What are the complications of stiff person syndrome?

Updated: Jun 14, 2018
  • Author: Nancy Theresa Rodgers-Neame, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

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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