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Spinal muskuler atrofi SMA , medulla spinalis ön boynuz hücrelerinin dejenerasyonu sonucu atrofi ve kas zayıflığı ile karakterize genetik bir hastalıktır. Spinal muskuler atrofili bireylerde pelvik obliklik ve instabil oturmayla birlikte zamanla progresif skolyoz gelişir. Skolyoz, solunum kapasitesini, oturma dengesini ve üst ekstremite fonksiyonelliğini olumsuz etkiler. Yazımızda spinal muskuler atrofi tanılı 9 yaşında, 90˚ lumbal skolyoz nedeniyle skolyoz cerrahisi geçirmiş olan erkek olgunun, cerrahi öncesi ve sonrası üst ekstremite performansı, kas kuvveti ve kavrama kuvveti sunulmuştur. Cerrahi sonrası kaba kavrama kuvveti her iki elde azalırken, 3 nokta kavrama kuvveti arttı. Ayrıca lateral kavrama kuvvetinde sol elde nisbeten bir artış gözlenirken, sağ elde azalma saptandı. Kol elevasyon skoru solda azalırken, sağda değişmedi. T-shirt giyme süresi ise postoperatif 1. ayda azalırken postoperatif 3. ayda nisbeten arttığı, postoperatif 6.ayda ise T-shirt giyemediği gözlendi. Bunun yanısıra biceps, triceps, el bileği fleksör ve ekstansör, deltoid kaslarının kuvvetinde azalmalar belirlendi.
Spinal muscle atrophy SMA is a genetic disease characterized by atrophy and muscle weakness as a result of degeneration of the front cornea cells of medulla spinalis. In spinal muscle atrophy individuals, progressive scoliosis develops over time, along with pelvic shaping and unstable sitting. Scoliosis negatively affects breathing capacity, seating balance and upper end functionality. In our article, atrophy of the spinal muscle is identified at 9 years old, 90 ̊ lumbal scoliosis has suffered scoliosis surgery, the upper end performance, muscle strength and perception strength are presented before and after surgery. After surgery, the brutal capture force was reduced, while the capture force increased by 3 points, while both were obtained. In addition, a relative increase in the strength of lateral conception was observed, while a reduction in the right gain was observed. The arm elevation score was reduced to the left, but not changed to the right. The time of wearing the T-shirt was reduced in the postoperative 1st month, while the postoperative 3rd month was relatively increased, and the postoperative 6th month was not able to wear the T-shirt. In addition to this, biceps, triceps, handheld flexors and extensors, deltoid muscles have been determined by reductions in strength.
Spinal muscular atrophy SMA is a genetic disorder characterized by atrophy and muscle weakness results from the degeneration of the medulla spinalis anterior horn cells. In individuals with spinal muscular atrophy, progressive scoliosis develops over time with pelvic oblique and instabilized sitting. Scoliosis has a negative effect on respiratuary capasity, sitting balance and upper extremity function. In this study, a 9-year-old man with spinal muscular atrophy and a 90 ° lumbar scoliosis who had undergone scoliosis surgery, preoperative and postoperative upper extremity performance, muscle strength and grip strength is presented. Gross grip strength after surgery decreased with both hands, 3 point grip strength increased. In addition, while the lateral gripping force was relatively high in the left hand, the right hand was detected. While the arm elevation score decreased to the left, it did not change on the right. The duration of wearing T-shirts was decreased in postoperative 1 month, relatively increased in postop 3 months and can’t wearing T-shirt in postoperative 6 month. Biceps, triceps, wrist flexors and extansors, deltoid muscles were found to decrease in strength.
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