以发作性意识丧失及入睡后呼吸暂停为特征的颅底畸形:1例报告  

Skull base deformity characterized by paroxysmal loss of consciousness and sleep apnea:one case report

作　　者：陈倩[1] 唐军[1] 陶文强[1] CHEN Qian;TANG Jun;TAO Wenqiang(Department of Neurology,Chongqing Traditional Chinese Medicine Hospital,Chongqing 400021,China)

机构地区：[1]重庆市中医院脑病科,重庆400021

出　　处：《神经病学与神经康复学杂志》2020年第4期186-190,共5页Journal of Neurology and Neurorehabilitation

摘　　要：目的:颅底畸形的主要临床表现包括肢体麻木无力和共济失调等,而以发作性意识丧失为特征的颅底畸形的相关报道很少。本文报道1例以发作性意识丧失及入睡后呼吸暂停为特征的颅底畸形病例,以期为临床提供有益参考。方法:报道1例以发作性意识丧失及入睡后呼吸暂停为特征的颅底畸形病例的临床诊治经过。结果:1例33岁男性患者因"发作性意识丧失2 d"就诊,以发作性意识丧失及入睡后呼吸暂停为特征性临床表现。入院时头颅计算机体层成像显示延髓结构模糊,因病情不稳定,故未能进一步评估颅底结构,导致延误诊断。住院期间,患者反复发作意识丧失及呼吸暂停,翻身时诱发四肢强直痉挛伴面唇紫绀,给予呼吸机辅助通气及抗感染和对症支持治疗。脱机后行头部磁共振成像检查,显示小脑扁桃体下疝合并颅底畸形(Arnold-Chiari畸形)。因保守治疗效果差,遂行手术治疗,切除下移小脑扁桃体等方式予以减压,解除脑干受压状态。术后1周随访,患者神志清晰,脱机后可维持自主呼吸。结论:计算机体层成像诊断颅底畸形较为困难,需与延髓占位性病变进行鉴别。如果临床上高度提示颅底畸形,应尽早开展磁共振成像检查,可以为临床诊断提供可靠依据。尽早实施外科手术有助于改善重症病例的预后。Objective:The main clinical manifestations of skull base deformity include limb numbness and ataxia,but there are few reports about skull base deformity characterized by paroxysmal loss of consciousness.This paper reports a case of skull base deformity characterized by paroxysmal loss of consciousness and sleep apnea.Methods:A case of skull base deformity characterized by paroxysmal loss of consciousness and sleep apnea is reported.Results:A 33-year-old male patient complained of"paroxysmal loss of consciousness for 2 days",characterized by paroxysmal loss of consciousness and apnea after sleep.The structure of medulla oblongata is unclear on computed tomography(CT)scan,and because of the unstable condition,the skull base structure could not be further evaluated,resulting in delayed diagnosis.During hospitalization,the patient had repeated episodes of loss of consciousness and apnea,limb rigidity and face and lip cyanosis when turning over,and was given assisted ventilation,anti-infection and symptomatic support treatment.Magnetic resonance imaging(MRI)showed that cerebellar tonsillar hernia combined with skull base deformity(Arnold-Chiari malformation).Because of the poor effect of conservative treatment,surgical treatment was carried out,and the cerebellar tonsils were removed to relieve the pressure of brain stem.One-week follow-up showed that the patient was conscious and could maintain spontaneous breathing after weaning.Conclusion:It is difficult to diagnose skull base deformity by CT,and it needs to be differentiated from medullary occupying lesions.If the skull base deformity is highly suggested in clinic,MRI should be carried out as soon as possible,which can provide reliable information for clinical diagnosis.Early implementation of surgery is helpful to improve the prognosis of severe cases.

关 键 词：颅底畸形 意识丧失 呼吸暂停 磁共振成像 

分 类 号：R766[医药卫生—耳鼻咽喉科]