痉挛性脑瘫周围神经及骨骼肌亚显微研究的临床意义

The clinical significance of the ultrastucture of skeletal muscle and peripheral nerve in patients with spastic cerebral palsy

目的 探讨痉挛性脑瘫感觉和运动能力障碍的特点与机制。方法 分析 10 2例痉挛性脑瘫患儿临床感觉和运动能力的观察结果。取材 2 5例痉挛性脑瘫的周围神经标本 40个 ,骨骼肌标本 5 4个 ,进行透射电镜观察。结果 本组临床资料可见 ,浅感觉—痛觉基本正常 ,深感觉—关节觉障碍明显 ;主动随意活动能力 ,上肢远端肌肉比近端肌肉减退明显 ,而下肢远近端差异不显著。电镜观察 ,周围神经呈原发性脱髓鞘表现 ,有髓纤维病变明显于无髓纤维 ;骨骼肌纤维亚显微结构Ⅰ型纤维表现突出。结论 痉挛性脑瘫有明显的深感觉障碍 ,而浅感觉基本正常 ,周围神经的病变可能是其原因之一。运动能力障碍 ,在运动精细肌群表现更明显。骨骼肌纤维构型 ,Ⅰ型纤维占优势 。

Objective To study clinical features of the motor and sensory disordeg in patients with spastic cerebral palsy, and to elucidate clinical significance of the ultrastucture of skeletal muscle and peripheral nerve in patients with spastic cerebral palsy.Methods The sense and movement in 102 patients with spastic cerebral palsy were examined. The peripheral nerve and skeletal muscle of 25 patients with spastic cerebral palsy were observed under electron microscope. Results Clinical examination showed that the superficial sense was normal but the deep sense was abnormal. The motor disabilities were obvious in the distal muscle of upper limbs. The damage of primary demyelination was extensive on the peripheral nerves, and the pathological changes of myelinated nerve fibers were more obvious than the nonmyelinated nerve fibers. The electron microscope showed that the mitochondrion of muscle fiber increased in number, the sarcoplasmic reticulum and transverse tubule decreased and developed poorly. Conclusions It is obvious that the superficial sense was normal, and the deep sense was abnormal in spastic cerebral palsy. These results suggest that motor disabilities in cerebral palsy patients are caused not noly by primary neural impairment but also by secondary deteriorar in muscular contractile properties, probably resulting from type 1 fiber predominance and type 2 fiber deficiency.