Lan Zhi Gang, Richard Seidu A, Liu Jiagang, You Chao
Department of Neurosurgery, West China Hospital, Sichuan University, Sichuan.
Neurol Int. 2017 Oct 2;9(3):7336. doi: 10.4081/ni.2017.7336. eCollection 2017 Aug 29.
Chiari type I malformation with cervicothoracic syringomyelia although very common in clinical practice usually in children can progress slowly and mimic muscular dystrophies in adulthood. We present a rare adult case of Chiari type I malformation with cervicothoracic syringomyelia subterfuge as Flail arm syndrome. A 44-year-old man was diagnosed with congenital type I Chiari malformation with cervicothoracic syringomyelia about 21 years ago without surgery. His health status deteriorated over the years until 21 days prior to presentation when he had severe pain in the right knee. In his upper limbs, he had bilateral corresponding severe weakness of 0/5 proximal strength and 0/5 strength in his distal muscles. Magnetic resonance imaging (MRI) revealed an enlargement of the spinal cord from C1-C4 level with a mass that appeared hypo-dense on T1 and hyperdense on T2. Syringomyelia is a potentially serious neurologic condition that can mimic other neuromuscular disorders. Early detection and diagnosis with MRI is crucial to avoid irreversible neurological complications. We suggest that whether asymptomatic or symptomatic, decompressive surgery should be carried out to allow for free flow of cerebrospinal fluid thereby improving the quality of life for the patient.
尽管I型Chiari畸形合并颈胸段脊髓空洞症在临床实践中非常常见，通常发生于儿童，但其在成年期可能进展缓慢并类似肌肉萎缩症。我们报告一例罕见的成年I型Chiari畸形合并颈胸段脊髓空洞症伪装成连枷臂综合征的病例。一名44岁男性约21年前被诊断为先天性I型Chiari畸形合并颈胸段脊髓空洞症，未接受手术治疗。多年来他的健康状况逐渐恶化，直到就诊前21天，他出现右膝剧痛。其上肢双侧近端肌力为0/5，远端肌肉肌力为0/5，严重无力。磁共振成像（MRI）显示C1 - C4水平脊髓增粗，有一个在T1加权像上呈低密度、T2加权像上呈高密度的肿块。脊髓空洞症是一种可能严重的神经系统疾病，可类似其他神经肌肉疾病。通过MRI进行早期检测和诊断对于避免不可逆的神经并发症至关重要。我们建议，无论有无症状，均应进行减压手术，以使脑脊液自由流动，从而改善患者的生活质量。
