皮质基底节综合征八例临床特点及其伴慢性疼痛的临床特征初探

Clinical features of corticobasal syndrome and associated chronic pain:analysis of 8 cases

目的分析皮质基底节综合征(CBS)的临床表现以及伴慢性疼痛的临床特征。方法回顾分析2010年1月1日至2020年6月30日北京医院收治的临床诊断为很可能或可能的CBS患者的病历资料,包括性别、年龄、病程、主诉、神经系统查体及血液生化、肿瘤标志物、感染等实验室检查、简易智力状态检查(MMSE)量表、汉密尔顿抑郁量表(HAMD)评分以及影像学检查结果。结果共收集到8例患者临床特征符合很可能或可能的CBS诊断标准。主要临床表现为不对称的运动障碍,包括强直、震颤、肌阵挛、姿势步态异常等,对左旋多巴反应差,其中7例患者存在失用,5例患者出现异己手,5例患者存在不同程度的认知功能障碍。8例患者中,7例患者头颅MRI检查均显示不同程度的额、顶、颞叶等皮层萎缩,受累肢体对侧为著。5例患者行头18氟-脱氧葡萄糖正电子发射计算机体层成像(18F-FDG PET)检查,结果显示额、顶、颞、枕叶及基底节有广泛的葡萄糖代谢减低,症状较重的对侧更加明显。6例患者伴有疼痛,疼痛类型包括肌张力障碍性疼痛3例、神经病理性疼痛、肌肉骨骼性疼痛和不明原因疼痛各1例,其中1例患者以疼痛为首发症状,1例患者服用左旋多巴后疼痛缓解。结论皮质基底节综合征主要临床特征是不对称的运动障碍和认知功能障碍,伴有失用、皮层感觉障碍和异己肢。头颅MRI和PET检查有助于明确临床诊断。疼痛可能是CBS常见的非运动症状之一。

Objective To investigate the clinical features and associated chronic pain in corticobasal syndrome(CBS).Methods Clinical data of 8 patients diagnosed as probable CBS or possible CBS admitted to Beijing Hospital during January 2010 to June 2020 were retrospectively analyzed.The clinical information included sex,age,course of disease,chief complaint,neurological examination,blood biochemistry,tumor marker,infection and other laboratory tests;the neuropsychological evaluation included Mini-Mental State Examination(MMSE)scale and Hamilton Depression Scale(HAMD);the imaging studies included cranial magnetic resonance imaging(MRI)and/or 18F-Fluorodeoxyglucose positron emission tomography(18F-FDG PET).Results The main clinical manifestations were asymmetrical movement disorders,including rigidity,tremor,myoclonus and abnormalities in posture and gait.Patients showed poor response to levodopa treatment.Among 8 patients,7 had apraxia,5 patients had alien hand,and 5 patients had various degrees of cognitive dysfunction.The cranial MRI demonstrated mild cerebral atrophy which was slightly more severe in the contralateral side of the initially affected limb in 7 of the 8 patients.The 18F-FDG PET scan revealed asymmetric decreased metabolism in the frontal,parietal,temporal,and occipital lobe,as well as in basal ganglia,which was more severe in the contralateral side of the initially affected limb in 5 of the 8 patients.Six of the 8 patients were associated with pain,including dystonic pain in 3 patients,neuropathic pain in 1 patient,musculoskeletal pain in 1 patient,and unexplained pain in 1 patient.Pain was the onset symptom in 1 patient and pain was relieved by taking levodopa in another patient.Conclusions CBS is characterized by asymmetric dyskinesia and cognitive impairment,and often associated with apraxia,cortical sensory deficits,and alien limb.The MRI and PET are helpful for CBS diagnosis.Pain may be one of the common non-motor symptoms in CBS.