Introduction:

stiff-person syndrome (SPS) is a rare immune-mediated central nervous system disorder characterized by painful contraction, stiffness and spasms affecting the axial and peripheral musculature in a progressive manner.

Case report:

a 47-year-old male patient, affected by stiffness and weakness in the right lower extremity, progressing in weeks to the other lower extremity and in months to the axial and proximal musculature of the four extremities; making ambulation, trunk flexion and arm movement difficult. He presented lumbar hyperlordosis and muscle spasms. Electromyography showed continuous muscle activity in agonist and antagonist muscles. Stiff person syndrome (SPS) was diagnosed. The treatment applied was immunomodulator (steroids and intravenous immunoglobulins) and symptomatic with benzodiazepines, baclofen and physical therapy, the evolution was satisfactory.

Conclusions:

SPS is not frequent in neurological-clinical practice, its definitive diagnosis depends on specific complementary studies, and its clinical manifestations make it detectable allowing timely management and prevention of disabilities associated with these patients.

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