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.
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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.
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Plasmapheresis (plasma exchange)
In some patients, plasmapheresis has been demonstrated to be of clinical utility in the treatment of stiff person syndrome. [34]
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.
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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. [26] (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.
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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.
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.
Transfer
Transfer to a tertiary or university medical center is often a difficult decision for a clinician. The clinician may feel that it reflects on him or her personally as a physician. However, in reality, even in some major metropolitan areas, hospitals have found offering the full range of facilities and expertise to be impossible. These resources extend beyond those of the individual clinician so that even though the treatment of the patient may be within the capability of the physician, it is not within the capability of the facility. Therefore, when patients approach the point at which they strain the capability of the facility, transfer should be initiated.
Questions to be answered are as follows:
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Does the treating facility have regular availability of plasmapheresis? Is intravenous immunoglobin therapy available on a regular basis at the treating facility?
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Does the treating facility have rehabilitation-grade physical therapy and occupational therapy?
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Does the treating facility have excellent inpatient psychiatric consultation for patients with chronic diseases? Do consulting psychiatrists have knowledge and interest in patients with chronic diseases, or are they mostly consulted for chemical restraint or behavior problems?
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Does the treating facility have an intensive care unit that is used for neurologic acute care, or does staff of the intensive care unit perform primarily cardiac, respiratory, and end-of-life care?
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Does the treating facility have an associated rehabilitation center capable of handling unusual diseases and physiatrists interested in unusual diseases?
Most patients with the early stages of stiff person syndrome do not require specialized care and do not require transfer by an experienced clinician. They can be treated successfully in an outpatient setting. However, attention to the above issues can alert a concerned physician to the need for transfer and help the physician justify the transfer to the patient, family, and insurance providers.
Activity
Exercise or physical therapy may be helpful in preserving range of motion and in relieving symptoms related to prolonged muscle tension in patients with stiff person syndrome. 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.
