Medication Summary

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

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.
Pain management
- Muscle pain is often a problem with patients and can cause worsening of the spasms and a cycle of spasms, pain, more spasms, and more pain.
- Nonsteroidal anti-inflammatory medications can be used for less severe cases.
- Long-term therapy with amitriptyline or similar tricyclics may be helpful. Time-release opiates may also be of benefit.
Novel medications
- Novel medications that may be of use because of their utility in other centrally mediated causes of spasticity include tizanidine (Zanaflex) and gabapentin (Neurontin).
- Hypothetically, botulinum toxin type A (BOTOX®) may also be helpful in selected cases. However, the number and size of the muscles involved would possibly limit its usefulness. Complications of BOTOX® therapy are more frequent when multiple muscles are injected with larger amounts of toxin.
- Although these medications have not been reported specifically in stiff person syndrome, they have been used with success in other cases of spasticity.

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)

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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)

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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.

Class Summary

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

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

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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%).

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)

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

Questions

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Author

Nancy Theresa Rodgers-Neame, MD Assistant Professor, Department of Molecular Pharmacology and Physiology, University of South Florida College of Medicine; Director, Florida Comprehensive Epilepsy and Seizure Disorders Program

Nancy Theresa Rodgers-Neame, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Women's Association, Society for Neuroscience, Southern Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Glenn Lopate, MD Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University in St Louis School of Medicine; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and CEO/CMO, PHLT Consultants; Former Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for Physician Leadership

Disclosure: Nothing to disclose.

Additional Contributors

Paul E Barkhaus, MD, FAAN, FAANEM Professor of Neurology and Physical Medicine and Rehabilitation, Chief, Neuromuscular and Autonomic Disorders Program, Director, ALS Program, Department of Neurology, Medical College of Wisconsin

Paul E Barkhaus, MD, FAAN, FAANEM is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Neurological Association

Disclosure: Nothing to disclose.

Sections Stiff Person Syndrome
Overview
Presentation
- History
- Physical
- Causes
- Complications
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DDx
Workup
Treatment
Medication
Questions & Answers
References

Stiff Person Syndrome Medication

Medication Summary

Benzodiazepines