Han Jingzhe, Cao Duanhua, Ma Guomei, Wang Tingting, Ji Ye, Kang Zhilei
Department of Neurology Department of MRI, Harrison International Peace Hospital, Hengshui, Hebei, China.
Medicine (Baltimore). 2017 Nov;96(45):e8647. doi: 10.1097/MD.0000000000008647.
Patients with Klippel-Feil syndrome (KFS) are always anomaly associated with vertebrobasilar dysplasia. That may present commonly as infarction of brainstem, medulla, and cerebellum. In this article, we reported a rare case of lateral medullary infarction (LMI) with similar features of Brown Sequard syndrome caused by vertebrobasilar dysplasia and KFS, and the 2 rare conditions that are causally related. The case is being reported because of its unusual and rare presentation.
A 38-year-old female presented with acute unsteadiness, along with a tendency to lean to the left side while walking or sitting, and paresthesia in the right lower limb and trunk, at 2 days before admission. She had no history of hypertension and diabetes, but had a 20 years history of neck pain and dizziness, which was related to head movement.
Brown Sequard syndrome and a lesion of the left thoracic spinal cord were suspected initially. KFS was confirmed by the cervical magnetic resonance imaging (MRI) and computed tomographic angiography (CTA) results. Transcranial Doppler (TCD) results confirmed that there was a causal link between LMI and KFS.
The patient rejected the operation of stabilization of the cervical spine with fusion at appropriate levels.
No recurrence of stroke, but neck pain and dizziness remained after 6 months of discharge.
For such patients, the conventional treatment of cerebral infarction might be ineffective, but stabilization of the cervical spine with fusion at appropriate levels can successfully prevent further episodes of syncope and stroke.
克-费综合征(KFS)患者常伴有椎基底发育异常。这可能通常表现为脑干、延髓和小脑梗死。在本文中,我们报告了一例罕见的外侧延髓梗死(LMI)病例,其具有由椎基底发育异常和KFS引起的类似布朗-塞卡尔综合征的特征,以及这两种因果相关的罕见情况。报告该病例是因其不寻常且罕见的表现。
一名38岁女性在入院前两天出现急性步态不稳,行走或坐立时倾向于向左倾斜,右下肢和躯干感觉异常。她无高血压和糖尿病病史,但有20年的颈部疼痛和头晕病史,与头部运动有关。
最初怀疑为布朗-塞卡尔综合征和左胸段脊髓病变。颈椎磁共振成像(MRI)和计算机断层血管造影(CTA)结果证实为KFS。经颅多普勒(TCD)结果证实LMI与KFS之间存在因果关系。
患者拒绝了在适当节段进行颈椎融合固定手术。
出院6个月后未再发生中风,但颈部疼痛和头晕仍存在。
对于此类患者,传统的脑梗死治疗可能无效,但在适当节段进行颈椎融合固定可成功预防进一步的晕厥和中风发作。