脑白质

Objective To analyse the mutation types of the NOTCH3 gene with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

目的 分析伴皮质下梗死及白质脑病常染色体显性遗传性脑动脉病的NOTCH3基因突变类型。

Objective To evaluate the peripheral neuropathy in patients with cerebral autosomal dominant arteriopathy with subeortieal infarcts and leukoeneephalopathy in respect of neurophysiology, neuropathology and possible mechanism.

目的报道常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病患者的周围神经电生理和病理改变特点。

The age of onset were 7 to 11 years old.Three patients revealed mental impairment and limbs weakness.Other symptoms included alalia,ataxia in three cases and visual and auditive disturbances in 2 case,One case had visual nerve atrophy and seizures.Testost in one case and 17-hydroxide steroid in another case were degressive.Cerebral biopsy in one case suggest lipide sediment.Testicle impairment in one case was found by ultrasonography.The cerebral magnetic resonance imaging of all the 4 patients showed the characteristic pattern of symmetrical long T1 and long T2 signal in the parieto-occipital region.

结果发病年龄7~11岁，均有智能下降、肢体无力，3例伴言语不清、共济失调，2例伴视听力下降，1例伴视神经萎缩、癫发作，血睾酮降低及血17-羟类固醇降低各1例，B超示双侧睾丸弥漫性病变、脑活检示类脂质沉积病各1例，4例磁共振均见双侧枕顶叶对称性蝶翼状长T1、长T2信号灶，可累及颞叶、内囊和脑干白质。

Low signal was also seen in the motor cortex on T2-weighted images in three of the eight normal volunteers and four of the 16 disease controls: one with a bulbospinal muscular atrophy, two with leukoencephalopathy and one with Binswanger's disease.

在8例正常人对照者中有3例、16例其他神经疾病患者中有4例（1例脊髓延髓性肌萎缩症患者、2例表现为下肢痉挛性轻瘫的不明原因的白质脑病患者、1例表现为痴呆和下肢痉挛性轻瘫的皮质下动脉硬化性脑病患者）在T2像上于运动皮质区也可见低信号。

Results The Fos reactive products were distributed in the piriform cortex,accumbens nucleus,bed nucleus of stria terminalis, cingulate gyrus,nucleus of diagonal band, hypothalamic paraventricular nucleus, supraoptic nucleus, suprachiasmatic nucleus, arcuate nucleus, central amygdaloid, subfornical organ, paraventricular thalamic nucleus, lateral habenular nucleus,ventrolateral part of the central gray,lateral parabrachial nucleus,locus ceruleus,medullary visceral zone.

结果 Fos阳性产物分布于梨状皮质、伏核、终纹床核、扣带回、斜角带核、下丘脑室旁核、视上核、视交叉上核、弓状核、中央杏仁核、穹窿下器、丘脑室旁核、外侧缰核、中脑中央灰质腹外侧区、脑桥臂旁外侧核、蓝斑、延髓内脏带等脑区，而在大脑白质及小脑中无明显的密集分布区。

The myelinization of cerebellar white matter,middle cerebellar peduncles,anterior limb of internal capsule,splenium and genu of corpus callosum,centrum semiovale,the white matter in parietal-occipital area and frontal-temporal area were documented at birth to 5,2～4,3～7,3～5,4～7,2～7,3～9 and 4～12 months old,respectively.

髓鞘形成至成熟时间：小脑上下脚、桥脑背侧、丘脑腹外侧和内囊后肢后部出生时已成熟，小脑自出生至5个月，小脑中脚2～4个月，内囊前肢3～7个月，胼胝体后部、膝部分别是3～5个月、4～7个月，半卵圆中心2～7个月，枕顶叶白质3～9个月，额颞叶白质4～12个月。

Result: The lesions preponderately located in the white matter of temporal lobe, brain stem and cerebellum, with irregular rim/nodular enhancement. All of the 3 MRS showed declined Cho and NAA peak.

结果：本组病例放射性脑损伤病灶主要位於颞叶、脑干及小脑照射野白质内，多呈不规则花环样或结节样强化。3例MRS均显示Cho及NAA下降。

AIM: To study the pathological characteristics of the spongiform leukoencephalopathy in heroin addicts.

目的：分析吸食海洛因患者海绵状白质脑病的病理特征，探讨其白质病变的病理机制。

Usually these are found in the subcortical and central white matter and cortical gray-white junction of both cerebral hemispheres , cerebellum , brainstem , and spinal cord [11] , but periventricular white matter and gray matter of the cortex , thalami and basal ganglia may also be involved.

通常，这些被发现的皮质和中央白质和皮质灰色白色双方交界处的大脑半球，小脑，脑干和脊髓[ 11 ] ，但周围白质和灰质的皮质，丘脑和基底节也可参与。

Methods The MR findings of14patients with cerebral palsy caused by hypoxic-ischemic damage were studied retrospectively and correlated with clinical findings.All those patients underwent MRI.Cerebral cortex and subcortical white matter,deep white matter,basal regions and thalami,and ventricles and subarachnoid space were observed on T1WI and T2WI.

对14例缺氧缺血性脑损伤所致脑瘫患儿行MR检查，在T1WI及T2WI上对大脑皮层及皮层下白质、深部白质、基底节、丘脑、以及脑室和蛛网膜下腔等部位进行观察。

But we don't care about Battlegrounds.

但我们并不在乎沙场中的显露。

Ah! don't mention it, the butcher's shop is a horror.

啊！不用提了。提到肉，真是糟透了。