Stiff-Person Syndrome

What is Stiff-Person Syndrome?

Stiff-person syndrome is a rare progressive neurological disorder characterized by constant painful contractions and spasms of voluntary muscles, particularly the muscles of the back and upper legs. It usually begins in young adults, first involving muscles of the trunk and progressing to affect muscles of the legs.

Stiff-person syndrome may begin as recurring episodes of stiffness and spasms, often precipitated by surprise or minor physical contact.

Autoimmune disorders such as diabetes, pernicious anemia, and thyroiditis may occur more frequently in patients with stiff-person syndrome.

What Causes Stiff-Person Syndrome?

The exact cause of stiff person syndrome is not known.

Symptoms of Stiff-Person Syndrome

Symptoms of stiff-person syndrome may occur gradually. The most common symptoms are:

• Constant painful contractions
• Muscle spasms - esp. back and upper legs but sometimes the arms and neck.
• Twisted muscles
• Stiff-legged gait
• Unsteady gait
• Hunched posture
• Swayback
• Difficulty making sudden movements

Symptoms may worsen when the affected individual is anxious or exposed to sudden motion or noise. Affected muscles may become twisted and contracted, resulting in bone fractures in the most severe cases. Sleep usually suppresses frequency of contractions.

Can Stiff-Person Syndrome be Treated?

Stiff-person syndrome can be treated. The drug diazepam, which relaxes the muscles, provides improvement in most cases. Baclofen, phenytoin, clonidine, or tizanidine may provide additional benefit. In some patients, immunomodulatory treatments such as intravenous immunoglobulin may be beneficial. Physical and rehabilitation therapy may also be needed.

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