Stiff Person Syndrome Definition, Reason, Symptoms, Treatment & Prevention
Definition
Stiff person syndrome is a rare neurological disease characterized by symptoms of progressive muscle rigidity and recurrent episodes of painful muscle spasms.
At first, muscle stiffness may come and go. However, in the end, the rigidity lasted constantly.
Over time, leg muscles become stiff, which is followed by stiffness in other muscles, including the arms and even the face.
This syndrome can gradually cause changes in body posture. In fact, in severe cases, stiff-person syndrome limits the patient’s ability to walk and move around.
Treatment is aimed at managing symptoms and increasing mobility and comfort for sufferers.
Stiff-Person Syndrome
| Medical specialist | Neurologist |
| Symptoms | Stiff muscles in the trunk and legs, sudden attacks of muscle spasms, difficulty walking, deformity of the back |
| risk factor | Women, people with autoimmune diseases such as type 1 diabetes, thyroid, vitiligo, anemia perniciosa |
| diagnosis | The complete interview, physical examination, complete blood count and glutamic acid decarboxylase (GAD) examination, Electromyography (EMG) |
| Treatment | Drug therapy |
| Drug | Diazepam, gabapentin, baclofen, immunoglobulin, kortikosteroid |
| Complications | Anxiety disorders, depression, frequent falls |
| When to see a doctor? | If there are muscle spasms and stiffness throughout the body |
Stiff Person Syndrome Symptoms
The main symptom of stiff-person syndrome is stiff muscles throughout the body. The severity varies from person to person.
This condition can develop and get worse over several months to years.
In addition, there are other signs of stiff-person syndrome, namely:
1. Back Pain
Symptoms of muscle stiffness in certain areas of the body, especially the back, shoulders and neck. This condition makes sufferers often feel pain in the back area.
2. Muscle Spasms
Muscle spasms occur spontaneously without realizing it and are easily triggered by sound, touch, cold temperatures, stress or emotional stress.
3. Agoraphobia
Sufferers of stiff-person syndrome often experience anxiety disorders, especially when in open spaces.
4. Myoclonus
Sufferers of stiff-person syndrome often experience sudden and hard-to-control muscle contractions, known as myoclonus.
5. Deformity of the Spine
As the disease progresses, the posture of a person with stiff-person syndrome can develop into an abnormal form, such as slouching.
Stiff Person Syndrome Reason
The cause of Stiff-person syndrome (SPS) is not known. However, some medical literature says that this disease is related to autoimmune disorders.
Most of the SPS patients studied had antibodies to glutamic acid decarboxylase or GAD.
GAD is a protein in inhibitory nerve cells that is involved in the creation of the main inhibitory neurotransmitter called gamma-aminobutyric acid (GABA). GABA itself helps control muscle movement.
SPS symptoms can develop when the body’s immune system mistakenly attacks certain nerve cells that produce GAD, depriving the body of GABA.
Risk Factors
Women with stiff-person syndrome are known to be twice as likely as men.
In addition, people with autoimmune diseases, such as type-1 diabetes, thyroiditis (inflammatory thyroid disease), vitiligo, and pernicious anemia, are also more at risk of developing stiff-person syndrome.
Diagnosis
The diagnosis of stiff-person syndrome is established based on a complete medical interview, physical examination, and complete blood count.
The doctor will check the typical symptoms, medical history of the patient, and a thorough clinical examination.
Investigations are carried out to support the diagnosis and rule out other conditions, including:
- Blood screening test to detect the presence of antibodies to GAD-65
- Electromyography (EMG), which is a test that records the electrical activity of skeletal muscles at rest and during muscle contraction.
Stiff Person Syndrome Treatment
Treatment for stiff-person syndrome is based on the patient’s symptoms.
A neurologist may provide drug therapy, such as oral diazepam (anti-anxiety and muscle relaxant); or medication to relieve muscle spasms, such as baclofen and gabapentin.
A study funded by the National Institute of Neurological Disorders and Stroke (NINDS) showed that intravenous immunoglobulin (IVIg) treatment was effective in reducing stiffness and sensitivity of patients to stress, noise, and touch, as well as helping balance in SPS patients.
IVIg itself has immunoglobulins (antibodies naturally produced by the immune system) that come from healthy donors.
Prevention
Because the cause is not known for certain, there is no way to prevent stiff-person syndrome.
Complications
Stiff-person syndrome will cause limited movement and muscle spasms. These problems can cause complications, such as:
- Anxiety and depression
- Sprained or broken bone due to severe muscle spasms
- Falls often
- Excessive sweating (hyperhidrosis)
When to See a Doctor?
Check with your doctor if you experience frequent and even severe muscle spasms and stiffness that interfere with your daily activities.