Blondeau M, Brunet D, Blanche J M, Debauchez C, Etienne M
Sem Hop. 1984 Mar 8;60(11):771-5.
Two cases of tetraparesis due to cervico-occipital spinal cord compression in infants with achondroplasia are reported. Neurological disorders in achondroplasia are produced by structural anomalies of the cranium and spinal canal. They can be divided into two categories: hydrocephalus and radicular and spinal cord compression syndromes. Cervico-occipital compression is more frequent in childhood and may occur very early, in the first months of life. Early diagnosis of this complication and anatomic evaluation can now be obtained by computed tomography, which shows the exact dimensions of the foramen magnum, and myelography. Achondroplasia requires very close neurologic monitoring and, when findings are abnormal, radiologic investigations of the cervico-occipital zone. This allows for early surgical management which is the only means of obtaining significant therapeutic results.
报告了两例患有软骨发育不全的婴儿因枕颈部脊髓受压导致四肢轻瘫的病例。软骨发育不全中的神经功能障碍是由颅骨和椎管的结构异常引起的。它们可分为两类:脑积水以及神经根和脊髓受压综合征。枕颈部受压在儿童期更为常见,并且可能在生命的最初几个月很早就发生。现在可以通过计算机断层扫描来早期诊断这种并发症并进行解剖学评估,计算机断层扫描可以显示枕骨大孔的确切尺寸,还可以通过脊髓造影来进行评估。软骨发育不全需要非常密切的神经学监测,当发现异常时,需要对枕颈部区域进行放射学检查。这有助于早期进行手术治疗,而手术治疗是获得显著治疗效果的唯一方法。