Stiff person syndrome

Abstract

Stiff person syndrome (SPS) is a rare disorder, characterised by fluctuating rigidity and stiffness of the axial and proximal lower limb muscles, with superimposed painful spasms and continuous motor unit activity on electromyography. Although rare in general neurology practice, once observed it is unforgettable. The general neurologist may see only one or two cases during his or her career and as such it remains underdiagnosed. Left untreated, SPS symptoms can progress to cause significant disability. Patients have a poor quality of life and an excess rate of comorbidity and mortality. The severity of symptoms and lack of public awareness of the condition create anxiety and uncertainty for people with the disease. This review aims to raise awareness of SPS and to improve the likelihood of its earlier diagnosis and treatment.

A Stiff Person Case Admitted Phsysical Medicine and Rehabilitation Outpatient Clinic with Low Back Pain

“…It occurs more often among women than men, and it usually occurs when patients are in their 30s. In approximately 60%-80% of SPS cases, anti-GAD autoantibodies in CSF were found to be positive (1). Moreover, in our case, anti-GAD autoantibodies in CSF were positive.…”

“…These contractions initially reveal a fluctuating course. As the disease progresses, the rigidity of the lumbar paraspinal muscles, which is characteristic for SPS, causes excessive increase in the lumbar lordosis (hyperlordosis) (1,14). Rigidity spreads from the chest to the hips and proximal lower limb muscles.…”

“…Stiff person syndrome (SPS) is a rare neurological disease that was first described by Moercsh and Woltman in 1956 as "stiff man syndrome" in 14 patients with complaints of stiffness in the dorsal, abdominal, and femoral muscles (1,2).…”

“…Stiff person syndrome is an autoimmune disease that is characterized by the rigidity of the abdominal, axial, and extremity muscles; lumbar hyperlordosis; difficulty in moving; gait disturbance; and painful muscle spasms (1). Showing a fluctuating or stable course, it leads to disability (3).…”

“…Among 60 patients who were monitored by Dalakas et al (6), it was most frequently observed in patients with type 1 diabetes mellitus (DM) in addition to pernicious anemia, vitiligo, and thyroiditis SPS. Five percent of patients have been reported to have the paraneoplastic condition (1,3,6,8). In some cases that develop with neoplasms, such as breast cancer, anti-amphiphiles or anti-gephyrin autoantibody positivity has been reported (9,10).…”