基底节出血30天死亡概率的预测研究

目的：探讨基底节出血30天死亡的危险因素,建立基底节出血30天死亡的概率探测模型。方法：回顾性分析我院近5年来病房收治的290例基底节出血病例,对影响基底节出血死亡的危险因素进行多元Logistic回归分析。结果：290例基底节出血病人,30天病死率为13．4％,单因素X检测表明,意识障碍,脑出血破入脑室,大量出血,伴发糖尿病,缺血性心脏病,消化道出血等6个因素为基底节出血30天死亡的危险因素（＿＜0．05）,多因素Logistic回归分析发现,仅有意识水平＊（OR＝6．949,P＜0．01）和消化道出血（OR＝29．480,P＜0．01）是基底节出血30天死亡的独立预测因子,基底节出血30天死亡的概况预测模型为Logit P=-5.150 2.282X意识＋3．637X消化道出血,该模型对基底节出血30天死亡或生存预测总的正确率为92．8％。结论：意识水平和伴有消化道出血是影响基底节出血30天死亡的最重要危险因素,由此建立的基底节出血30天死亡概率预测模型对临床具有一定的指导意义。     

Familial basal ganglia calcifications visualized by computerized tomography

Intracranial calcification can now be detected easily and precisely with the advent of computerized tomography. A familial case of striopallidal calcification with a rare hereditary pattern of autosomal dominancy is presented. None of the family members, aged from 8 to 62, displayed any neurological abnormality. All female family members had, bilaterally, short fourth metatarsais. Serum calcium and phosphorus values were not abnormal, although such physical findings are compatible with pseudohypoparathyroidism. The family tree suggested autosomal dominant heredity with a penetrance rate of 100%. Our survey revealed that no more than 10 cases of familial striopallidal calcification excluding ours have been reported to date. Only by utilizing CT was the hereditary pattern of our case determined accurately.     

Excessive synchronization of basal ganglia neurons at 20 Hz slows movement in Parkinson's disease

Excessive synchronization of neuronal activity at around 20 Hz is a common finding in the basal ganglia of patients with untreated Parkinson's disease (PD). Correlative evidence suggests, but does not prove, that this spontaneous activity may contribute to slowness of movement in this condition. Here we investigate whether externally imposed synchronization through direct stimulation of the region of the subthalamic nucleus at 20 Hz can slow motor performance in a simple unimanual tapping task and whether this effect is frequency selective. Tapping rates were recorded on 42 sides in 22 patients with PD after overnight withdrawal of medication. Tapping was performed without stimulation and during bilateral stimulation at 20 Hz, 50 Hz and 130 Hz. We found that tapping rates were slowed by 8.2+/-3.2% (p=0.014) during 20-Hz stimulation in subjects with relatively preserved baseline function in the task. This effect was frequency selective. The current data provide proof of the principle that excessive beta synchrony within the basal ganglia-cortical loop may contribute to the slowing of movements in Parkinson's disease.     

基底节病变时语言障碍的书写特点

目的通过对基底节病变时患者语言障碍及汉字书写特点的研究,探讨其可能的神经心理学机制。方法对103例基底神经节病变患者采用汉语失语检查法(ABC法)和汉语失写检查法(CAB法),从听、说、读、写4个方面全面评估患者的语言能力,筛选出语言障碍者40例,确定其书写障碍的类型、严重程度及其与其他语言障碍之间的相关性。结果除书面理解和口语表达间差异无显著性意义外,其余口语和书面语语言障碍间的差异均有显著性意义。在口语表达与书面语表达各项之间均存在正相关关系,在书面语理解与书面语表达之间均存在正相关关系,基底节病变书写障碍程度左侧重于右侧,双侧最轻。结论在基底神经节病变的口语和书面语损害之间,既相互联系又各具特点,基底神经节参与言语和书写过程的神经心理学机制是有所差异的。     

Neurological disorders in 166 patients with basal ganglia calcification: a statistical evaluation

Summary Patients investigated at our institute during the last decade included 166 (1.2%) who showed uni- orbilateral basal ganglia calcification on computed tomography. We tested the significance of this neuroradiological observation by statistical comparison of these patients' clinical disorders with the findings in a random sample of 622 patients without basal ganglia calcification. The odds for the most common neurological disturbances were similar in patients with and without basal ganglia calcification. After adjustment for differences in age and brain atrophy there was no evidence of a significantly increased risk of dementia (odds ratio 1.1), cerebral infarction (1.4), epilepsy (0.9), vertigo (1.6), headache (1.8), or alcoholism (0.9), which represented the most common diagnoses. We conclude that basal ganglia calcification cannot be considered as a clinically relevant neuroradiological finding in the majority of cases and that it should not be used as an explanation for frequently observed neurological disturbances.     

酷似高血压脑出血的基底节区血管畸形的显微外科治疗

目的探讨基底节区血管畸形出血的诊断及显微外科治疗。方法回顾性收集2011年2月~2014年12月在我院收治的12例基底节区血管畸形出血患者的临床和影像学资料、手术所见及病理结果。结果 10例行小骨窗开颅术,2例行大骨瓣开颅术,术中见血肿壁异常血管组织,无明显粗大的供血动脉及引流静脉,术后病检结果动静脉畸形8例,海绵状血管瘤4例。结论对于基底节区脑出血的患者,应警惕血管畸形出血的可能,术前应注意鉴别,该部位血管畸形多较小,无明显粗大的供血动脉及引流静脉,术中应注意在血管畸形周边切除畸形血管团。     

Macro-aneurysm in the basal ganglia region

A 59-year-old female patient suddenly developed vomiting and gait disturbances followed by decreasing consciousness. CT scans revealed a hemorrhage within the left basal ganglia region with rupture into the ventricles and consecutive hydrocephalus. On angiography an aneurysm in the region of the caput nuclei caudati was shown to be the source of the bleeding. On repeat-angiography 4 months later the aneurysm was no longer visualized, probably due to thrombosis. This is an extraordinary case of a basal ganglia aneurysm comparable with the aneurysms of Willis' circle, but located in a region where generally microaneurysms — mostly combined with hypertension or moyamoya disease can be found.     

Mobius syndrome with basal ganglia calcification

Basal ganglia calcification has not been described in Mobius syndrome. A family with two children with Mobius syndrome are reported. Bilateral basal ganglia calcification was seen on computed tomography in both. This is the first family where cerebral involvement has been clearly documented in this syndrome.     

Calcification of the basal ganglia in Down's syndrome and Alzheimer's disease

Summary The prevalance and severity of calcification in the basal ganglia (BGC) has been examined histopathologically in 194 patients divided into ten diagnostic categories. The prevalence and severity of BGC was greater (for age) in Down's syndrome and in patients under 75 years of age with Alzheimer's disease. The severity, but not the prevalance, of BGC was greater in Down's syndrome than in patients of similar age with Alzheimer's disease. Both the prevalence and the severity of BGC in patients over 75 years of age with Alzheimer's disease were as expected for age alone. The increased prevalence and severity of BGC in Down's syndrome and in younger patients with Alzheimer's disease appeared not to be related to the presence of dementia or degenerative disease per se, nor was it affected by the presence of cerebral infarction. BGC may result from an age-related disturbance of the structure of arteries within the globus pallidus, which is accelerated (or occurs prematurely) in Down's syndrome and in younger patients with Alzheimer's disease, but probably does not form part of that spectrum of changes that constitutes the pathological basis of Alzheimer's disease.     

丘脑及基底节区生殖细胞瘤

报告５例经手术及病理证实的丘脑或基底节区生殖细胞瘤的临床及影像学特点。其发生率占同期所有颅内生殖细胞瘤的６％。５例均为男性。主要临床症状为偏侧肢体无力，两例检查发现精神异常及性早熟。与松果体区及鞍上区生殖细胞瘤的ＣＴ及ＭＲ表现相比，本组丘脑或基底节区生殖细胞瘤的主要特点在瘤体的大小及密度信号、瘤内出血及相邻结构的变化几方面。     

帕金森病患者基底节区的磁共振氨基质子转移成像研究

目的 探讨磁共振氨基质子转移成像(APT)技术对于发现帕金森病患者基底节异常改变的可行性.方法 收集27例帕金森病患者和23名年龄及性别相匹配的健康对照者进行头颅APT成像和常规磁共振检查.测量双侧苍白球、壳核和尾状核的酰胺质子不对称磁化转移率(MTRasym),分别采用独立样本t检验和配对样本t检验比较帕金森病患者与健康对照者、帕金森病患者起病侧和对侧各脑结构MTRasym (3.5 ppm)的差异.使用单因素方差分析比较健康对照者和不同严重程度帕金森病患者间各脑结构MTRasym(3.5 ppm)的差异.结果 帕金森病患者苍白球、壳核和尾状核的MTRasym (3.5 ppm)均高于健康对照者[分别为(0.89±0.12)％与(0.57 ±0.16)％,(1.05±0.11)％与(0.82±0.15)％,(1.15±0.13)％与(0.78 ± 0.19)％;t=3.311、2.562和3.277,均P＜0.05].健康对照组、轻度和中重度帕金森病组基底节各脑结构MTRasym(3.5 ppm)的差异具有统计学意义,且轻度帕金森病患者苍白球、壳核和尾状核的MTRasym(3.5 ppm)明显高于健康对照者.帕金森病患者基底节各脑结构起病侧的MTRasym(3.5 ppm)虽均略低于对侧,但差异均无统计学意义.结论 APT成像技术可以敏感地显示早期帕金森病患者和健康对照者基底节各脑结构MTRasym(3.5 ppm)的差异,是一种评价帕金森病患者脑代谢异常的有效工具.     

The germinomas arising from the basal ganglia and thalamus

Objective To introduce the features of germinomas arising from the basal ganglia (BG) and thalamus. Method Retrospective analysis was done with the clinical cases of germinomas in BG and thalamus from 1996 to 2000. The data included the symptoms, signs, neuroimaging findings, treatment, and outcomes. Result Fourteen cases were included, only one female was included. The main symptoms are disorder of numbness and weakness in limbs. Neuroimaging showed no or mild peritumor high signal in T2 weighted imaging of magnetic resonance, accompanied with cyst, calcification or bleeding. Total gross resection was obtained in nine cases, subtotal resection in four. Follow-up data were available in 11 cases with average of 56 months. Eight cases underwent only postoperative radiotherapy, one underwent only chemotherapy, and two underwent both. One case died of complication 6 months after chemotherapy, the rest lived good life. Conclusion Germinoma in BG and thalamus predominate in a boy. The neuroimaging features are very informative for diagnosis. Surgical resection should not be the first choice although it is has lesser complications. The long-term outcome is favorable.     

Germinoma involving the basal ganglia in children

Background Germinoma originating in the basal ganglia is rare, and the majority of reported papers have been from Japan. In a collection of the first 500 cases of primary brain tumors in children in Taipei Veterans General Hospital, six pure germinomas with tissue diagnosis situated in this location. Materials and methods We reviewed the clinical features, neuroimaging studies, tumor markers, management, and outcome of these six patients. Results All of them were boys. The median age of onset of symptoms was 9.7 years. They uniformly presented with hemiparesis. The average duration of symptoms before surgical management was 1 year. One patient had bilateral basal ganglia tumors. Serum β-human chorionic gonadotropin levels was elevated (128 mIU/ml) in one patient. Longitudinal neuroimaging studies in four patients clearly showed that the tumor arose as a tiny lesion at the lenticular nucleus. Five patients had cysts within tumors. Five patients received partial, subtotal, to total resection. One patient had stereotactic biopsy of the tumors. Postoperative primary adjuvant therapies included radiotherapy, chemotherapy alone, and combined chemotherapy and radiotherapy. Five patients survived, and one patient died of radiation-induced sarcoma with median follow-up period of 13.7 years. Local recurrence was observed in all of three patients after solitary postoperative chemotherapy. Conclusions The lenticular nucleus is a significant locus for germinomas and can be bilateral. Although rarely reported in Western countries, it does exist in Taiwan as well. Treatment of germinomas in this specific location is similar to germinoma in other intracranial locations.     

基底节生殖细胞瘤的临床和影像学分析

目的分析伴脑干萎缩的基底节生殖细胞瘤临床特点及影像学改变，以提高对此病的认识。方法结合文献分析7例基底节生殖细胞瘤患者的临床和影像学特征。结果7例患者均为年轻男性，主要表现为缓慢进展的轻偏瘫；影像学显示肿瘤主要位于基底节，MRIT。加权像上呈低或等低混杂信号，T2加权像上呈高或等高混杂信号，伴有同侧大脑半球或脑干萎缩。结论基底节生殖细胞瘤临床及影像学缺乏特异性；年轻患者出现缓慢进行性偏瘫，伴有病变同侧大脑偏侧萎缩或／和大脑脚萎缩的基底节病变应高度怀疑基底节生殖细胞瘤。     

丘脑基底节区高血压脑出血的显微外科治疗及预后相关因素分析

目的分析两种手术策略(小骨窗开颅血肿清除术和开颅血肿清除并去骨瓣减压术)治疗丘脑基底节区高血压脑出血的效果,并探讨预后相关因素。方法回顾性分析经显微外科手术治疗的132例丘脑基底节区高血压脑出血患者的临床资料,统计患者的年龄、血肿量、中线移位程度、手术时机、手术方式、术前GCS评分,及术后24小时GCS评分,并对存活患者术后3个月的日常生活能力进行了随访,采用Logistic多元回归分析影响预后的相关因素。结果统计分析显示血肿量、血肿破入脑室、中线移位大于10mm、术前GCS评分和手术时机5个变量均与预后相关,而与年龄、术后24 h的GCS评分以及手术方式无显著关系。结论小骨窗开颅血肿清除术和开颅血肿清除并去骨瓣减压术均是治疗丘脑基底节区高血压脑出血的有效方法,患者预后与血肿量、血肿是否破入脑室、中线移位程度、术前GCS评分和手术时机密切相关。     

基底节区腔隙性脑梗死患者认知功能障碍分析

目的分析基底节区腔隙性脑梗死(lacuna infarction,LI)患者认知功能障碍的临床特征。方法收集基底节区LI患者120例(根据病灶数量分为少发组和多发组),以头部MRI正常的82例健康体检老年人为对照组,以认知能力筛查测验(中文2.0版)(CASI C-2.0)和事件相关电位P300(ERPs P300)作为认知评估工具,观察LI患者与对照组认知功能的差异。结果少发组LI患者CASI总分与对照组无明显差异(P>0.05),但少发组CASI子项目"近期记忆"和"定向感"均低于对照组(P<0.05)。多发组LI患者不仅CASI总分明显低于对照组(P<0.05),而且多个CASI子项目("近期记忆"、"集中及心算力"、"定向感"、"抽象推理"及"空间慨念")评分均低于对照组(P<0.05)。而多发组患者"近期记忆"、"定向感"及"空间概念"等子项目的评分与对照组的差异尤为明显(P<0.01)。少发组ERPs P300潜伏期与对照组无明显差异(P>0.05),而多发组ERPs P300潜伏期明显长于对照组(P<0.05)。结论基底节区LI患者认知功能明显下降,其中近期记忆、定向感及空间慨念等认知能力损害尤为明显。     

The basal ganglia in extrapyramidal dysfunction

The rapid advances in knowledge of basal ganglia circuitry and function in recent years have allowed the construction of a functional scheme to explain many facets of known pathologic states. The dichotomy of Parkinson's disease; akinesia with increased tone, and the mirror effects in Huntington's disease; hemiballismus and tardive dyskinesia, hyperkinesia with decreased tone are explained as due to two outputs of the system with an intervening inhibitory neuron which reverses the sign. The two outputs control different motor functions; pallidothalamic involved primarily with movements and nigrobrainstem involved primarily with muscle tone.     

个体化显微手术清除基底节区出血

目的探讨基底节区脑出血的个体化治疗及显微操作技巧与细节,提高手术疗效。方法根据术前CT检查结果,对需手术治疗的52例基底节区脑出血进行详细的术前分析,制定个体化的手术入路,术中注意操作细节的把握。采用日常生活活动能力评分系统（ADL）评价患者预后。结果术后复查CT,绝大多数患者血肿清除满意,无一例发生再出血;根据ADL评分,良性预后率达80％以上。结论对于清除基底节区脑出血,个体化手术入路和良好的操作技巧至关重要。     

高血压性基底节区出血引流术后颅内感染的危险因素

目的 探讨高血压性基底节区出血引流术后发生颅内感染的危险因素。方法 回顾性分析2015年5月~2020年4月引流术治疗的481例高血压性基底节区出血的临床资料。结果 481例中,术后发生颅内感染28例,发生率为5.8%;治愈24例,治愈率85.7%。28例颅内感染中,培养出致病菌26例,其中革兰阳性菌16例（61.5%）,革兰阴性菌10例。多因素logistic回归分析显示年龄≥60岁、白蛋白水平<35 g/L、术前GCS评分≤8分、置管时间>3 d、鞘内注射>6次、引流管口漏是术后颅内感染的独立危险因素（P<0.05）。结论 高血压性脑出血引流术后颅内感染是受多种原因影响的,预防为主,防治结合,以降低术后颅内感染发生率及病死率。     

Germinoma of basal ganglia in female: case report and review of the literature

Introduction  Germinoma of basal ganglia in female is very rare. The authors present a case of germinoma located in the right temporal lobe and basal ganglia in female and raise a hypothesis for the sex disparity. Materials and methods  A 13-year-old Chinese girl complained of weakness in the left extremities and intermittent headache for 2 years. Neuroimaging revealed an irregular lesion in the temple lobe and basal ganglia. A temporal polectomy was performed for the tumor. Results and discussion  Histopathologic examination confirmed germinoma. The patient remains stable and tumor-free for 36 months after radiotherapy and chemotherapy. The authors’ hypothesis is that it is the individual hormone balance which leads to the male predominance of germinoma in the basal ganglia.