Stiff person syndrome (SPS) is the rare disease and cause great inefficient. It is characterized by progressive stiffness muscles of trunk and the limbs on which the cramps of muscles overlap. In the electrophysiological investigation of the patients the involuntary discharge of motor unit potentials find and also simultaneous contraction agonistic and antagonistic muscles. SPS is presented with insulin-dependent diabetes mellitus often or is the symptom of the paraneoplastic syndrome. It is commonly associated with high anti-glutamic acid decarboxylaze (GAD) antibody titters in the serum of the blood of patients. Establishing the diagnosis can cause difficulties. We observed the patient in the last period about the atypical course the disease. The diagnosis was confirmed occurrences of the high titters of antibodies anti-GAD, the discharge of motor unit potential in paraspinal muscles in the rest and good response the treatment with diazepamem.