Stiff Person Syndrome Medication

Updated: Jun 14, 2018
  • Author: Nancy Theresa Rodgers-Neame, MD; Chief Editor: Nicholas Lorenzo, MD, CPE, MHCM, FAAPL  more...
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Medication Summary

The goals of pharmacotherapy are to reduce symptoms, reduce morbidity, and prevent complications.



Class Summary

Most frequently cited as useful in the treatment of stiff person syndrome. Activate the GABA-A receptor to enhance central inhibitory circuits. Benzodiazepines include diazepam (Valium) or lorazepam (Ativan).

Diazepam (Valium)

Depresses all levels of CNS (eg, limbic and reticular formation), possibly by increasing activity of GABA.

Individualize dosage and increase cautiously to avoid adverse effects.

Lorazepam (Ativan)

Sedative hypnotic with short onset of effects and relatively long half-life.

By increasing the action of GABA, which is a major inhibitory neurotransmitter in the brain, may depress all levels of CNS, including limbic and reticular formation.


Immune modulators

Class Summary

These agents alter immune response to antigens. IVIG can be used.

Immune globulin intravenous (Bivigam, Gammagard, Gamunex-C, Octagam)

Neutralizes circulating antibodies through anti-idiotypic antibodies. Down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; may increase CSF IgG (10%).


Skeletal muscle relaxants

Class Summary

These agents increase activity of central inhibitory systems. Theoretically less sedating in relationship to GABA-A agonists such as benzodiazepines. 

Baclofen (Lioresal, Gablofen)

May induce hyperpolarization of afferent terminals and inhibit both monosynaptic and polysynaptic reflexes at the spinal level.