Suffering from “Stiff Person Syndrome” (or Stiff man), singer Céline Dion has announced her return to the stage in France in 2026. Cause, symptoms, life expectancy: reminder of what this disease is.
After triumphing at the Opening Ceremony of the Paris 2024 Olympic Games, Celine Dion announces her return to the stage in Paris in 2026. However, the singer has suffered for several years from a little-known illness: stiff person syndrome (SPS) or Stiff-man syndrome. This disorder is characterized in particular by “(muscle) spasms (which) affect my everyday life, on several levels. I meet people difficulty walking And I can’t always use my vocal cords to sing as I wish” she explained in 2022 on her Instagram account. At that time, she had to cancel her concerts.
What is stiff man syndrome?
Stiff person or stiff man syndrome (SHR) is a very rare neurological diseasewhose prevalence is estimated at about 1 in 1 million people. Approximately 2/3 of patients affected would be womenwith a peak incidence around 45 years old. It mainly manifests itself as a stiffness of the trunk muscles with spinal deformity, and painful spasms. This syndrome was first described in 1956 by Moersch and Woltman, two American neurologists.
Specific symptoms
The clinical picture is very evocative. This neurological disease is characterized by:
- Of the spontaneous muscle spasms or reflexes, painful, which may cause falls and episodes of fracture when the spasms are very severe
- A progressive muscle stiffness which can lead to immobility of the trunk and hips
- Stiffness of the fingers and feet like violent cramps
- A stiff and atypical gait
- A agoraphobia, associated with carrying out certain tasks
- A tendency to jump exaggeratedly
- Of the ankylosing deformities, such as a lumbar posture fixed in hyperlordosis (accentuation of curvatures either at the cervical level or at the lumbar level)
What life expectancy?
Generally, symptoms stabilize with treatment by benzodiazepines. However, most patients still have a clear functional limitation. The vital prognosis is not a priori impacted. When stiff person syndrome is associated with progressive encephalomyelitis with rigidity and myoclonus (called PERM), the prognosis is somewhat more severe and the symptoms are more difficult to control.
What is the cause of this syndrome?
The cause of this pathology is still not certain. Nevertheless, it seems that we find the presence ofanti-glutamic acid decarboxylase antibody (GAD) in more than 70% of cases, which suggests that it is a autoimmune pathogenesis. These antibodies could block the synthesis of gamma amino butyric acid (GABA), an inhibitory neurotransmitter, thus leading to a reduction in the inhibition of spinal motor neurons (responsible for muscle contraction). According to a study published in the Letter of the Rheumatologist n°269, there is frequently an association with autoimmune diseases, such as insulin-dependent diabetes (in 30% of cases), dysthyroidism, vitiligo, Biermer’s anemia. In 5 to 10% of cases, we note the occurrence of epileptic seizures. “The link between these two pathologies is not very clear.“, we can read in the article published in the rheumatologist’s journal.
The diagnosis of this pathology is essentially based on clinical examination. It is then confirmed by the highlighting ofcirculating anti-GAD antibodies and characteristic electromyographic abnormalities visible during a electromyography (EMG). In order to exclude other – mechanical – pathologies such as herniated disc or the presence of a cyst in the spinal cord, it may be necessary to carry out a spinal cord scan. Also to exclude spinal cord diseases such as multiple sclerosis or tumors. A cerebrospinal fluid examination also helps eliminate an inflammatory process.
What is the treatment for stiff person syndrome?
To treat the symptoms of the disease, the doctor may prescribe benzodiazepines (especially the Diazepam®) which slow down brain activity and baclofen. Diazepam is generally admitted intravenously or orally at a dosage of 10 to 100 mg per day, a dosage of course to be adapted according to effectiveness and side effects. Immunomodulatory therapies (corticosteroids, intravenous immunoglobulins, plasmapheresis) can be proposed, but with variable results. Rehabilitation with the physiotherapist must often be undertaken in order to combat spasms.
