Stiff-Person Syndrome

In News

French-Canadian singer Celine Dion has opened up about being diagnosed with Stiff-Person Syndrome (SPS),

About Stiff person syndrome

• It is a rare autoimmune neurological condition that affects the central nervous system and can cause rigidity throughout the body and painful muscle spasms.
  •  It was first coined in the 1920s (as “stiff man syndrome”) after doctors described patients falling over like “a wooden man.”
• It frequently affects women with a median onset of 35 to 40 years of age.
• Causes: the cause behind SPS is not fully understood yet, “there have been reports that spasms occur at any random time and can be triggered by loud noises, touch, and emotional distress

• Symptoms: Symptoms may include stiff muscles in the trunk (torso), arms, and legs; and greater sensitivity to noise, touch, and emotional distress, which can set off muscle spasms.
• Treatment: Several symptoms improve with oral diazepam (an anti-anxiety and muscle relaxant drug) or with drugs that alleviate muscle spasms, such as baclofen or gabapentin.
  • Treatment involves the use of both symptomatic agents to enhance GABAergic influences and Immuno modulating treatment aimed at the autoimmune basis of the disease
  • Corticosteroids are rarely used as Immuno modulating agents in Stiff Person Syndrome because of a high incidence of concurrent diabetes mellitus,

Source:IE