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癫痫患者的小脑影像学研究进展

回顾癫痫患者小脑结构和功能影像学研究，发现癫痫患者存在小脑萎缩、血流灌注异常、代谢异常和功能障碍。癫痫患者小脑萎缩的相关因素有原发性脑损伤、癫痫发作相关因素(如癫痫发作时间和全身性强直阵挛性癫痫发作)、抗癫痫药物(如苯妥英钠)等。癫痫患者存在小脑血流动力学的改变，且常表现为小脑的过度灌注。癫痫发作间期小脑糖代谢水平下降，但特异的神经递质改变并未被发现。此外，癫痫患者的小脑与其他脑结构(如旁扣代回、丘脑)有静息态功能联系受损，并存在注意力、视空间记忆等功能网络的异常。研究均提示小脑参与了癫痫受损的神经功能网络，或癫痫发作的控制环路。

儿童巨大桥小脑肿瘤手术的围手术期护理

目的总结儿童巨大桥小脑肿瘤手术的护理质量控制，保证术中护理质量。 方法回顾性总结2012年1月－2013年3月18例儿童巨大桥小脑肿瘤手术，了解术前家长担心的问题，术前麻醉、皮肤的准备，术中皮肤护理、体温护理等手术室护理质量管理的相关环节，根据围手术期患儿的应激特点及患儿自身特征，以及手术要求，实施有针对性的术前、术中、术后护理。 结果18例儿童巨大桥小脑肿瘤手术体位安全，暴露充分，术者满意，手术时间8.33～11.66 h，平均10.42 h，出血量100～300 mL，未因手术体位的摆放和护理因素而发生并发症。术后1 d恢复良好，格拉斯哥昏迷评分9～12分。 结论在行儿童巨大桥小脑肿瘤手术时，根据围手术期患儿的应激特点及患儿自身特征，以及手术要求，实施有针对性的术前、术中和术后护理，可使手术野暴露充分，保证术中护理质量，减少手术并发症。

一个遗传性脊髓小脑共济失调7型家系患者的眼底及眼电生理特征

von Hippel-Lindau 病一例

Usefulness of Magnetic Resonance Spectroscopy in the Differentiation of Neurinoma and Meningioma of the Cerebellopontine Angle

摘要:目的: 探讨磁共振波谱(MRS)对鉴别桥小脑角神经鞘瘤与脑膜瘤的价值。 方法 :对8例神经鞘瘤和8例脑膜瘤病例进行MRI平扫和增强扫描,并采用点分辨波谱序列(PRESS,TR/TE=2000/136ms)进行单体素波谱分析。从事磁共振诊断专业的医师根据肿瘤的MRI及MRS表现特征进行鉴别诊断。 结果 :尽管多数神经鞘瘤(5/8)和脑膜瘤(6/8)具有典型的MRI表现特征并仅经MRI即可得到正确鉴别,但部分病例(5/16)为不典型表现者,如脑膜瘤呈长T1长T2信号并伴有囊变,以及神经鞘瘤呈等信号、均匀强化且不伴有囊变或内听道扩大者,鉴别诊断困难。MRS显示脑膜瘤的胆碱/肌酸比值(Cho/Cr)(2.74±1.47)略高于神经鞘瘤(2.70±1.21),但差异无统计学意义。丙氨酸(Ala)在脑膜瘤中的出现率(4/8)显著高于神经鞘瘤(0/8)(Plt;0.05),谷氨酸盐/谷氨酰胺(Glx)在脑膜瘤中的出现率(5/8)也高于神经鞘瘤(2/8);而肌醇(Ins)在神经鞘瘤中的出现率(4/8)高于脑膜瘤(2/8)。结合MRS表现,在MRI上尚难鉴别的病例可被明确诊断,并与最终病理结果相符。 结论 : MRI有助于多数桥小脑角神经鞘瘤与脑膜瘤的鉴别诊断,但对不典型病例有一定局限性;MRS对不典型病例的鉴别具有重要的参考价值。Abstract: Objective: To explore the value of magnetic resonance spectroscopy (MRS) in the differentiation of neurinoma and meningioma in the cerebellopontine angle. Methods : 16 cases, including 8 cases of meningiomas and 8 cases of neurinomas, underwent MRI (plan and gadoliniumenhanced scan) and singlevoxel proton MRS (PRESS series, TR/TE=2000/136ms). MRI and MRS results were reviewed and differential diagnoses were made by professional radiologists. Results : Although most cases (11/16) were typical and clearly classified on MRI, atypical cases were also observed, such as meningioma of long T1/T2 signal intensity with small cystic foci, and neurinoma of isointensity and homogenous enhancement without cystic change or enlargement of internal acoustic meatus. The choline to creatine ratio (Cho/Cr) of meningioma (2.74±1.47) was higher than that of neurinoma (2.70±1.21), however the difference was not significant (Pgt;0.05). Alanine was observed in 4/8 meningiomas but none of neurinomas (Plt;0.05), and glutamine/glutamate was more frequently visualized in meningiomas (5/8) than in neurinomas (2/8), whilemyoinositol was more frequently observed in neurinomas (4/8) than in meningiomas (2/8). With MRS, cases difficult to differentiate were clearly and correctly classified. Conclusion : Although most CPA neurinomas and meningiomas can be diagnosed by MRI, MRS is useful for the differential diagnosis of atypical cases.

自发性低颅压综合征致小脑扁桃体下疝及硬膜下血肿护理一例

Clinical and electroencephalogram features of dyssynergia cerebellaris myoclonica

ObjectiveWe report two family and one sporadic case with dyssynergia cerebellaris myoclonica, investigate the clinical and neural electrophysiological features. MethodsThe proband and sporadic patient was examined by clinical, neuroimaging, video-EEG and synchronous electromyography. ResultsThere were 6 patients with dyssynergia cerebellaris myoclonica of the 27 family members in the first family(3 male and 3 female). There were 4 patients with dyssynergia cerebellaris myoclonica of the 20 family members in the second family(2 male and 2 female). All patiens had disproportionately myoclonus, epilepsy and progressive cerebellar ataxia. EEG showed bursts of spike-slow wave, polyspilke-slow wave distributing in the bilateral brain both in ictal and interictal period, sometimes it is especially in central, parietal and frontal area. EEG showed bursts of spike-slow wave, polyspilke-slow wave distributing in the central, parietal and frontal area in interictal period. Pathology of the skin and muscles are normal. ConclusionThe diagnosis of dyssynergia cerebellaris myoclonica was mainly based on typical clinical manifestations, brain MRI and EEG changes.Long time video EEG and synchronous EMG is important for the diagnosis. Skin and muscles pathology can be normal.

Applied Anatomy of the Trochlear Nerve in the Tentorial Lateral Region

目的:通过对滑车神经行经小脑幕侧方区域的应用解剖学研究,寻找小脑幕侧方区域手术时避免损伤滑车神经的临床解剖标志。方法:对15例（男10例,女5例）防腐固定无畸形、无病变的成人头颅标本用红色乳胶灌注后,10倍手术显微镜下观察滑车神经在小脑幕侧方区域的行径,及其与周围重要神经、血管结构的毗邻关系,测量滑车神经长度、宽度、厚度及其与周围标志点的距离,并对所得结果进行统计学分析。结果:滑车神经在小脑上动脉和大脑后动脉之间向前行,进入小脑幕侧方区域,在动眼神经三角的后部穿越游离缘硬膜,其长度为（6.78±1.87）mm,宽度为（1.09±0.21）mm,厚度为（0.78±0.11）mm。滑车神经进入小脑幕侧方区域的入口处位于前床突、颈内动脉床突上段起始部、动眼神经入口后方,位于后床突后外方；距离前床突（23.24±3.18）mm、颈内动脉床突上段起始部（17.57±3.26）mm、动眼神经入口（11.42±3.32）mm；距离后床突（14.21±3.25）mm。结论:行小脑幕侧方区域手术时,为避免损伤滑车神经,前床突、后床突、颈内动脉床突上段起始部和动眼神经入口可以作为寻找滑车神经入口的重要标志,同时注意区分小脑上动脉和大脑后动脉。

新生儿大枕大池伴小脑半球发育不全一例及文献复习

目的　报道并分析1例胎儿颅后窝池增宽的新生儿预后。 方法　2011年3月在产科建卡产检发现胎儿颅后窝池增宽的1例孕妇进行定期产前超声检查，动态观察其胎儿颅后窝池宽度的变化，并对新生儿行出生后随访。 结果　本例孕妇在产前超声检查随访中，其胎儿颅后窝池宽度从孕33周的11 mm增至孕38周的18 mm，出生后其新生儿诊断为颅后窝大枕大池伴小脑半球发育不良。 结论　产前检查发现胎儿颅后窝池增宽，应进行超声或核磁共振动态随访，注意观察胎儿有无其他部位的结构异常或畸形。同时应重视产前对胎儿小脑发育情况的评估，以便早期诊断处理。

Correlation analysis of body mass index and disease severity in patients with spinocerebellar ataxia type 3

Objective To explore the correlation between body mass index (BMI) and disease severity in patients with spinocerebellar ataxia type 3 (SCA3). Methods Patients who visited the Department of Neurology of the First Affiliated Hospital of Fujian Medical University with a confirmed diagnosis of SCA3 between July 2022 and August 2023 were selected as a case group, and healthy individuals between June 2024 and October 2024 were selected as a control group, and the BMI levels of the two groups were compared. Patient demographics and clinical statistics were collected, the severity of ataxia in SCA3 patients was assessed using the Scale for the Assessment and Rating of Ataxi, and the relationship between BMI and disease severity was evaluated. Results A total of 101 patients and 101 healthy individuals were included. The BMI levels of SCA3 patients were significantly lower than those of normal controls (t=−2.370, P=0.019). The results of the multiple linear regression model showed that the BMI, disease duration and smoking history had an effect on the disease severity of SCA3 patients (P<0.05), and disease duration and disease severity had a significant effect on the disease progression in SCA3 patients (P<0.05). Conclusion There may be a correlation between BMI and disease severity in SCA3 patients, and controlling the BMI level may help to control the disease in SCA3 patients.