Celine Dion Reveals Stiff Person Syndrome Diagnosis

Celine Dion performing in Las Vegas

Celine Dion performing during her Las Vegas residency. Image courtesy of CNN.

Decorated Canadian singer-songwriter Celine Dion reveals she was recently diagnosed with a rare neurological autoimmune disorder called Stiff Person Syndrome. The diagnosis has lead her to cancel her summer 2023 shows, as well as re-schedule others to 2024.

According to Yale Medicine, Stiff Person Syndrome is believed to be an autoimmune reaction that occurs when the body’s own immune system attacks and destroys a vital protein called Glutamic Acid Decarboxylase (GAD). This protein is responsible for making a substance called gamma-aminobutyric acid (GABA), which helps to regulate motor neuron cells, and ensure they’re not over-active.

People with low levels of GABA have neurons that continuously fire, even when they’re not supposed to. This results in debilitating symptoms like violent muscle spasms, muscle stiffening in the torso and limbs, and difficulty with walking and movement. GABA also helps to regulate symptoms of depression and anxiety, so those with Stiff Person Syndrome are at a higher risk for developing these mental health conditions.

The 54-year-old Grammy award-winning artist has said that the condition has had a profound impact on her life, commenting: “Unfortunately, these spasms affect every aspect of my daily life, sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I’m used to.”

Getting diagnosed with Stiff Person Syndrome can be a challenge, since the symptoms can mimic many other neurological health conditions, like multiple sclerosis, Parkinson’s disease, fibromyalgia, and more. Patients typically undergo a thorough examination, such as blood tests and spinal fluid tests, to find elevated levels of anti-GAD antibodies, in order to get diagnosed.

Being diagnosed was not a straightforward process for Dion herself. “While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms that I’ve been having,” she said.

Although anyone can develop Stiff Person Syndrome, the National Organization for Rare Disorders reports that adults ages 30 to 60 are most commonly diagnosed with the condition. The condition is considered rare, with only one in a million individuals being diagnosed with SPS among the general population.

There is no cure for Stiff Person Syndrome, but treatments like steroids to control inflammation, plus the use of sedatives and muscle relaxants to control muscle spasms, can help. Sometimes Stiff Person Syndrome patients are also prescribed immunotherapies to help calm an over-active immune system that’s destroying their GAD proteins.

In an emotional video on her Instagram, Dion said, “I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again. But I have to admit it’s been a struggle.”

To learn more about Stiff Person Syndrome, visit the SPS Research Foundation’s website.

Could Alzheimer’s Be an Autoimmune Disease?

Prominent neurologist awarded grant to research Alzheimer’s as an autoimmune disease

Alzheimer’s is the most common cause of dementia; according to the Alzheimer’s Association, Alzheimer’s disease accounts for up to 80% of dementia cases.

Although little is still known about this disease, which causes significant loss of memory and other cognitive abilities, the most well-accepted hypothesis is that Alzheimer’s is caused by the build up of a protein called beta amyloid. When too much beta amyloid is accumulated in the brain, toxic clumps of the protein, called plaques, can form. These plaques are believed to be the culprit for Alzheimer’s; as a result, recent clinical trials have aimed to find a way to target and reduce the amount of plaques in the brain.

However, a prominent neurologist and medical researcher from Toronto, Ontario, Canada has put forth a new hypothesis on the development of Alzheimer’s. Dr. Donald Weaver theorizes that beta amyloid is actually a normal part of the brain’s innate immune system, and is there to kill bacteria and serve as a messenger protein. When the body’s immune response is triggered by an infection, trauma, or exposure to noxious substances, brain cells are triggered to release beta amyloid.

The problem arises, however, when beta amyloid mistakes brain cells for bacteria, and begins to kill these cells instead. This leads to fragments being created in the brain, which go on to trigger the continued release of beta amyloid. The result is a self-perpetuating cycle of releasing beta amyloid and killing more brain cells, resulting in a chronic disease.

Dr. Weaver’s theory on Alzheimer’s as an an autoimmune disease has garnered the attention of the medical community. He has been awarded the silver Oskar Fischer Prize, a grant worth US$400,000 from the University of Texas at San Antonio, to pursue research related to his theory.

Dr. Weaver believes that by exploiting the body’s natural way of controlling the immune system, Alzheimer’s symptoms can be reduced, and the disease could even be prevented. He commented, “If we accept the fact that Alzheimer’s disease is an immune-based disease that has certain triggers, then I think that we need to go back and revisit the risk factors.” Examples of risk factors include air pollution, head trauma, and genetic susceptibility.

Ultimately, Dr. Weaver’s research represents hope for a new way of tackling Alzheimer’s disease. Even more exciting is that Dr. Weaver’s research may have applicability beyond Alzheimer’s to other neurological conditions as well, such as Parkinson’s, Multiple Sclerosis (MS), and Encephalitis.

Jenny Hsieh, director of the University of Texas at San Antonio’s Brain Consortium, believes it’s important to provide researchers the opportunity to pursue ideas that are outside the box. “We just need people to be able to work on different ideas…because the bottom line is all of the current approaches to Alzheimer’s disease [are] not working.”

To learn more about Dr. Weaver and his work, visit: www.weaverlab.ca

Study Finds Link Between Dairy Consumption and Multiple Sclerosis (MS)

Is there a link between cow's milk and autoimmune disease like multiple sclerosis (MS)?
Could there be a link between the consumption of dairy products and multiple sclerosis (MS)? A new study from the University of Bonn sheds light on that question. Image courtesy of Health Europa.

A new study from the University of Bonn in Germany has revealed a link between the consumption of dairy products and multiple sclerosis (MS), reports Science Daily.

The researcher who led the study, Stefanie Kürten, a professor or neuroanatomy at the University Hospital Bonn, is considered to be an expert on MS, an autoimmune disease that often has debilitating and disabling symptoms. Kürten says it was her patients themselves that prompted her theory that there could be a link between the consumption of dairy products and MS symptoms.

“We hear again and again from sufferers that they feel worse when they consume milk, cottage cheese, or yogurt,” Kürten explained. “[So] we injected mice with different proteins from cow’s milk. We wanted to find out if there was a protein that they were responding to with symptoms of disease,” she said.

The myelin sheath of healthy mice who do not have demyelinating disease, as shown under a microscope.
In healthy mice, the myelin sheath (black) fits snugly as a compact layer around nerve fibers. Image courtesy of Prof. Kürten/the University of Bonn.

Her team’s research had some interesting results: when they administered the cow’s milk protein casein to mice, together with an effect enhancer, the mice went on to develop neurological disorders. A microscopic look at the mice’s nerve fibers showed damage to the myelin sheath, which is the insulating layer that gets damaged by the body’s immune response in patients with MS.

Rittika Chunder, a postdoctoral fellow in Professor Kürten’s research team, explains: “We suspected that the reason [for the damage] was a misdirected immune response, similar to that seen in MS patients.” “The body’s defenses actually attack the casein, but in the process they also destroy proteins involved in the formation of myelin.”

The myelin sheath of mice injected with casein is looser, as shown under a microscope.
In mice injected with casein, the structure of the myelin sheath loosens, and sometimes is missing altogether. Image courtesy of Prof. Kürten/the University of Bonn.

So why would one’s body attack the casein, the protein found in milk, to begin with? The researchers theorize that presumably, the multiple sclerosis patients studied developed an allergy to casein at some point in their lives as a result of consuming milk. Then, the immune system mistook a protein called MAG, which is important for myelin production, with casein.

“We compared casein to different molecules that are important for myelin production,” Chunder explained. “In the process, we came across a protein called MAG. It looks markedly similar to casein in some respects – so much so that antibodies to casein were also active against MAG in the lab animals.”

So, if you have MS, should you avoid milk and other dairy products altogether? Not necessarily, say the researchers, as this only affects MS patients who are allergic to cow’s milk casein.

“We are currently developing a self-test with which affected individuals can check whether they carry corresponding antibodies,” said Kürten. “At least this subgroup should refrain from consuming milk, yogurt, or cottage cheese.”

Another multiple sclerosis study out of Harvard University has pointed to the Epstein-Barr virus being the trigger for the demyelinating autoimmune disease. And, the MS Society of Canada has published vitamin D recommendations, due to the link between vitamin D deficiency and MS, demonstrating that there isn’t necessarily one catch-all cause of MS.

Still, Kürten’s research has opened an interesting conversation for further studies related to the link between diet and autoimmune disease – and many leading physicians and scientists believe that there is, in fact, a link. Dr. Terry Wahls, a physician who has MS herself, published a book called The Wahls Protocol about how she used Paleo eating principals to put her MS symptoms into remission.

For all of our readers with multiple sclerosis: what do you think about the new research about the link between dairy and MS? Do you follow a certain diet to control your MS symptoms? Let us know in the comments below!