Stiff Person Syndrome: Symptoms, Treatment, and Disease Progression

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Stiff person syndrome: symptoms and treatment

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Céline Dion announced that she suffers from ‘stiff person syndrome (SPS)’, which is a rare chronic disease that causes muscle stiffness and painful muscle cramps. The condition cannot be cured, but treatment can slow its progression. How exactly does the disease progress?

Also read: What causes nighttime muscle cramps and what can you do about it?

What is Stiff person syndrome?

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Stiff person syndrome is a neurological disorder and has the characteristics of an autoimmune disease. Muscle stiffness usually occurs in the trunk and abdomen. Over time, patients also develop stiffness and spasms in the legs and other muscles. Walking becomes difficult and people are more likely to fall and sustain injuries. There is also an increased sensitivity to stimuli. “This neurological condition can affect anyone at any age,” says Dr. Andrew McKeon, a neurologist and expert in autoimmune neurology and movement disorders at the Mayo Clinic. “It is more common in women, usually from the age of 40 or 50. More than 50 percent of patients have a concurrent non-neurological autoimmune disease, such as type 1 diabetes or autoimmune thyroid disease.” SPS is very rare: about 1 in 1 million people suffer from it.

The severity of symptoms varies from person to person. The condition cannot be cured, but with treatment the symptoms can be controlled and the progression of the syndrome can be slowed down.

Also read: Which pain should you never ignore?

What are the symptoms?

Characteristics of SPS include: Stiffness and rigidity in the trunk muscles Stiffness in the upper and lower limbs Difficulty turning and bending Painful muscle spasms An atypical stooped posture Stiff gait and difficulty walking Falling Exaggerated startle responses to stimuli such as sound or emotions People with SPS often also develop psychological complaints such as anxiety and depression

In the later stages, SPS can affect the facial muscles. Some people experience severe, prolonged spasms that require intravenous muscle relaxants. In rare cases, the respiratory muscles are also affected.

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What is the cause?

Stiff person syndrome is an autoimmune disease. This means that the immune system mistakenly attacks healthy cells and tissues in the body.

In 80 percent of patients with SPS, people produce a type of antibody called glutamic acid decarboxylase (anti-GAD). This attacks proteins in the neurons in the brain that affect muscle movement, affecting the spinal cord and brain function. Experts don’t know exactly why autoimmune diseases occur, although heredity likely plays a role.

Is there a treatment?

The condition cannot be cured, but a treatment plan can manage symptoms and improve mobility and comfort.

A doctor may prescribe sedative and muscle relaxant medications or neuropathic pain medication. Antidepressants and antiepileptic drugs are also sometimes prescribed to treat psychiatric symptoms and seizures that may accompany SPS. Someone with SPS may also benefit from disease-modifying therapies. This treatment aims to change the immune response and reduce or remove the harmful antibodies that attack healthy cells. SPS usually involves intravenous immunoglobulin (IVIG), which can improve symptoms for up to one year after a course of five sessions.

In addition, physiotherapy, occupational therapy and aqua therapy can provide relief.

Also read: What is occupational therapy?

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Last updated: September 2023

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2023-09-18 22:03:27
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