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[扁平颅底、基底沟、齿状突与脑干扭结:与特发性脊髓空洞症、脊柱侧弯和 Chiari 畸形的共同病因]

[Platybasia, basilar groove, odontoid process and kinking of the brainstem: a common etiology with idiopathic syringomyelia, scoliosis and Chiari malformations].

作者信息

Royo-Salvador M B

机构信息

Servicio de Neurocirugia, Clinica Tres Torres, Barcelona, Espanña.

出版信息

Rev Neurol. 1996 Oct;24(134):1241-50.

PMID:8983722
Abstract

INTRODUCTION

A common aetiopathogenic theory for basilar groove (IMB), platybasia (PTB), odontoid retrocession (RTO), kinking of the brainstem (KTC) applied to idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID) and Arnold-Chiari malformation (ARCH) is presented. Confirmation is based on an abnormally low position of the conus medullaris (CMB) in the patients with SMI.

MATERIALS AND METHOD

292 patients with syringomyelia (SM), 231 with SMI were selected. Of these, 55 were chosen who had SMI and in whom the level of the conus medullaris (NCM) could be determined, together with the figures for SMI, IMB, PTB, RTO, KTC, ESCID and ARCH on cervical and lumbar MR. The position of the conus medullaris in 50 patients who did not have SM, ESCID nor ARCH on cervical and lumbar RM was determined.

RESULTS

32 patients had an increased basal angle (58.18%). There was an IMB in a quarter of the patients (25.45%). RTO was observed in half of the patients (47.27%). Just over one third presented a KTC. 6% of the control group had CM at the level of the body of L1, whilst 84.21% of the patients with SMI presented a partial or complete CM image at this level.

CONCLUSIONS

CMB in SMIU and its close relationship with IMB, PTB, RTO AND KTC and also with ESCID and ARCH make it likely that they share the same aetiopathogenic mechanism: an abnormal lack of synchronization of the growth of the neuro-axis and the neural canal (AACNN), causing a specific disorder which is seen as different syndromes.

摘要

引言

提出了一种适用于特发性脊髓空洞症(SMI)、特发性脊柱侧凸(ESCID)和阿诺德-奇亚里畸形(ARCH)的关于基底沟(IMB)、扁平颅底(PTB)、齿状突后移(RTO)、脑干扭结(KTC)的常见病因致病理论。确认依据是脊髓空洞症患者脊髓圆锥(CMB)位置异常低。

材料与方法

选取292例脊髓空洞症(SM)患者,其中231例为特发性脊髓空洞症。在这些患者中,选择了55例能确定脊髓圆锥水平(NCM)的特发性脊髓空洞症患者,以及颈椎和腰椎磁共振成像上特发性脊髓空洞症、基底沟、扁平颅底、齿状突后移、脑干扭结、特发性脊柱侧凸和阿诺德-奇亚里畸形的数据。确定了50例颈椎和腰椎磁共振成像上无脊髓空洞症、特发性脊柱侧凸和阿诺德-奇亚里畸形患者的脊髓圆锥位置。

结果

32例患者基底角增大(58.18%)。四分之一的患者有基底沟(25.45%)。一半的患者观察到齿状突后移(47.27%)。略超过三分之一的患者出现脑干扭结。对照组6%的患者脊髓圆锥位于L1椎体水平,而84.21%的特发性脊髓空洞症患者在此水平呈现部分或完整的脊髓圆锥影像。

结论

特发性脊髓空洞症中的脊髓圆锥及其与基底沟、扁平颅底、齿状突后移、脑干扭结以及特发性脊柱侧凸和阿诺德-奇亚里畸形的密切关系表明,它们可能具有相同的病因致病机制:神经轴和神经管生长异常缺乏同步性(AACNN),导致一种特定疾病,表现为不同综合征。

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