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[Chiari畸形相关脊髓空洞症的临床及神经放射学特征]

[Clinical and neuroradiological features of syringomyelia associated with Chiari malformation].

作者信息

Isu T, Iwasaki Y, Akino M, Abe H, Tashiro K, Sudo K, Miyasaka K, Saito H

机构信息

Department of Neurosurgery, University of Hokkaido School of Medicine, Sapporo, Japan.

出版信息

No To Shinkei. 1990 Jan;42(1):87-94.

PMID:2337491
Abstract

The clinical presentation and radiological features were analyzed in 30 cases of syringomyelia associated with Chiari malformation. None of the patients had spinal dysraphism. The age on admission ranged from 6 to 59 years with a mean of 27 years. Syringomyelia was diagnosed by CT myelography and or MRI from 1982 to 1988. The initial symptoms were skeletal abnormality (43%) such as scoliosis (12 cases) or pes cavus (one case), unilateral pain or numbness (40%) and unilateral motor weakness (17%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (57%), muscle weakness (57%), muscle atrophy (37%) and lower cranial nerve palsy (40%). The neurological findings were asymmetrical in all patients. The characteristic neurological findings in the cases presenting under 20 years of age were unilateral sensory and motor deficits (61%) with decreased or absent deep tendon reflex on the same side. The localization of the syrinx in axial section varied according to the level even in the same case. In 15 cases with unilateral sensory disturbance or unilateral sensory and motor deficit, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance in the cervical or thoracic level. On the other hand, in 15 cases with bilateral sensory and motor deficit, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

对30例与Chiari畸形相关的脊髓空洞症患者的临床表现和影像学特征进行了分析。所有患者均无脊柱裂。入院年龄为6至59岁,平均27岁。1982年至1988年期间,通过CT脊髓造影和(或)MRI诊断为脊髓空洞症。初始症状为骨骼异常(43%),如脊柱侧凸(12例)或高弓足(1例)、单侧疼痛或麻木(40%)以及单侧运动无力(17%)。入院时常见体征为感觉障碍(100%)、脊柱侧凸(57%)、肌肉无力(57%)、肌肉萎缩(37%)和下颅神经麻痹(40%)。所有患者的神经学表现均不对称。20岁以下患者的特征性神经学表现为单侧感觉和运动障碍(61%),患侧深部腱反射减弱或消失。即使在同一病例中,脊髓空洞在轴位的定位也因水平而异。在15例单侧感觉障碍或单侧感觉和运动障碍的病例中,脊髓空洞位于颈段或胸段与感觉障碍同侧后外侧部分相对应的区域。另一方面,在15例双侧感觉和运动障碍的病例中,脊髓空洞位于中央部分,并延伸至受累较重一侧的后外侧部分。(摘要截选至250词)

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