What is going wrong with Celine Dion? and what is stiff person syndrome
Definition
Moersch-Woltman Syndrome, often known as Stiff Person Syndrome (SPS), is a rare
autoimmune neurological disorder. It is described by the muscles of the trunk stiffening first,
then the legs and other body muscles gradually becoming tight and stiff. The disease has an
insidious beginning and progressively worsens over time. Painful muscular spasms may also
result from it, triggered by sound, touch, and emotional stress. A changing posture may develop
over time as a result of stiff person syndrome. Serious cases could make it difficult to walk or
move.
Who is prone to stiff person syndrome?
According to estimates, there are 1 to 2 cases of SPS per million people in the general
population, with:
- Females are impacted twice as frequently as males.
- Individuals between the ages of 20 and 60, most commonly in their thirties and forties
Moreover, there is a greater probability of seeing stiff person syndrome in patients with specific
conditions, such as:
- Other autoimmune disorders, including Diabetes Mellitus Type 1 (DM-1), autoimmune
thyroid disease, pernicious anemia, and celiac disease. - Breast, colon, thyroid, and lung cancer
Although studies suggest that SPS is the result of an autoimmune reaction that went wrong
in the brain and spinal cord, the exact origin of SPS is still unknown.
Symptoms
SPS is a disorder that develops slowly over several months with an insidious start of symptoms:
- Muscular stiffness and rigidity in the trunk, particularly in the thoracolumbar area
- Having trouble bending and twisting
With the progression of the condition, the person experiences:
- Proximal upper and lower extremities becoming stiff
- Increased lumbar lordosis
- Joint deformities
- Abnormal posturing which results in a “statue-like” appearance
- Gait disturbances and multiple falls
- Depression
- Task-specific phobias
- Fear of open spaces
- Anticipatory anxiety due to triggered spasms
Management
Treatment aims to reduce symptoms and increase your comfort and mobility.
1. Physical therapy
Physical therapy may increase SPS symptoms during specific illness stages thus some
precautions should be followed before starting treatments. However, physical therapy is
important in the treatment of this condition because patients must learn how to stretch
correctly and commit to continuous joint mobility maintenance. Studies showed that the
following approaches helped improve pain and muscle spasms, as well as improvements in
gait and range of motion:
- Exercise (stretching and relaxation)
- Functional retraining (transfers, stairs, sit-to-stand, etc.)
- Myofascial deep tissue methods
- Ultrasound
- Passive range of motion
- Heat therapy, and perhaps hydrotherapy
2. Pharmacological treatment
Oral diazepam, an anti-anxiety and muscle relaxant, and medications that reduce muscular
spasms like baclofen and gabapentin help with a number of symptoms. In addition,
intravenous immunoglobulin treatment is effective in reducing stiffness, sensitivity to noise,
touch, and stress, and improving gait and balance for people with SPS.
