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Stiff Person Syndrome Treatment & Management

  • Author: Nancy Theresa Rodgers-Neame, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
 
Updated: May 05, 2016
 

Medical Care

Initial medical treatment may involve either baclofen or a benzodiazepine.[3] Although no studies have been performed, tizanidine (Zanaflex) may be a less sedating alternative. Other medications that have been tried include antiepileptic medications, dantrolene, and barbiturates, but no clinical trials have been performed.

  • Intrathecal baclofen therapy
    • Some patients may be candidates for intrathecal baclofen therapy for long-term treatment. Because symptoms may be variable, an externally programmable pump may be the best option.
    • Evaluation for intrathecal baclofen therapy by an experienced evaluator, the neurosurgeon involved, and the neurologist caring for the patient should coordinate the procedure so that the goals of therapy are clear. Deaths have been reported in stiff person syndrome from baclofen pump failure; share this fact with the team and the patient. Baclofen pump therapy should not be considered the sole therapy for the disease.
  • Plasmapheresis (plasma exchange)
    • In some patients, plasmapheresis has been demonstrated to be of clinical utility in the treatment of stiff person syndrome.[33]
    • No real prescribed dosage exists for plasmapheresis. The time of plasmapheresis, amount of supplementary albumin, and other parameters are controlled on a patient-by-patient basis by the pathologist running the blood bank involved in the procedure. A 5-treatment series administered every other day is considered a standard regimen for autoimmune diseases, but longer and shorter regimens have been used.
    • The efficacy is then evaluated and further treatment is decided on a patient-by-patient basis, usually as a collaborative effort with the insurance company physicians because it is such an expensive procedure.
    • Possible adverse effects include hypotension, bleeding, arrhythmias, and infection.
  • Intravenous immunoglobulin
    • Intravenous immunoglobulin (IVIG) has also been used in the inpatient setting for the treatment of stiff person syndrome. The usual dose is 2 g/kg, administered over 2-5 days.
    • The length of the series is variable and dependent upon patient response. Treatment may extend past the inpatient period.[25] (Documentation of patient response is usually necessary for ongoing reimbursement by third party payers.)
    • Remember that IVIG is contraindicated in patients with IgA deficiency because of increased anaphylaxis in these patients.
  • Physical therapy and occupational therapy
    • Physical therapy and occupational therapy are critical to the recovery of the patient under treatment. Medical treatment may make the patient feel weak, a feeling that may respond well to therapy.
    • The patient may also have a great deal of problems with voluntary movement and fine motor skills.
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Consultations

Psychiatry may be consulted especially when symptoms of depression or anxiety are prominent. The psychiatrist should be made aware of the pathophysiology of stiff person syndrome and that the anxiety symptoms may be directly related to the presence of glutamic acid decarboxylase antibodies in the central nervous system. If possible, consult a psychiatrist that has shown interest in the disease.

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Activity

Exercise or physical therapy may be helpful in preserving range of motion and in relieving symptoms related to prolonged muscle tension. In addition, muscular biofeedback may be helpful, although careful studies of physical therapy treatments have not been done. Keep in mind that activity or exercise may exacerbate spasms.

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Contributor Information and Disclosures
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 Womens 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 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, MHA, CPE Founding Editor-in-Chief, eMedicine Neurology; Founder and CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc

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

Disclosure: Nothing to disclose.

Additional Contributors

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

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

Disclosure: Nothing to disclose.

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