Dr. Ahmed
Stiff Person Syndrome (SPS), also known as Stiffman Syndrome or Celine Dion Disease, is a rare neurological condition.
It was first called Stiffman Syndrome, but this name wasn’t accurate since the disease affects children and women too. So, it was renamed Stiff Person Syndrome.
The disease became well-known when the famous singer Celine Dion was diagnosed with it, leading some people to call it Celine Dion Disease.
Lately, Celine Dion has suffered an excruciating spasm on camera while recording a documentary “I am: Celin Dione”. Her team rapidly administer diazepam nasal spray to abort her spasm.
Understanding SPS (Stiff Person Syndrome):
Stiff Person Syndrome (SPS) is a rare disorder that affects the communication between your nerves and muscles. This disruption causes muscles, especially in the torso, to become stiff and spasm.
Over time, the muscles in the arms and thighs can also be affected. This can cause a hunched posture and frequent falls due to sudden muscle spasms.
People with SPS might avoid leaving their homes because the spasms can be triggered by things like loud noises, stress, touching, or even happening on their own.
The disease resembles tetanus, which is a life-threatening infection caused by Clostridium tetani. Like tetanus, people with stiff person syndrome have episodic spasms triggered by external stimuli.
However, unlike tetanus, stiff person syndrome is a chronic autoimmune condition with limited treatment options. It is progressive and has a variable prognosis.
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Causes of SPS (Stiff Person Syndrome):
Scientists are still studying the causes of SPS, but they have found that it involves harmful antibodies called anti-GAD. [ref].
These antibodies mistakenly target a protein called glutamic acid decarboxylase (GAD), which plays a vital role in helping nerve cells function in the brain stem.
A wrong attack may distort the fragile balance of signals between nerves and muscles, causing the stiffness and spasms typical of SPS.
Stiff Person Syndrome is closely associated with Diabetes Mellitus, Thyroiditis, Breast Cancer, Cerebellar ataxia, and Epilepsy.
Differentiation of SPS from other conditions:
It is important to differentiate between SPS and other abnormal conditions. Stiff person syndrome (SPS) can cause muscle strains, tension headaches, and anxiety.
People with SPS often experience worsening muscle spasms over time, which can be triggered by touch, noise, or even stress.
Some people with SPS might feel calm for a while, but the muscle spasms in the lower part of the body usually return and become more severe [ref].
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The proven symptoms of SPS are:
- Muscle spasms:
These can be sudden, severe, and excruciatingly painful, often affecting the back, abdomen, and legs.
- Rigidity:
The muscles, particularly in the core, become stiff and inflexible, making it difficult to move or maintain posture.
- Hyperexcitability:
Even minor stimuli like a touch, a loud noise, or emotional distress can trigger a spasm or worsen existing stiffness [ref].
Diagnosis and Treatment of Stiff Person Syndrome:
It can be lengthy and frustrating to diagnose SPS as it is challenging for doctors to identify the real symptoms, which can overlap with symptoms of other neurological disorders.
Patients might be wrongly diagnosed with Parkinson’s disease, multiple sclerosis, or even anxiety.
However, SPS may be diagnosed correctly with the help of a combination of tests and a detailed understanding of the patient’s medical history.
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Blood Tests, EMG, and Lumbar Puncture
Doctors can make accurate diagnoses with the help of several tools and get a clearer picture of the disease.
Blood tests can find anti-GAD antibodies, which are present in 70-80% of SPS patients [ref].
The electromyography (EMG) test shows the electrical activity of muscles and unusual patterns of SPS.
Sometimes, a lumbar puncture, which takes a sample of the fluid around the brain and spinal cord, is needed to rule out other conditions.
Is the a possible cure for SPS?
Regrettably, there is no cure for SPS; however, treatments can help manage symptoms and improve quality of life.
Proper medications like diazepam and baclofen play a vital role in reducing muscle spasms and stiffness [ref].
Intrathecal baclofen, plasma exchange, and intravenous immunoglobulins have been tried with variable success rates [Ref].
Physiotherapy can help maintain flexibility and improve the movement of other body parts.
In addition, some supportive therapies, like pain management strategies and emotional support groups, can also be beneficial.
It is important to acknowledge that this is not a complete solution, but with the help of stated methods, you may reduce the pain and improve the quality of life.
Due to the limitations of some therapies, consequential research may be needed in this field. Scientists are looking for new treatments, like therapies that target the autoimmune process. They hope to find a cure for SPS one day.
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Frequently asked questions:
What is the life expectancy of someone with SPS?
Many treatments are available, but none can cure it. With the help of these treatments, you may improve your quality of life and ease your SPS.
Gradually, SPS can restrict your ability to move or walk. The condition can last from 6 to 28 years, from when symptoms start until death.
Do you lose weight with stiff-person syndrome (SPS)?
The spam can affect the whole body or just one part, but mostly the lower portion of the body.
This spam can also be seen in the abdominal area; due to this, a person feels a fuller stomach, which leads to unintended weight loss.
What triggers stiff-person syndrome?
These spasms can be triggered by stimuli like loud noises, or emotional stress. A person can fall due to severe muscle spasms.
The muscles gradually relax after the stimulus is gone. This syndrome can lead to difficulty in walking and further disability.
