Which medications are used in the treatment of stiff person syndrome?

Updated: Jun 14, 2018
  • Author: Nancy Theresa Rodgers-Neame, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

See the list below:

  • Baclofen

    • Baclofen is a specific GABA-B receptor agonist. The dramatic response of many patients with centrally mediated spasticity to this medication, including those with stiff person syndrome, implies the importance of this underresearched receptor in the CNS.

    • The oral dosage is 10-30 mg every 8 hours, while the intrathecal dosage is in the range of micrograms per day.

    • The major adverse effect with oral dosage is somnolence. The major adverse effect with intrathecal dosage is hypotonicity. Other, more severe complications related to baclofen pump failure have been reported.

  • Benzodiazepines

    • Diazepam and other benzodiazepines are also useful in the treatment of stiff man syndrome. In milder cases, small dosages can be used (2 mg q8h), but resistant severe cases can require very large doses (ie, 15-20 mg q8h; do not administer initially to benzodiazepine-naïve patients).

    • Benzodiazepines have the added benefit of relieving the inevitable anxiety associated with the disease.


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