Celine Dion’s announced that she was canceling her 2023 tour due to a diagnosis of stiff person syndrome (SPS).

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SPS is characterized by dramatically stiff muscles in the back, chest, abdomen, or arms and legs. These are not your garden variety back spasms or muscle aches that we all experience from time to time. SPS patients’ back muscles can feel as hard and inflexible as steel rods.

On top of this, muscle spasms in SPS can occur unexpectedly and can last for seconds or hours. They are often triggered by cold temperatures, unexpected loud noises, physical touch, or emotional stress. In the most severe cases, the spasms can dislocate joints, break bones, or cause a patient to fall.

Also in more severe cases, the muscle spasms and stiffness can spread to other muscle groups (especially the legs), and patients may develop difficulty walking. Some patients also develop anxiety or depression, or agoraphobia (fear of going outside), which is not uncommon for patients with rare, complex conditions.

Diagnosis and treatment of SPS

The exact role of the GAD enzyme and GAD antibodies in stiff person syndrome isn’t fully understood – approximately 20% of patients with the condition do not have GAD antibodies. Conversely, many people with GAD antibodies don’t have SPS (for example, millions of people with Type 1 diabetes in the U.S. likely have GAD antibodies).

There are specific tests and exams a health care provider can order to rule out other more common conditions, such as MS or fibromyalgia. Some of these include:

  • Blood test to check for antibodies to GAD.
  • Lumbar puncture (spinal tap) to check for antibodies to GAD in the cerebral spinal fluid or signs of inflammation.
  • Electromyography (EMG) to measure electrical activity in the muscles – SPS decreases muscle relaxation, not muscle strength.

Treatment for SPS focuses on managing symptoms, reducing pain, and improving mobility. Neurologists usually start by prescribing muscle relaxants, such as diazepam or lorazepam (which help activate the GABA receptor) or baclofen. In SPS, very high doses of muscle relaxants are sometimes required.

Some patients do well with muscle relaxants alone. For more severe cases, patients may need immunotherapy or immunosuppressant medications to reduce GAD antibodies. These could include intravenous immunoglobulin (IVIG), plasmapheresis, or rituximab. However, because SPS is rare, very few clinical trials have been done to prove the benefits of these treatments.

Non-medication treatments such as physical therapy, occupational therapy, aqua therapy, and acupuncture may provide relief from muscle pain. Cognitive behavioral therapy may help patients avoid or better handle the emotional triggers that can cause muscle spasms. It also can help them develop skills for living with a chronic condition like SPS.

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