Stiff Person Syndrome Follow-up

Updated: May 30, 2017
  • Author: Nancy Theresa Rodgers-Neame, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Follow-up

Further Outpatient Care

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  • Ongoing physical therapy and occupational therapy
    • Physical and occupational therapists can help with long-term muscle control and also serve as an adjunct to clinical observation for worsening signs and symptoms. They can also use passive muscle relaxation techniques that can help to relieve symptoms of long-term muscle spasm and to avoid loss of range of motion.
    • Be sure to encourage therapists to send reports or call staff about changes.
  • Medications
    • Symptomatic medications, particularly benzodiazepines, may need to be changed or rotated often to avoid receptor down-regulation.
    • Because the disease is rare, do not overlook novel medications that have not been reported but that have potential utility.
  • Intravenous immunoglobulin
    • Outpatient IVIG can be used in patients with responsive symptoms as ongoing therapy. [25]
    • Appropriate monitoring of vital signs should be available, and the procedure should be performed in an approved chemotherapy unit.
    • As in the inpatient setting, response should be documented carefully.
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Inpatient & Outpatient Medications

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  • 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.
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Transfer

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  • 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:
    • Does the treating facility have regular availability of plasmapheresis? Is intravenous immunoglobin therapy available on a regular basis at the treating facility?
    • Does the treating facility have rehabilitation-grade physical therapy and occupational therapy?
    • 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?
    • 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?
    • 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.
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Complications

The earliest and most common complications of the disease are anxiety and depression. Unfortunately, the nature of the disease and the reaction of physicians and family to the problems may act in concert to produce this comorbidity.

The function of GAD is to convert glutamate to GABA. Although this is not the only source of GABA for the CNS, it is a significant source; depending on the situation, GABA can be depleted rapidly. GABA serves as a natural antianxiety compound. The most potent antianxiety medications are based on augmentation of the GABA-A receptor. Because a significant portion of patients with stiff person syndrome have antibodies to GAD, not surprisingly patients also have anxiety. Tragically, anxiety worsens the spasms.

In the early stages, signs of the disease are often subtle to physicians and other health care workers. The patient feels uncomfortable and is aware of the stiffness, but his or her daily life is not disrupted significantly. Unfortunately, the failure of physicians and family to respond to the problem may result in increased anxiety and lead to dysphoria on the part of the patient. Ironically, the anxiety and dysphoria may become more disruptive to the patient's quality of life than the disease, and the patient may be diagnosed with a somatization disorder.

In the late stages of the disease, patients may experience spasm of the pharyngeal muscles, making swallowing difficult and necessitating alternative methods of feeding. Severe paroxysms of spasms may result in skeletal fractures, particularly of the vertebral elements. They also have been reported in long bones. Muscle rupture has been reported in severe cases during spasms.

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Prognosis

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  • Prognosis is variable. Many patients have an indolent course that is primarily asymptomatic and is punctuated by occasional episodes of stiffness. Other patients may have a much more aggressive course, rapidly progressing to the late stages of disease.
  • Other forms of the disease have been described that are accompanied by cerebellar findings, encephalopathy, and other CNS abnormalities, but whether they are separate diseases or different manifestations of the same disease is unclear.
  • Prognosis for stiff baby syndrome is perhaps better. It is generally believed to be self-limiting and resolves with maturation of the CNS. Unfortunately, long-term follow-up studies are lacking.
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Patient Education

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  • For excellent patient education resources, visit eMedicineHealth's Infections Center. Also, see eMedicineHealth's patient education article Tetanus.
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