What is Stiff Person Syndrome?

Stiff Person Syndrome is an extremely rare neurological condition which affects the nervous system of the body. Stiff Person Syndrome affects mainly the spinal cord and the lower parts of the body. It is basically an autoimmune disorder that is characterized by extreme muscle rigidity. Any sort of emotional stress sparks off this condition. An individual with Stiff Person Syndrome will experience muscles spasms with severe pain stimulated normally by noise, touch, and most commonly emotional stress.

What is Stiff Person Syndrome

Individuals with Stiff Person Syndrome will have a hunched or a stiff posture due to muscle rigidity. Stiff Person Syndrome makes it difficult for the individual to move or walk fluidly as a normal person does. Stiff Person Syndrome can be triggered by small things like a horn of a car or being stuck in traffic resulting in severe muscle pain and spasm due to Stiff Person Syndrome. This condition is more common in females than in males and is associated with other medical conditions like diabetes, thyroid disorder, and anemia.

What Causes Stiff Person Syndrome?

Coming to the causes of Stiff Person Syndrome, researchers have still not been able to come up with a clear cut cause for this disorder but most researchers are of the belief that Stiff Person Syndrome occurs because of some abnormalities in the immune system of the body, especially in the brain and spinal cord. Since Stiff Person Syndrome is an extremely rare condition hence it is more often than not misdiagnosed as Parkinson's disease, multiple sclerosisfibromyalgia or anxiety.

What are the Symptoms of Stiff Person Syndrome?

Stiff Person Syndrome is mainly characterized by extreme muscle rigidity with painful muscle spasms. Other than that an individual with Stiff Person Syndrome will always have a stiff upright posture which may cause back pain. Individuals with Stiff Person Syndrome also find it difficult to sleep due to the continued stiffness. The symptoms of pain and spasms become more significant with triggers like extreme anger, stress, and fright. The stiffness in Stiff Person Syndrome in its initial stages may be limited to the extreme lower parts of the body but as the disease process advances this may spread all over the body. In severe forms of Stiff Person Syndrome, there may also be deformities which may be visible. With time as the disease process advances, an individual with Stiff Person Syndrome will become totally disabled and completely unable to move.

How is Stiff Person Syndrome Diagnosed?

The diagnosis of Stiff Person Syndrome is quite complex and difficult as the disease is quite rare and the symptoms mimic other disease conditions like Parkinson disease or fibromyalgia and this is the reason for this condition being misdiagnosed, although with specific blood tests a confirmed diagnosis of Stiff Person Syndrome can be made. Specifically, the level of Glutamic Acid Decarboxylase pinpoints towards a diagnosis of Stiff Person Syndrome. In an individual with Stiff Person Syndrome, the levels of GAD will be higher than normal. Other than this, EMG is also an important test which can diagnose Stiff Person Syndrome. In an individual with Stiff Person Syndrome, the low frequency and motor unit activity of the agonist and antagonist muscles will clearly confirm the diagnosis of Stiff Person Syndrome. Apart from this, the physical features and the symptoms experienced by the patient also gives a decisive hint towards a diagnosis of Stiff Person Syndrome.

How is Stiff Person Syndrome Treated?

At present, there is no definite cure for Stiff Person Syndrome, although with the advancement that medical science has made over the decades the progression of the disease can be slowed down and the symptoms can be significantly controlled. Studies suggest that use of IVIG or intravenous immunoglobulin has shown quite a lot of effectiveness in controlling the symptoms of Stiff Person Syndrome by reducing the sensitivity to noise, touch, and stress among many patients diagnosed with Stiff Person Syndrome. Anxiolytics, muscle relaxants, and anticonvulsants are also quite helpful in controlling the symptoms of Stiff Person Syndrome. Pain medications may also be given for pain relief to an individual with Stiff Person Syndrome.

What is the Prognosis of Stiff Person Syndrome?

Stiff Person Syndrome patient will always have some muscle pain and stiffness despite treatment as the treatments are aimed at controlling the symptoms and they are not a cure for the condition. The patient will always be at risk for frequent falls due to muscle rigidity and stiffness and difficulty moving the lower extremities. Also, the individual will be at risk for frequent injuries due to fall and other risk factors due to Stiff Person Syndrome.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: October 18, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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