Chiari malformation presenting as a focal motor deficit
Chiari malformation presenting as a focal motor deficit
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DOI：
10.3171/ped/2008/1/5/392
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发表时间：
2008-05-01
影响因子：
1.9
通讯作者：
Souweidane, Mark M.
中科院分区：
文献类型：
--
作者：
Laufer, Ilya;Engel, Murray;Souweidane, Mark M.
Chiari畸形可能表现出广泛的症状和体征。然而，局灶性足部无力作为Chiari畸形的表现尚未在儿科神经外科文献中描述。两名患有Chiari畸形和完全性脊髓空洞症的儿童表现为孤立性腰椎神经根病。经枕下减压和颈椎板切除术后神经功能缺损完全消除。这些病例强调了对整个颅脊髓轴进行成像的重要性，当初始成像未能显示脊柱水平缺陷的结构异常时，应避免针对神经根病过程进行治疗干预。讨论了这种不寻常表现的可能病理生理起源。根据从这些患者身上获得的经验，对出现局灶性缺陷的儿童的诊断检查和管理提出了建议，这些儿童没有受影响的根水平的成像支持。Chiari畸形很少伪装成下肢运动和感觉缺陷，适当的治疗可使功能恢复良好。
Chiari malformations may present with a wide range of symptoms and signs. Nevertheless, focal foot weakness as a presentation of a Chiari malformation has not been described in the pediatric neurosurgical literature. Two children with Chiari malformations and holocord syringomyelia presented with manifestations of a supposed isolated lumbar radiculopathy. Neurological deficits completely resolved after decompressive suboccipital craniectomy and cervical laminectomy. These cases emphasize the importance of imaging the entire craniospinal axis and avoidance of therapeutic intervention specifically aimed at a radiculopathic process when initial imaging fails to show a structural abnormality at the spinal level of deficit. The possible pathophysiological origins for this unusual presentation are discussed. Based on the experience gained with these patients, recommendations are made regarding the diagnostic workup and management of this entity in children presenting with focal deficits that are not supported by imaging of the affected root levels. Chiari malformations may rarely masquerade as lower motor and sensory deficits, and appropriate treatment may result in excellent recovery of function.