Stiff person syndrome (SPS) is a rare neurological disorder that affects women more than men. Symptoms include muscle stiffness, abnormal postures, and sensitivity to stimuli. SPS can be misdiagnosed and is often treated with anticonvulsant medications, physical and behavior therapy, and immunosuppressive therapy. There is no known cure.
Stiff person syndrome (SPS) is a neurological disorder. It appears to be similar to an autoimmune disease. This rare condition appears to affect more women than men, but does not heavily affect any particular racial or ethnic group. Sufferers usually experience a progressive worsening of symptoms.
A patient with stiff person syndrome typically begins to experience symptoms around the age of forty-five. Muscle stiffness and stiffness of the limbs and torso are common in these patients. Patients may affect abnormal postures, such as being markedly hunched over.
Certain stimuli can trigger an episode of symptoms. Patients can be particularly sensitive to touch, noise and emotional distress. Any of these triggers can lead to muscle spasms, which can lead to falls.
The exact cause of stiff person syndrome is not known. SPS may be due to the brain or spinal cord misinterpreting an autoimmune response. It may also have a connection to some autoimmune diseases. For example, SPS patients may also suffer from diabetes, vitiligo, and thyroid disease. These patients may also be more prone to developing epilepsy.
Patients may initially be misdiagnosed. Stiff person syndrome can be misdiagnosed as multiple sclerosis, Parkinson’s disease, or fibromyalgia. Sometimes, it can also be attributed to phobias, anxiety or psychosomatic illnesses.
Diagnosis for stiff person syndrome will typically begin with a physical exam. The doctor will evaluate the patient’s medical history and symptoms. Once the symptoms are known, the doctor can then rule out other possible medical causes.
A blood test is also helpful for a diagnosis. These results can detect the number of glutamic acid decarboxylase (GAD) antibodies. An extremely high level of GAD antibodies may indicate a possible diagnosis of SPS.
If the blood test reveals a large number of GAD antibodies, the next step in the diagnosis is an electromyography (EMG). This test evaluates the body’s electrical activity in the muscles. Typically, with stiff person syndrome, the EMG will reveal low frequency activity.
There are several possible treatments available for stiff person syndrome, however there is no known cure yet. To control muscle spasms and stiffness, a doctor may prescribe anticonvulsant medications. Medications such as benzodiazepines can help control symptoms quickly. Patients taking these drugs should be careful to closely follow their drug regimen. If a patient stops the drug, she may experience crushing muscle stiffness, which can lead to fatal breathing problems.
Other possible treatments for stiff person syndrome include physical therapy and behavior therapy. The patient may also find relief with immunosuppressive therapy, such as a drug therapy called intravenous immunoglobulin (IVIg) treatment. Patients should be informed about all possible treatment options. Additionally, patients should wear a medical alert bracelet in case of an emergency.
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