Celine Dion, who was fighting back tears today, revealed she has a neurological disorder that’s one in a million.
The condition, stiff person syndrome, causes muscles in the upper body and limbs to stiffen and become rigid. It also causes painful muscle spasms that can eventually lead to difficulty walking.
The autoimmune disease causes severe muscle spasms that can cause people to freeze to death and eventually lead to falls and difficulty walking.
The Canadian singer, 54, told fans in an emotional Instagram video that she had to cancel her upcoming European tour in February – but said she has a great team of doctors as well as her children to support them.
Ms Dion said: “While we are still learning about this rare condition, we now know that this was what caused all of the cramps I had. Unfortunately, these spasms affect every aspect of my daily life, sometimes causing difficulty in walking and not allowing me to use my vocal cords in the way I’m used to singing.’
But what exactly is stiff person syndrome? Here we describe what causes the disorder and how doctors treat it….
Celine Dion announced today that she has stiff person syndrome, which is one in a million
What is stiff person syndrome?
Stiff person syndrome is an extremely rare condition in which the muscles in the upper body and limbs alternately spasm and stiff.
It has been dubbed “human statue disease.” The spasms this causes can be severe enough to dislocate joints and break bones.
It is estimated to affect around 70 people in the UK and 330 in the US and is still little known. Around twice as many women as men are affected.
The disease worsens over time and can paralyze patients, requiring them to use a walker or wheelchair.
There are three types of the syndrome:
- Classic person-male syndrome: If you experience stiffness and cramps in the back and abdomen, and occasionally in the thighs and neck. It can cause your back to curve over time.
- Stiff Limb Syndrome: Spasms particularly affect the legs and feet, occasionally resulting in fixation. The hands can also be affected.
- Jerking stiff person syndrome: The rarest, most aggressive form, it includes symptoms of both others and also affects the head and eyes.
what causes it
Experts do not know exactly what is behind the disease.
But they believe it can be caused by an autoimmune reaction when the body attacks its own nerve cells that control muscle movement.
Around 40 percent of those affected also suffer from type 1 diabetes, another autoimmune disease. Type 1 diabetes is particularly associated with the classic personality syndrome.
Other autoimmune diseases such as vitiligo, which causes white skin patches, and pernicious anemia are also associated.
It’s also more common in people with breast, lung, kidney, thyroid or colon cancer, and lymphoma, but researchers don’t yet know why.
In stiff-person syndrome, the immune system attacks a protein that helps make gamma-aminobutyric acid (GABA), which regulates motor neurons — the nerves that control movement.
Low levels of GABA cause neurons to fire non-stop when they shouldn’t, leading to spasms and stiffness.
What are its symptoms?
The main symptoms of stiff person syndrome are spasms and stiffness of the upper body and limbs.
Convulsions can be triggered by loud noises, and the condition also causes increased sensitivity to noise.
Touch and emotional stress can also be felt more intensely as a result of the disease.
The spasms can be severe enough to cause people to fall or cause difficulty walking and other disabilities.
Stress and anxiety are also usually higher in sufferers, particularly because of the unpredictability of spasms.
The lack of GABA — which regulates anxiety — in their system also impacts mental health.
How is it diagnosed?
Because of its rarity and confusing symptoms, which are often confused with Parkinson’s disease or multiple sclerosis (MS), the syndrome can take a long time to diagnose.
But when doctors suspect stiff person syndrome, they can confirm it with two tests.
The first looks in the blood for antibodies against the already mentioned protein glutamic acid decarboxylase (GAD).
High levels of GAD antibodies suggest that stiff-person syndrome may be occurring, although levels are also elevated in people with type 1 diabetes.
The second test is an electromyogram (EMG), which assesses muscle and motor neuron health.
Doctors insert a needle directly into the affected muscles and record electrical activity within them.
Is there a cure?
no Unfortunately, doctors cannot reverse or cure the lifelong condition.
However, in the majority of patients, treatments can be used to control symptoms.
Medications like diazepam and baclofen — both of which control spasms — can help regulate episodes and reduce stiffness.
Some patients with more severe symptoms are also given therapies to manipulate their immune system to increase GABA levels.
In some cases, immunoglobin transfusions may be given to affect antibody levels in the blood.
Medications such as tranquilizers and steroids may also be prescribed.
In the meantime, patients are often prescribed physical and water therapy to improve the functioning of their muscles.
Source: | This article originally belongs to Dailymail.co.uk
https://www.soundhealthandlastingwealth.com/health-news/what-is-stiff-person-syndrome-celine-dion-condition-turns-patients-into-human-statues/ What is stiff person syndrome? Celine Dion’s condition turns patients into ‘human statues’