Kulkarni Girish Baburao, Mustare Veerendrakumar, Pruthi Nupur, Pendharkar Hima, Modi Shailesh, Kulkarni Advaith
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
J Stroke Cerebrovasc Dis. 2014 Nov-Dec;23(10):2819-2826. doi: 10.1016/j.jstrokecerebrovasdis.2014.07.003. Epub 2014 Oct 12.
Craniovertebral junction (CVJ) anomalies are rare and treatable risk factors for posterior circulation strokes (PCSs). Most of the literature comprises case reports and from varied specialities.
Chart review of patients with PCS with CVJ anomalies admitted in a single tertiary stroke care unit. We describe their clinical profile, investigative findings, and therapeutic outcomes.
We saw 7 patients (6 males, mean age 20.1 ± 12.0 years), all previously undiagnosed during the 6-year period. Three patients had a combination of atlantoaxial dislocation (AAD), basilar invagination (BI) and Klippel-Feil anomalies, 2 patients had os odontoideum with AAD, and 1 patient each had isolated AAD and BI. Clinically, they presented with first or recurrent episodes of stroke with neck pain. Examination revealed noticeable skeletal markers and neurologic deficits involving the arterial territory. Routine stroke risk factors were absent. X-ray of CVJ was abnormal and diagnostic in all the patients. Other imaging modalities had their own contributions for identifying associated defects, demonstrating pathophysiology, and management of these patients. Acute care with anticoagulation and supportive measures helped in recovery of all of them. Posterior fusion was done in 4 patients, and 2 patients are awaiting the surgery. During follow-up all patients have improved and none had recurrences.
In case of a young patient with PCS (first/recurrent) in the absence of routine risk factors for stroke, careful physical examination and imaging with basic X-ray may help in the diagnosis CVJ abnormalities, which may be effectively managed by a multidisciplinary team to prevent recurrences.
颅颈交界区（CVJ）异常是后循环卒中（PCS）罕见但可治疗的危险因素。大多数文献为病例报告且来自不同专业。
对一家三级卒中护理单元收治的患有CVJ异常的PCS患者进行病历回顾。我们描述了他们的临床特征、检查结果及治疗结局。
我们共诊治7例患者（6例男性，平均年龄20.1±12.0岁），在6年期间均未被先前诊断出。3例患者合并有寰枢椎脱位（AAD）、基底凹陷（BI）和Klippel-Feil畸形，2例患者为齿突骨合并AAD，另外各有1例患者分别患有孤立性AAD和BI。临床上，他们以首次或复发性卒中发作伴颈部疼痛就诊。检查发现明显的骨骼体征及累及动脉供血区的神经功能缺损。无常规卒中危险因素。所有患者的CVJ X线检查均异常且具有诊断价值。其他影像学检查方法在识别相关缺陷、阐明病理生理及这些患者的管理方面都有各自的作用。抗凝及支持措施的急性治疗有助于所有患者康复。4例患者接受了后路融合手术，2例患者正在等待手术。随访期间所有患者病情均有改善，无复发情况。
对于无常规卒中危险因素的年轻PCS患者（首次/复发），仔细的体格检查及基础X线成像可能有助于诊断CVJ异常，多学科团队可对其进行有效管理以预防复发。
