Stiff-person syndrome is rare but can be contained: Doctors

HYDERABAD: After singer Celine Dion, who gave us the iconic number ‘My Heart Will Go On’, rescheduled her Spring 2023 shows to 2024, and cancelled eight of her summer 2023 shows, on Friday, the 54-year-old shared a video on her Instagram explaining the reason behind it all — stiff-person syndrome (SPS) — which immediately got everyone wondering what it was.

It is a rare neurological disorder that causes muscle stiffness and spasms, which can be very disabling and can hamper going about one’s daily activities. Often associated with autoimmune disorders like diabetes or lupus, SPS can sadly be difficult to diagnose and treat.

"This is because it can present symptoms similar to other neurological disorders. Hence, patients need to work closely with their healthcare provider to manage the symptoms and improve their quality of life. It can be a challenging condition to manage but with the right treatment regimen and support, individuals can lead fulfilling lives," said neurologist Avinash Kumar A., adding that it was important to raise awareness about this rare disorder.

SPS most frequently affects individuals in the 20-50 years age-group. The rarity of the four different variants is such that one in a million people are affected by it.

The para-neoplastic variant of the condition affects a very small percentage of patients, while the common variation, the stiff-limb syndrome, primarily affects one particular limb.

"Superimposed spasms and extreme sensitivity to touch and sound are both experienced by SPS patients. These spasms mainly affect the muscles in the proximal limb and the axial region. Spasms typically last minutes and can reoccur for hours, are unpredictable and are frequently triggered by fast movements, emotional distress, or unexpected sounds or touches," said Dr Shyam Jaiswal, a consultant neurologist.

In rare cases, facial muscles, hands, feet and the chest can be affected, resulting in unusual eye movements and vertigo. One may even experience clonus (contractions) and have brisk stretch reflexes.

Thankfully, SPS is be treatable with benzodiazepines, muscle relaxants, steroids, plasma exchange and other immunosuppressive agents, said Dr Jaiswal. He added that one can keep this illness at bay by shifting to a healthy lifestyle, regular exercising, walking, yoga, good food habits, keeping away from alcohol and smoking and having other comorbidities under control.