Explainer: The little-known stiff person syndrome

Published on : 07/12/2024, Time: 2:34 PM

Recent Celine Dion video prompts conversations about the extremely rare neurological disorder with no cure.

Celine Dion, the Canadian singer, has been in the news recently after a video of her experiencing a “10-minute seizure” went viral online. The singer has maintained a low public profile since being diagnosed with stiff person syndrome (SPS) in 2022. Given that it is an extremely rare disorder – the incidence is one in a million – public awareness about SPS is dismal.

First Check gets you all the facts about this rare acquired neurological disorder. SPS involves the gradual stiffening of the muscles of the body accompanied by recurring episodes of muscle spasms. It could lead to disability, hindering movement, causing falls, and serious injuries. Incidentally, SPS symptoms manifest twice as much in women as they do in men; typically, in individuals aged between 30 and 60.

While the exact cause of SPS is not clearly understood, researchers contend that it could be an autoimmune disorder, resulting from an autoimmune response gone awry in the brain and spinal cord. In some cases, the condition co-exists with other autoimmune disorders, such as type 1 diabetes, vitiligo, and pernicious anemia.

In some cases, SPS could result in a psychological condition or paranoia about leaving one’s home for the fear of experiencing a spasm. The triggers for the muscle spasm could be loud noise, physical contact, exposure to cold, and emotional distress.

It is easy to confuse the disorder with other health conditions such as Parkinson’s disease, fibromyalgia, and psychosomatic ailments. These can be ruled out based on a comprehensive medical history examination and additional blood tests. Patients with SPS exhibit higher levels of certain antibodies such as glutamic acid decarboxylase (GAD) antibodies. Further, electromyography (EMG) – to measure and assess the electrical activity in the muscles – can assist with the diagnosis of SPS.

Currently, there is no cure for SPS. However, symptoms can be managed with medications. Greater awareness about the condition can help improve the likelihood of early diagnosis and timely treatment.

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