as well as a neurological exam.  After that they may want to run some tests. 

Your doctor may suggest an antibody test.  This test will check for antibodies to glutamic acid decarboxylase.  Glutamic acid decarboxylase plays a role in making the neurotransmitter gamma-aminobutyric acid which helps control muscle movement.  The antibodies test can also help rule out other conditions. 

Another test that your doctor may suggest is an Electromyography.  This tests the electrical activity in your muscles and can help rule out other causes as well. 

A spinal tap, or lumbar puncture, is when a needle is used to draw out fluid from the spinal canal.  This can also help check for different antibodies.  A spinal tap can rule out other conditions too.  

 

Treatment

Treatment is based on symptoms.  The goal of treatment is to manage, improve mobility, comfort, and increase quality of life. 

There are two different avenues for treatment.  One is medications and therapies for symptom management.  Medications can help decrease stiffness, rigidity, and painful muscle spasms.  Benzodiazepines are usually the first line in treatment.  They help affect the signals of gamma-aminobutyric acid.  Another option to do this is using neuropathic pain medications.  Muscle relaxants can help treat muscle spasms.  They also help reduce stiffness.  Therapies that can be helpful are physical therapy, massage, hydrotherapy, heat therapy, and acupuncture. 

The second option for treatment is immunotherapy.  Intravenous immunoglobulin has shown effectiveness in decreasing symptoms caused by stiff person syndrome.  Intravenous immunoglobulin contains immunoglobulins or natural antibodies donated by thousands of people with healthy immune systems.  Other immunotherapies include plasma exchange, and oral immunosuppressants. 

There is no cure for stiff person syndrome.  Managing symptoms is the best treatment can do.  It is important to avoid narcotics, alcohol, tricyclic antidepressants, and serotonin norepinephrine reuptake  inhibitors when you have stiff person syndrome. 

Starting treatment quickly and soon after symptoms start can help.  Prognosis depends on how quickly the condition progresses, symptom severity, and how well treatment helps.  Managing triggers can be the most difficult.  Walking may become more difficult overtime as well as performing daily tasks.  

 

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