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Cognition disturbance and allolalia were the chief clinical manifestations. The imageology showed that the impairment involved occipital lobe and temporal lobe most. 17 cases presented red-ragged-fibres and parking lot-like ultrastructure in 3 cases. Brain biopsy showed layerage structure. There were 15 of them carried mtDNA A3243G mutation. Conclusions MELAS Syndrome usually involved central nerve system and skeletal muscle.

主要临床表现为认知功能障碍及言语障碍,头颅影像学检查示枕叶、颞叶脑回受累为主。18例行肌肉病理检查显示17例存在破碎样红纤维,3例行肌肉电镜检查发现线粒体堆积呈停车场样排列,3例脑活检示病变皮层组织呈分层样改变,15例发现mtDNA A3243G点突变。

Results The clinical manifestations of TOBS comprised transient unconsciousness, vertigo, dyskinesia of eyeball, abnormality of pupils, dyskinesia of limbs, hemianopsia or cortical blindness, ataxia and memory impairment. MRI showed the local infarction in thalami, cerebellum, midbrain, pons, occipital lobe, temporal inner surface. The features of DSA were occlusion or stenosis in basilar artery and vertebral artery.

结果 TOBS的临床主要表现为一过性意识障碍、眩晕、眼球运动障碍及瞳孔的变化、肢体运动障碍、偏盲或皮质盲、共济失调及记忆功能损害;磁共振显示梗死灶在丘脑、小脑、中脑、脑桥、枕叶、颞叶内侧面;数字减影血管造影显示为基底动脉及椎动脉闭塞或狭窄。

And (20. 5 ± 1.7) ml· 100 g-1 ·min-1, respectively. When compared in 3 groups, the temporal and parietal lobe rCBF had a decreasing tendency in aMCI group, while in VCI-ND group, the most dominant decreasing parts were mainly concentrated in white matter region (17. 7±2. 3 ml·100 g-1·min-1, F = 5. 740, P = 0. 002. Whatever the depth or the width was, beth periventricular and subeortical deep white matter, anterior and posterior ventricular regions were all involved. There are no dominant difference of rCBF in caudate nucleus, lentiform nucleus and thalamus.

2NC组、aMCI组和VCI-ND组患者的rCBF表现比较:aMCI组颞、顶、枕叶的rCBF值呈一定趋势的减少,VCI-ND组额叶的rCBF值呈一定趋势的减少,而VCI-ND组rCBF下降最明显的部位集中在白质区域[(17.7±2.3)ml·100 g-1·min-1,F=5.740,P=0.002],从深度看覆盖了脑室旁白质和皮质下深部白质两部分,从长度看覆盖了白质的前后区域。3组基底节区核团包括尾状核、豆状核和丘脑的rCBF值比较差异无统计学意义。

Patchy hyperdensities are present in both occipital lobes and in the precentral and frontal subcortical white matter.

图2。 MRI 的T2像于双侧枕叶、中央前回及额叶的皮质下白质见斑片状高信号病灶。

Head CT: Bilateral temporal/occipital, bilateral parietal, and right frontal hypodensities with extension across the splenium of the corpus callosum.

头部CT显示:双侧颞/枕叶,双侧顶叶和右侧额叶可见低密度影,并通过胼胝体压部延伸扩展,低密度影无强化,无占位效应。

The trilobed structure of the brain, lying posterior to the pons and medulla oblongata and inferior to the occipital lobes of the cerebral hemispheres, that is responsible for the regulation and coordination of complex voluntary muscular movement as well as the maintenance of posture and balance.

小脑脑的三裂叶状组织,位于脑桥和延髓之后脑半球的枕叶下面,负责调节和协调复杂的自发肌肉运动,同时负责保持身体姿势的平衡注意,注意力集中

The trilobed structure of the brain, lying posterior to the pons and medulla oblongata and inferior to the occipital lobes of the cerebral hemispheres, that is responsible for the regulation and coordination of complex voluntary muscular movement as well as the maintenance of posture and balance.

小脑脑的三裂叶状组织,位于脑桥和延髓之后脑半球的枕叶下面,负责调节和协调复杂的自发肌肉运动,同时负责保持身体姿势的平衡支配心脏的副交感神经发源于延髓的迷走神经核。

The trilobed structure of the brain,lying posterior to the pons and medulla oblongata and inferior to the occipital lobes of the cerebral hemisphere s,that is responsible for the regulation and coordination of complex voluntary muscular movement as well as the maintenance of posture and balance.

小脑脑的三裂叶状组织,位于脑桥和延髓之后脑半球的枕叶下面,负责调节和协调复杂的自发肌肉运动,同时负责保持身体姿势的平衡

The trilobed structure of the brain, lying posterior to the pons and medulla oblongata and inferior to the occipital lobes of the cerebral hemispheres, that is responsible for the regulation and coordination of complex voluntary muscular movement as well as the maintenance of posture and balance.

小脑脑的三裂叶状组织,位于脑桥和延髓之后脑半球的枕叶下面,负责调节和协调复杂的自发肌肉运动,同时负责保持身体姿势的平衡

Sections from frontal lobe, occipital lobe, striatum and hippocampus of normal subjects and sections from hippocampus of AD patients were used in hematoxylin eosin, Lox Fast blue, toluidine blue stains and ulex europaeus agglutinin immunostaining.

应用荆豆凝集素(ulex europaeus agglutinin, UEA)免疫组织化学及甲苯胺蓝等染色观察正常增龄病例额叶、枕叶、壳核、海马及AD患者海马的神经元、毛细血管形态改变,应用图像分析技术测算各部位神经元与毛细血管密度比值并分析其与年龄之间的相关性。

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