Celine Dion announces a new documentary on the rare disease that strikes her

Celine Dion fans will finally be able to find her again. Absent from stages and world tours since December 2022 due to a neurological illness, the Canadian singer announced on January 30 in an Instagram message the next release of I am: Celine Diona documentary about the stiff-man syndrome that has kept her out of the media for months.

Struck by stiff man syndrome

In December 2022, the singer announced that she was suffering from stiff man syndrome, a neurological disease that affects one in a million people and which notably causes muscle spasms and thus prevents her from using her voice. A sickness “which is not life-threatening, but which can be difficult to live with and which does not really have any treatment available to date” Dr Wilfrid Casseron, neurologist, told us in a previous article.

According to the singer’s own sister last December, despite several attempts, no treatment actually worked, and Celine Dion currently no longer has control of her muscles.

A documentary to better talk about the disease

It is therefore in the form of a documentary that Céline Dion today wishes to break the silence. A format in which she wishes to discuss her condition unvarnished and talk about her daily struggle.

“The last few years have been a great challenge for me, from discovering the illness to learning how to live with and manage it, without letting it define me.. (…) During my absence, I wanted to document this part of my life to raise awareness about this little-known disease and help people who share this diagnosis” she delivers in her Instagram message.

If no release date is confirmed today, I Am: Celine Dion, directed by Irène Taylor, will be visible on Amazon Prime, just like in 240 countries.

What is Stiff-man syndrome or stiff man syndrome?

According to orphanet, the portal for rare diseases and orphan drugs, stiff man syndrome is a rare neurological pathology that combines fluctuating rigidity of the trunk and limbs, painful muscle spasms, and a phobia associated with performing certain tasks. , a tendency to jump in an exaggerated manner and ankylosing deformities, such as a fixed lumbar posture in hyperlordosis (exaggeration of the curvature of the lower back). It affects 1 in a million people, most often around the age of 45. Symptoms evolve over months or years.

Progressive muscle stiffness leads to immobility of the trunk and hips and gait becomes stiff and peculiar. Spontaneous or reflex muscle spasms are added, which can cause severe falls. A specific fear of crossing open spaces (pseudoagoraphobia) can cause sudden stopping, sudden spasms and falls. This disease is often associated with insulin-dependent diabetes (30%), autoimmune thyroiditis (10%) or atrophic gastritis with pernicious anemia (5%) and some, breast, lung or colon tumors.

This disease is thought to be autoimmune because the presence of anti-glutamic acid decarboxylase (GAD) antibodies is found in more than 70% of cases. These antibodies could block the synthesis of gamma amino butyric acid (GABA), a neurotransmitter.

Currently few treatments available

The diagnosis is essentially based on clinical observation, confirmed by the demonstration of circulating anti-GAD antibodies and an EMG (electromyogram) which must detect characteristic activity. A CT scan of the spinal cord can help exclude mechanical causes such as a herniated disc or a cyst in the spinal cord. Examination of the cerebrospinal fluid by lumbar puncture is sometimes necessary.

In the case of stiff man syndrome, few treatments exist “Valium (diazepam) and baclofen will help with muscle stiffness. Some treatments exist based on intravenous immunoglobulin, or corticosteroids, but the results are not constant and do not work for everyone. Physiotherapy is also essential to improve the condition. However, if the illness is difficult to live with, it is not life-threatening.” Dr Wilfrid Casseron, neurologist, told us in July 2023.