Celine Dion- Stiff Person Syndrome

What is Stiff Person Syndrome?

Stiff Person Syndrome (SPS) is a rare neurological disorder that causes progressive muscle stiffness and spasms in the body. It is characterized by frequent, painful and sudden muscle contractions that can make everyday activities difficult or even impossible. 

This rare autoimmune neurological disorder initially affects the trunk and abdomen (the middle part of the body). Stiff Person Syndrome affects the muscles and nerves, resulting in muscle rigidity and spasms. The resulting pain and stiffness of the muscles can limit movement and cause posture to become rigid. 

SPS is characterized by episodes of muscle stiffness and/or spasms that can be painful and disabling. The muscles can become stiff enough to cause difficulty walking or even standing.

The cause of SPS is unknown, but it is believed to be an autoimmune disorder in which the body's own antibodies attack certain parts of the central nervous system. 

Symptoms

There are two main symptoms of stiff person syndrome:

• Muscle stiffness or rigidity. 

In most cases, the trunk muscles are the first to become stiff. The rigidity causes pain and an aching discomfort. 

• Painful muscle spasms.

It can involve the entire body or only a specific area and can last a few seconds, minutes or, occasionally, a few hours.

Spasms can be triggered by:

• Unexpected or loud noises.
• Physical touch or stimulation.
• Changes in temperature, including cold environments.
• Stressful events.

Causes

The exact cause of Stiff Person Syndrome is unknown, but research suggests that the condition is caused by an abnormal response of the body's immune system. Specifically, the body's own antibodies attack and damage neurons in the brain and spinal cord, which results in muscle stiffness and spasms. Environmental factors, such as infections or vaccinations, are believed to trigger this abnormal immune response.

In some cases, genetic factors might also play a role. Many people with SPS make antibodies against glutamic acid decarboxylase (GAD). GAD makes a neurotransmitter called gamma-aminobutyric acid (GABA), which helps control muscle movement. Although, it's important to note that the presence of GAD antibodies doesn't mean someone has SPS.

Diagnosis

• Antibody blood test: It checks for the presence of antibodies to GAD (or other relevant antibodies).
• Electromyography (EMG): It measures electrical activity in your muscles.
• Lumbar puncture (spinal tap): It uses a needle to draw fluid from the spinal canal to check for antibodies to GAD.

Treatment

• Medications and therapies for symptom management.
• Immunotherapy, or disease-modifying treatment.

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