






























© Getty Images
0 / 31 Fotos
What is stiff-person syndrome?
- Stiff-person syndrome (SPS) is a autoimmune and neurological disorder that causes rigidity in the torso and limbs.
© Shutterstock
1 / 31 Fotos
Muscle spasms
- Besides stiffening across the body, symptoms of this rare condition also include episodes of severe muscle spasms.
© Shutterstock
2 / 31 Fotos
Chronic pain
- People with the syndrome can expect chronic pain, impaired mobility, and lumbar hyperlordosis (excessive spine curvature in the lower back).
© Shutterstock
3 / 31 Fotos
Triggers
- Symptoms can occur at random but are often triggered by environmental stimuli (like loud noises), touch, or emotional stress.
© Shutterstock
4 / 31 Fotos
Severe reaction
- Muscle spasms can be so severe they can dislocate joints and even break bones, in some cases causing the person to fall down. The muscles gradually relax after the stimulus is gone.
© Shutterstock
5 / 31 Fotos
Difficulty walking
- Over time, these symptoms can lead to difficulty in walking and eventually greater immobility.
© Shutterstock
6 / 31 Fotos
Emotional toll
- As the disease progresses, so does the emotional toll exerted on the patient. Symptoms of depression and anxiety are commonplace.
© Shutterstock
7 / 31 Fotos
Heightened anxiety levels
- Levels of nervousness and apprehension are heightened by the fact that sufferers of SPS have lower levels of the neurotransmitter gamma-aminobutyric acid (GABA), which regulates anxiety.
© Shutterstock
8 / 31 Fotos
One in a million
- SPS occurs in about one in a million people and is most commonly found in middle-aged people.
© Shutterstock
9 / 31 Fotos
Communication problems
- Stiff-person syndrome inhibits the normal pathways of communication between the brain and the muscles.
© Shutterstock
10 / 31 Fotos
SPS cause remains unknown
- The cause of SPS remains clouded in mystery. Research suggests that its onset may be caused by an autoimmune reaction.
© Shutterstock
11 / 31 Fotos
Autoimmune reaction?
- The immune system appears to attack a protein called glutamic acid decarboxylase (GAD), which helps make the aforementioned substance called GABA.
© Shutterstock
12 / 31 Fotos
Motor neuron irregularity
- GABA helps regulate motor neurons by decreasing their activity. But lower than normal levels of GABA can cause those neurons to fire continuously even when they’re not supposed to. This impairment probably causes the stiffness and spasms that characterize SPS.
© Shutterstock
13 / 31 Fotos
Related disorders
- SPS patients commonly have another autoimmune disorder, typically type 1 diabetes, vitiligo, or pernicious anemia.
© Shutterstock
14 / 31 Fotos
The link with cancer
- Those stricken with SPS also tend to be cancer patients, specifically those suffering from breast cancer, lung cancer, kidney cancer, thyroid cancer, colon cancer, and lymphomas. The reason for this link is unknown.
© Shutterstock
15 / 31 Fotos
SPS background
- Stiff-person syndrome was first described in 1956 by neurologists Moersch and Woltman (the disease is sometimes referred to as Moersch-Woltman syndrome). However, it was only in 1989 that criteria for an SPS diagnosis were adopted.
© Getty Images
16 / 31 Fotos
Early signs and symptoms
- Generally, SPS begins slowly over several months or a few years. Affected individuals may initially experience aching discomfort, stiffness, or pain, especially in the lower back or legs.
© Shutterstock
17 / 31 Fotos
Worsening condition
- As the condition worsens, the shoulders, neck, and hips may also be affected.
© Shutterstock
18 / 31 Fotos
Slow, stiff gait
- Progression of SPS is characterized by stiffness of the leg muscles and a slow, rigid manner of walking.
© Shutterstock
19 / 31 Fotos
Slouched posture
- As stiffness increases, affected individuals may develop a hunched or slouched posture due to outward curving of the upper spine, a condition known as kyphosis. SPS can also cause hyperlordosis, where there is an excessive spine curvature in the lower back.
© Shutterstock
20 / 31 Fotos
Onset of muscle spasm
- Inevitably, sufferers of SPS will develop muscle spasms. The legs are often involved. But spasms can also involve the muscles of the chest and respiratory system, potentially requiring emergency medical treatment with ventilatory support.
© Shutterstock
21 / 31 Fotos
Wheelchair use
- The severity of advanced SPS can impact an individual's ability to perform daily activities and routines. Some patients may end up requiring the use of a wheelchair.
© Shutterstock
22 / 31 Fotos
Diagnosis
- Diagnosis of SPS can be tricky. Symptoms can mimic those of Parkinson’s disease, multiple sclerosis, fibromyalgia, or anxiety, and it often takes time to identify the exact cause.
© Shutterstock
23 / 31 Fotos
Treatment and prognosis
- There is no cure for stiff-person syndrome. A diagnosis of SPS is made based upon identification of characteristic symptoms, a detailed patient history, and a thorough clinical evaluation.
© Shutterstock
24 / 31 Fotos
Tackling the disorder
- Treatment for the condition typically involves relieving symptoms with medications such as sedatives, muscle relaxants, and steroids.
© Shutterstock
25 / 31 Fotos
Immunotherapy
- In some cases, immunotherapy may be prescribed, procedures such as intravenous immunoglobulin and plasmapheresis.
© Shutterstock
26 / 31 Fotos
Physical therapy
- When treating SPS, emphasis is also placed on regular activity. Physical, occupational, and aqua therapy are vital for patients with SPS.
© Shutterstock
27 / 31 Fotos
A difficult condition to treat
- By its nature, SPS is difficult to treat, more so than other autoimmune disorders like rheumatoid arthritis or Crohn's disease.
© Shutterstock
28 / 31 Fotos
Prognosis
- Symptoms of SPS and their severity levels vary. It's difficult therefore to predict whether a patient's condition will get worse over time.
© Shutterstock
29 / 31 Fotos
Outcome
- Early recognition and neurological treatment of SPS can limit its progression. But even with rapid intervention, quality of life generally declines as stiffness precludes many activities. Ultimately, about 65% of SPS patients are unable to function independently. About 10% of those will require intensive care at some point. Sources: (National Organization for Rare Disorders) (NBC News) (The Stiff Person Syndrome Research Foundation) See also: Stars who suffer from rare diseases
© Shutterstock
30 / 31 Fotos
© Getty Images
0 / 31 Fotos
What is stiff-person syndrome?
- Stiff-person syndrome (SPS) is a autoimmune and neurological disorder that causes rigidity in the torso and limbs.
© Shutterstock
1 / 31 Fotos
Muscle spasms
- Besides stiffening across the body, symptoms of this rare condition also include episodes of severe muscle spasms.
© Shutterstock
2 / 31 Fotos
Chronic pain
- People with the syndrome can expect chronic pain, impaired mobility, and lumbar hyperlordosis (excessive spine curvature in the lower back).
© Shutterstock
3 / 31 Fotos
Triggers
- Symptoms can occur at random but are often triggered by environmental stimuli (like loud noises), touch, or emotional stress.
© Shutterstock
4 / 31 Fotos
Severe reaction
- Muscle spasms can be so severe they can dislocate joints and even break bones, in some cases causing the person to fall down. The muscles gradually relax after the stimulus is gone.
© Shutterstock
5 / 31 Fotos
Difficulty walking
- Over time, these symptoms can lead to difficulty in walking and eventually greater immobility.
© Shutterstock
6 / 31 Fotos
Emotional toll
- As the disease progresses, so does the emotional toll exerted on the patient. Symptoms of depression and anxiety are commonplace.
© Shutterstock
7 / 31 Fotos
Heightened anxiety levels
- Levels of nervousness and apprehension are heightened by the fact that sufferers of SPS have lower levels of the neurotransmitter gamma-aminobutyric acid (GABA), which regulates anxiety.
© Shutterstock
8 / 31 Fotos
One in a million
- SPS occurs in about one in a million people and is most commonly found in middle-aged people.
© Shutterstock
9 / 31 Fotos
Communication problems
- Stiff-person syndrome inhibits the normal pathways of communication between the brain and the muscles.
© Shutterstock
10 / 31 Fotos
SPS cause remains unknown
- The cause of SPS remains clouded in mystery. Research suggests that its onset may be caused by an autoimmune reaction.
© Shutterstock
11 / 31 Fotos
Autoimmune reaction?
- The immune system appears to attack a protein called glutamic acid decarboxylase (GAD), which helps make the aforementioned substance called GABA.
© Shutterstock
12 / 31 Fotos
Motor neuron irregularity
- GABA helps regulate motor neurons by decreasing their activity. But lower than normal levels of GABA can cause those neurons to fire continuously even when they’re not supposed to. This impairment probably causes the stiffness and spasms that characterize SPS.
© Shutterstock
13 / 31 Fotos
Related disorders
- SPS patients commonly have another autoimmune disorder, typically type 1 diabetes, vitiligo, or pernicious anemia.
© Shutterstock
14 / 31 Fotos
The link with cancer
- Those stricken with SPS also tend to be cancer patients, specifically those suffering from breast cancer, lung cancer, kidney cancer, thyroid cancer, colon cancer, and lymphomas. The reason for this link is unknown.
© Shutterstock
15 / 31 Fotos
SPS background
- Stiff-person syndrome was first described in 1956 by neurologists Moersch and Woltman (the disease is sometimes referred to as Moersch-Woltman syndrome). However, it was only in 1989 that criteria for an SPS diagnosis were adopted.
© Getty Images
16 / 31 Fotos
Early signs and symptoms
- Generally, SPS begins slowly over several months or a few years. Affected individuals may initially experience aching discomfort, stiffness, or pain, especially in the lower back or legs.
© Shutterstock
17 / 31 Fotos
Worsening condition
- As the condition worsens, the shoulders, neck, and hips may also be affected.
© Shutterstock
18 / 31 Fotos
Slow, stiff gait
- Progression of SPS is characterized by stiffness of the leg muscles and a slow, rigid manner of walking.
© Shutterstock
19 / 31 Fotos
Slouched posture
- As stiffness increases, affected individuals may develop a hunched or slouched posture due to outward curving of the upper spine, a condition known as kyphosis. SPS can also cause hyperlordosis, where there is an excessive spine curvature in the lower back.
© Shutterstock
20 / 31 Fotos
Onset of muscle spasm
- Inevitably, sufferers of SPS will develop muscle spasms. The legs are often involved. But spasms can also involve the muscles of the chest and respiratory system, potentially requiring emergency medical treatment with ventilatory support.
© Shutterstock
21 / 31 Fotos
Wheelchair use
- The severity of advanced SPS can impact an individual's ability to perform daily activities and routines. Some patients may end up requiring the use of a wheelchair.
© Shutterstock
22 / 31 Fotos
Diagnosis
- Diagnosis of SPS can be tricky. Symptoms can mimic those of Parkinson’s disease, multiple sclerosis, fibromyalgia, or anxiety, and it often takes time to identify the exact cause.
© Shutterstock
23 / 31 Fotos
Treatment and prognosis
- There is no cure for stiff-person syndrome. A diagnosis of SPS is made based upon identification of characteristic symptoms, a detailed patient history, and a thorough clinical evaluation.
© Shutterstock
24 / 31 Fotos
Tackling the disorder
- Treatment for the condition typically involves relieving symptoms with medications such as sedatives, muscle relaxants, and steroids.
© Shutterstock
25 / 31 Fotos
Immunotherapy
- In some cases, immunotherapy may be prescribed, procedures such as intravenous immunoglobulin and plasmapheresis.
© Shutterstock
26 / 31 Fotos
Physical therapy
- When treating SPS, emphasis is also placed on regular activity. Physical, occupational, and aqua therapy are vital for patients with SPS.
© Shutterstock
27 / 31 Fotos
A difficult condition to treat
- By its nature, SPS is difficult to treat, more so than other autoimmune disorders like rheumatoid arthritis or Crohn's disease.
© Shutterstock
28 / 31 Fotos
Prognosis
- Symptoms of SPS and their severity levels vary. It's difficult therefore to predict whether a patient's condition will get worse over time.
© Shutterstock
29 / 31 Fotos
Outcome
- Early recognition and neurological treatment of SPS can limit its progression. But even with rapid intervention, quality of life generally declines as stiffness precludes many activities. Ultimately, about 65% of SPS patients are unable to function independently. About 10% of those will require intensive care at some point. Sources: (National Organization for Rare Disorders) (NBC News) (The Stiff Person Syndrome Research Foundation) See also: Stars who suffer from rare diseases
© Shutterstock
30 / 31 Fotos
What exactly is stiff-person syndrome, the disease that's stricken Céline Dion?
Unraveling the mystery behind this rare acquired neurological disorder
© Getty Images
The revelation that Canadian singer Céline Dion has been diagnosed with stiff-person syndrome (SPS) has placed this rare neurological disorder under the public spotlight. Little is known about the disease, which affects just one or two out of every million people. Its symptoms are painful and debilitating, and while treatment is available to lessen the impact SPS has on the mind and body, there is currently no cure for this condition that sees stiffening in the torso and limbs, along with episodes of severe muscle spasms.
But what causes stiff-person syndrome, and how is it treated? Click through for a diagnosis.
RECOMMENDED FOR YOU




































MOST READ
- Last Hour
- Last Day
- Last Week
-
1
CELEBRITY Relationships
-
2
FOOD Fruit
-
3
-
4
TRAVEL Italy
-
5
LIFESTYLE History
-
6
HEALTH Chronic traumatic encephalopathy
-
7
CELEBRITY Assaults
-
8
HEALTH Winter blues
-
9
MUSIC Curiosities
-
10
MUSIC Drums