Rare Disease Stiff-Person Syndrome: Dion’s Video Showcases Challenges of the Condition

Dr. Sheli explains that the syndrome of stiff-person is a rare disease, occurring in only one person out of a million, with most patients being women. This condition develops slowly and can lead to limitations in movement and severe pain, as seen in the video where the singer is going through a painful attack of muscle stiffness.

According to Dr. Sheli, the progression of the syndrome can be monitored through documentation, and it’s clear that the disease is not currently under control. In the video, Dion is seen receiving a spray into her nose, which is the recognized treatment for her condition. This spray, known as intranasal diazepam, contains benzodiazepines that work to calm down and ease the stiffness and contractions by increasing the effect of a neurotransmitter called GABA.

In some rare cases, the syndrome may appear as a secondary syndrome to an autoimmune disease, leading to patients receiving immunological treatments to stimulate the immune system. Due to her illness, Dion had to cancel her last tour in Europe, and she revealed in an interview with NBC that she had experienced muscle contractions so severe that she broke ribs.