Which clinical history findings are characteristic 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

Stiff person syndrome usually begins insidiously in the axial muscles, and, if the patient is referred at an early stage, little objective findings may be found at the initial presentation.

In the initial stage of the disease, the patient has an exaggerated upright posture and may report back discomfort or stiffness or pain in the entire back, which is worse with tension or stress. [31]

Patients may report disturbed sleep because, although the stiffness is relieved with sleep, when the patient transitions from rapid eye movement (REM) to stage 1 or 2 sleep they may lose the relief from the spasms, which may awaken them.

In some patients in the early stages, brief episodes of rather dramatic severe worsening that resolve spontaneously within hours or days may occur. Unfortunately, because of the subtle findings and apparent strong psychological components in the early stages, the patients are labeled as psychogenic, and effective treatment is often delayed.

Later in the disease, proximal limb muscles also begin to be involved, particularly when the patient is stimulated, surprised, angered, upset, or frightened. This sort of stimulus may evoke painful severe spasms in the proximal arm and leg muscles that resolve slowly. The patient begins to move very slowly because rapid movement induces severe spasms. Even the distal extremities may become involved when moved rapidly. Exaggerated lumbar lordosis is present combined with contraction of abdominal muscles. [32]

Not surprisingly, depression has been noted as a comorbidity during the late stage of the disease. The patient's quality of life is affected severely at this point, making it difficult or impossible to drive, work, or have a satisfying social life. About 65 percent of SPS patients are unable to function independently. [29]

In the end stages of the disease, few muscles in the body are spared. Trismus is absent. However, facial and pharyngeal muscles may be affected markedly. Joint deformities may occur. Skeletal fractures and muscle ruptures may occur during spasms. Postsurgically, abdominal incisions are at risk of spontaneous rupture. Eating, simple movement, and other simple activities of daily living (ADLs) may be problematic.


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