Stiff-person syndrome: Can it be treated?

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Stiff-person syndrome: Can it be treated?

Question

What is stiff-person syndrome? Can it be treated?

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Answer

There is no cure for stiff-person syndrome, formerly called stiff-man syndrome. But treatment may improve symptoms.

Stiff-person syndrome is a rare, disabling neurological disorder characterized by progressive, severe muscle stiffness or rigidity, mainly in the spine and legs. It also causes painful muscle spasms triggered by sensory stimuli, such as sudden loud noises or emotional distress. Stiff-person syndrome was first described in 1956 by doctors at Mayo Clinic.

The exact cause of stiff-person syndrome isn't clear. But it may be due to an autoimmune disorder.

In stiff-person syndrome, the immune system makes antibodies against the enzyme glutamic acid decarboxylase (GAD). GAD plays an important role in the production of gamma-aminobutyric acid (GABA), a neurotransmitter that inhibits certain activity in the spinal cord and brain. Rarely, stiff-person syndrome is associated with antibodies produced by certain cancers, such as breast cancer.

Treatment of stiff-person syndrome may include:

  • Muscle relaxants, such as diazepam and baclofen
  • Intravenous immunoglobulin (IVIg) with infusions of healthy antibodies

Last Updated: 06/29/2006
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