부산대학교 도서관

Stiff person syndrome (SPS) is a disease characterized by rigidity in the extremities, axial muscles, and abdominal muscles, severe and painful spasms, and accompanying gait disturbances. Stiff person syndrome is most common in adults between the ages of 20 to 50 and in female sex. The most frequently reported antibody in patients with SPS is antibodies developed against glutamic acid decarboxylase enzyme. In this article, two SPS cases from different age groups who presented with gait disturbance and painful spasms and were found to have glutamic acid decarboxylase antibody positivity were reported with clinical and electrophysiological findings in the light of the literature. As in our cases, SPS should be considered in the differential diagnosis of patients of all age presenting with muscle stiffness and spasms, specifically those that increase with stimulus. Keywords: Electrophysiological findings, glutamic acid decarboxylase, stiff person syndrome. Stiff person syndrome (SPS) is a disease characterized by rigidity in the extremities, axial muscles, and abdominal muscles, severe and painful spasms, and accompanying gait disturbances. This rare disease has an incidence of 1/1,000,000 and an estimated prevalence of 1-2/1,000,000.[1] Stiff person syndrome is two to three times more common in females. Although it is most frequently observed in adults between the ages of 20 to 50, it has also been reported in children and adolescents in the literature.[1,2] Stiff person sendromu (SPS) ekstremite, aksiyel ve abdominal kaslarda rijidite, siddetli ve agrili spazmlar ve yürüyüs bozuklugunun eslik ettigi bir hastaliktir. Stiff person sendromu, en sik eriskinlerde, 20-50 yas arasinda ve kadin cinsiyette görülmektedir. Stiff person sendromu saptanan hastalarda en sik bildirilen antikor glutamik asit dekarboksilaz enzimine karsi gelisen antikorlardir. Bu makalede yürüyüs bozuklugu, agrili spazmlar ile basvuran ve glutamik asit dekarboksilaz antikor pozitifligi saptanan farkli yas gruplarindan iki SPS olgusu klinik ve elektrofizyolojik bulgular literatür esliginde sunuldu. Olgularimizda oldugu gibi kaslarda sertlik ve özellikle uyaranla artan spazmlar ile gelen her yas grubundan hastanin tanisinda mutlaka SPS düsünülmelidir. Anahtar sözcükler: Elektrofizyolojik bulgular, glutamik asit dekarboksilaz, stiff person sendromu.
Stiff person syndrome was first described in 1956 by Moersh and Woltman[3] in 14 patients presenting with difficulty in walking, falls, and accompanying fluctuating, progressive rigidity and painful muscle spasms. [...]