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)(Plt;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 gadoliniumenhanced scan) and singlevoxel 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 isointensity 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 (Plt;0.05), and glutamine/glutamate was more frequently visualized in meningiomas (5/8) than in neurinomas (2/8), whilemyoinositol 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.
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.
How to realize the control of limb movement and apply it to intelligent robot systems at the level of cerebellar cortical neurons is a hot topic in the fields of artificial intelligence and rehabilitation medicine. At present, the cerebellar model usually used is only for the purpose of controlling the effect, borrowing from the functional mode of the cerebellum, but it ignores the structural characteristics of the cerebellum. In fact, in addition to being used for controlling purposes, the cerebellar model should also have the interpretability of the control process and be able to analyze the consequences of cerebellar lesions. Therefore, it is necessary to establish a bionic cerebellar model which could better express the characteristics of the cerebellum. In this paper, the process that the cerebellum processes external input information and then generates control instructions at the neuron level was explored. By functionally segmenting the cerebellum into homogeneous structures, a novel bionic cerebellar motion control model incorporating all major cell types and connections was established. Simulation experiments and force feedback device control experiments show that the bionic cerebellar motion control model can achieve better control effect than the currently widely used cerebellar model articulation controller, which verifies the effectiveness of the bionic cerebellar motion control model. It has laid the foundation for real brain-like artificial intelligence control.
ObjectiveTo study the clinical characteristics of patients onset epilepsy Dentatorubral-pallidoluysian atropy (DRPLA) in Epilepsy Center of Guangdong 999 Brain Hospital and improve understanding of the disease. MethodsCollected five patients from August 2014 to August 2016 in Guangdong 999 Brain Hospital, whom diagnosed through genetic testing of DRPLA, analysed their disease course, family history, video-EEG, brain MRI and treatment data. ResultsDRPLA performed as neurodegenerative diseases, and epilepsy population mainly performed as progressive myoclonic epilepsy (Progressive myoclonus epilepsy, PME). ConclusionDRPLA is autosomal dominant neurodegenerative disease. In patients with cerebellar atrophy, neurological regression, ataxia, drug refractory epilepsy, it is recommended routinely to detect ATN1 gene, so that timely diagnosis and genetic counseling.