Occlusive cerebrovascular disease in young adults

13 patients under 40 years of age had cerebral infarction and angiographic evidence of arterial stenosis or occlusion. None of them had cardiac disorders prone to cerebral embolism. Five patients had occlusion at the origin of the internal carotid artery, one had occlusion of the common carotid artery and one each had stenosis at the origin of the internal carotid and common carotid arteries, respectively. Two patients had unilateral occlusion of the supraclinoid portion of the carotid artery with basal collaterals that had some resemblance to the Moyamoya disease. Three patients demonstrated stenosis or occlusion of the middle and/or anterior cerebral arteries. Three patients had hypercholesterolemia, one of whom was hypertensive. None had confirmed diabetes mellitus. One female, who died, had taken oral contraceptives for 3 years. A male, with internal carotid artery occlusion, had serological evidence for syphilis.     

不典型眼肌型重症肌无力的临床特点

目的研究不典型眼肌型重症肌无力(OMG)的临床特点。方法回顾性分析29例不典型OMG患者的临床资料。结果本组患者表现为上眼睑下垂3例,单眼1条眼外肌瘫痪15例,双眼3条眼外肌瘫痪1例,单眼全部眼外肌瘫痪1例,辐辏障碍2例,单眼眼轮匝肌瘫痪1例,类似眼病表现6例(复视4例、眼部不适和视物模糊2例,均无眼外肌瘫痪);有晨轻暮重表现12例(41.4%)。新斯的明试验阳性26例(89.7%),低频重复神经电刺激阳性8例(27.6%),单纤维肌电图(SFEMG)异常23例(79.3%)。患者早期均被误诊。经泼尼松和/或胆碱酯酶抑制剂治疗,症状消失26例(89.7%),显著改善3例(10.3%)。结论不典型OMG患者的临床特点为受累眼肌少,症状局限,表现晨轻暮重的比率低。新斯的明试验和SFEMG检查可确诊。     

Miller Fisher综合征14例报道

目的:分析14例Miller Fisher综合征的临床特点和预后。方法:回顾性分析1998年1月至2007年3月我院收治的14例Miller Fisher综合征患者,应用Microsoft Access 2003建立数据库,分析患者的各种症状、体征和各项检查和预后。结果:病前感染者11例,其中肠道感染8例,双侧动眼神经损害14例,眼内肌的损害7例,对光反射消失5例,共济失调9例,头晕7例,腱反射减低12例,肌力减退7例,周围神经损害5例,病理征3例,蛋白-细胞分离12例,影像学异常2例,肌电图改变7例。结论:Miller Fisher综合征临床症状复杂,可在经典的三联征基础上伴有其他体征,也可仅有其中的两联征,预后较好。     

Dysphagia in olivopontocerebellar atrophy

Clinical, radiological and manometric studies on thirteen patients with olivopontocerebellar atrophy were performed in order to investigate the characteristics of dysphagia. As a clinical study, a detailed history of dysphagia was taken to distinguish two types of dysphagia, that is to say swallowing disturbance in a narrow sense and passage disturbance. In the radiological study, each phase of swallowing was observed by X-rays with contrast medium (Dionosil). In the manometric study, intraluminal resting pressure in the esophagus and pressure of esophageal contraction after swallowing were measured. The results were as follows: Eight patients had the sensation of swallowing disturbance in a narrow sense and five patients has the feeling of passage disturbance. In X-ray studies four patients had pooling in piriformis sinus and six patients had slight dilatation of the lower esophagus. In manometric studies, six patients had low intraluminal resting pressure of the upper esophageal sphincter, but almost all patients had normal intraluminal resting pressure throughout the esophagus and in the lower esophageal sphincter. Two patients, who had suffered for five and seven years, had loss of both negative and positive wave in the upper esophageal sphincter after swallowing. Another three patients, who had suffered for two, six and seven years, respectively had loss of negative wave in the upper esophageal sphincter after swallowing. Regarding peristaltic wave, eight patients had low amplitude of the wave. Two patients, who had suffered for five and six years, had diphasic shape of the wave. One patient, who had suffered for nine years, had synchronous wave. Nine patients had loss of negative wave in the lower esophageal sphincter.(ABSTRACT TRUNCATED AT 250 WORDS)     

Electroencephalographic abnormalities in children with congenital heart disease

Ninety-eight consecutive patients with clinically suspected congenital heart disease were prospectively studied with electroencephalographic (EEG) recordings before cardiac catheterization. Twenty-five patients had abnormal EEGs. Fifty-five patients had acyanotic heart disease and normal neurologic examination results, of whom 15 had abnormal EEGs. Thirteen had spikes or spike and wave discharges and two had mildly abnormal EEGs. Twenty-seven patients had cyanotic heart disease and normal neurologic examination results, of whom four had abnormal EEGs. Seven patients (8%) had abnormal neurologic examination results, of whom four had abnormal EEGs. There was a higher incidence of seizures with increasing age. Based on their medical history two children with acyanotic heart disease had had seizures without EEG abnormalities. Five children with normal catheterization findings and four children with Down's syndrome (two with abnormal EEGs) were excluded from the total of 98. These findings suggest that subclinical nervous system involvement may occur in congenital heart disease.     

70例脑动脉夹层患者临床特点分析

目的分析脑动脉夹层住院患者的临床特点,促进对脑动脉夹层的早期识别、早期诊断和治疗。方法回顾性分析首都医科大学附属北京天坛医院神经内科及神经介入科2014年12月~2015年4月经全脑血管造影或高分辨磁共振成像诊断的脑动脉夹层患者的临床特点、影像学特点及治疗措施的情况。结果共纳入70例脑动脉夹层患者。平均年龄(50.0±12.8)岁,男性52例(74%),女性18例(26%)。颈动脉颅外段夹层21例(30%),椎动脉段夹层30例(43%),基底动脉夹层15例(21%),大脑中动脉水平段夹层1例(1%),侧裂段夹层1例(1%)。大脑后动脉及锁骨下动脉夹层各1例(1%)。非蛛网膜下腔出血性头痛19例(27%),头晕3例(4%),颈痛11例(16%),缺血性卒中16例(23%),蛛网膜下腔出血5例(7%),短暂性脑缺血发作4例(6%),其中3例经检查后发现缺血性卒中,无症状者19例(27%)。1例同时存在头痛和颈痛。3例患者同时存在缺血性卒中和头痛,1例患者同时存在颈痛、短暂性脑缺血发作和缺血性卒中。动脉夹层的影像学特征主要表现为动脉局部瘤样扩张、内膜瓣、动脉壁内新月形高信号征等。接受抗栓治疗者9例(13%),单纯抗凝治疗2例(3%),支架辅助弹簧圈填塞17例(24%),单纯弹簧圈填塞5例(7%),单纯支架治疗11例(16%),抗凝结合支架治疗者1例(1%),没有治疗者23例(33%),死亡2例(3%)。结论脑动脉夹层的患者临床症状表现形式不同,轻症者可无症状,或单纯头颈痛,重症者可表现为短暂性脑缺血发作、缺血性卒中或蛛网膜下腔出血,正确诊断和相应的治疗十分重要。     

Temporal lobe epilepsy: analysis of patients with dual pathology

OBJECTIVES: To determine the frequency and types of dual pathology in patients with temporal lobe epilepsy (TLE) and to analyze the clinical manifestations and surgical outcome. MATERIAL AND METHODS: A total of 240 patients with TLE underwent temporal resections following a comprehensive pre-surgical evaluation. Thirty-seven (15.4%) of these had hippocampal sclerosis (HS) or temporal lobe gliosis in association with another lesion (dual pathology). RESULTS: Eighteen of 37 patients with dual pathology had heterotopia of the temporal lobe, nine had cortical dysplasia, four had cavernous angiomas or arteriovenous malformations, one had a dysembryoplastic neuroepithelial tumor, one had a contusion and four patients had cerebral infarctions in childhood. 68.5% had abnormal head magnetic resonance imagings, 91.3% had abnormal positron emission tomography scans, and 96% had abnormal ictal SPECT. The intracarotid amobarbital procedure (IAP) showed impaired memory of the epileptogenic side in 72% of the patients. Twenty patients had left and 17 had right-sided en bloc temporal resections, including the lesion and mesial temporal structures. Twenty-six (70.2%) became seizure-free, eight (21.6%) had rare seizures, two (5.4%) had worthwhile seizure reduction and one (2.7%) had no improvement (range of follow-up 1-16 years, mean = 7.4 years). CONCLUSIONS: 15.4% had dual pathology. The dual pathology was almost exclusively seen in patients whose lesions were congenital, or occurred early in life, suggesting that the hippocampus is more vulnerable and more readily develops HS in early childhood. Resections, including the lateral and mesial temporal structures led to a favorable outcome with no mortality and little morbidity.     

Ten-year follow-up of drug abusers with acute or chronic psychosis

To assess whether long-term outcome of former drug abuse patients who had psychotic symptoms was related to duration of psychosis before hospitalization, the authors rated the psychiatric, occupational, and residential outcome of 101 former drug abuse patients based on interviews conducted ten years after their admission to a psychiatric hospital. At admission, 45 had had psychotic symptoms for six months or more; 26 had had psychotic symptoms for less than six months; and 30 had not been psychotic. Seventy-one former psychiatric patients who had had a psychotic illness but had not abused drugs were also interviewed and rated. Drug abusers with chronic psychosis had significantly worse psychiatric outcome than the other drug abusers and significantly worse occupational outcome than acutely psychotic drug abusers. The authors conclude that drug abusers with chronic psychotic symptoms are a distinct subgroup of drug abusers who suffer from major psychotic illness.     

Forty-year follow-up of United States prisoners of war

The authors performed structured psychiatric examinations of 188 former prisoners of war (POWs). Sixty-seven percent had had posttraumatic stress disorder. Of those affected, 29% had fully recovered, 39% still reported mild symptoms, 24% had improved but had moderate residual symptoms, and 8% had had no recovery or had deteriorated. Presence of posttraumatic stress disorder was not significantly correlated with other mental disorders. Delayed onset was not seen. The findings confirm the DSM-III concept of and criteria for posttraumatic stress disorder.     

微栓子信号的发生率及其临床意义

目的探讨常规接受微栓子监测检查患者的微栓子信号发生率以及其相关临床特征。方法连续纳入自2016年7月至2018年12月接受常规30 min微栓子监测以及常规TCD检查中探及到微栓子信号的患者,收集基线信息。结果共5 886例患者进行了30 min微栓子监测检查,其中50例存在微栓子信号,阳性率0.85%。大脑中动脉微栓子信号的出现率最高,达84%。46%(23/50)微栓子来源于动脉,包括8例动脉粥样硬化性脑动脉或颈动脉狭窄、10例颅内动脉、4例烟雾病和1例ANCA相关性动脉炎; 30%(15/50)提示心脏来源的微栓子,包括1例心房颤动、4例人造瓣膜和10例卵圆孔未闭;其他原因微栓子来源占24%(12/50),包括8例从右到左分流和4例未知来源。微栓子阳性的患者脑白质病变及认知障碍发生率高,分别为72%及68%。结论微栓子信号较罕见,不同的疾病微栓子信号的临床意义及机制可能不一样。     

Neurologic and neurobehavioral sequelae in children with human immunodeficiency virus (HIV-1) infection

The range and extent of neurologic and neurobehavioral complications of human immunodeficiency virus (HIV-1) infection in children are under-described. Seventy-eight children with HIV-1 infection (32 females) were assessed for neurologic complications. Forty-six children had abnormal neurology examinations. Thirty-three children had global pyramidal tract signs, 5 had a hemiparesis, 4 had peripheral neuropathy, 18 had visual impairment, and 5 had hearing impairment. Thirty-nine of 63 children over 1 year of age had neurobehavioral problems. Of 24 children with HIV encephalopathy, 74% had severe immunosuppression and 45% were not receiving antiretroviral therapy. Twelve children had prior opportunistic central nervous system infections, and 9 had epilepsy. Diverse neurologic and neurobehavioral deficits are common in children with HIV-1 infection. Children with severe immunosuppression, who were not receiving antiretroviral therapy, were growth impaired and less than 1 year of age, were at greatest risk for developing neurologic complications.     

Clinical application of neuroimaging in epilepsy

OBJECTIVE: To evaluate the use of neuroimaging in clinical practice and to assess the prevalence of detected structural abnormalities in epilepsy patients in a clinical set up. METHODS: 919 outpatients were identified and the scan results reviewed. A total of 677 patients had chronic active epilepsy (88 had idiopathic generalised epilepsy (IGE), 588 had localisation related epilepsy, one had symptomatic generalised epilepsy), 57 had a single epileptic seizure, 46 were in remission, and 139 had non-epileptic attacks. RESULTS: 391 patients had no scan (53 patients in this group had IGE, 182 had localisation related epilepsy, one had generalised symptomatic epilepsy, 18 had single epileptic attacks, 21 were in remission, 116 had non-epileptic attacks). Altogether 528 patients had a scan, the results were not available in 33, 163 had x ray computed tomography (CT) only, 178 had standard magnetic resonance imaging (MRI) (slice thickness 5 mm), and 154 had high resolution MRI (including a T1 weighted sequence with 1.5 mm thick slices). Some 252 of 495 scans (51%) were abnormal. Abnormalities were hippocampal sclerosis (n=128), atrophy or non-specific white matter lesions (n=35), vascular abnormalities (n=27), tumours (n=25), brain damage (n=24), malformations of cortical development (n=13). Excluding atrophy and non-specific white matter lesions the prevalence of detected abnormalities was 54% in localisation related epilepsy, 18% in single seizure patients, 16% in epilepsy in remission, and 0% in IGE and non-epileptic attacks. CONCLUSIONS: Abnormalities were detected in more than half of all patients with localisation related epilepsy, and in about one in five patients with single seizures or epilepsy in remission. Many patients had no scan or only CT or standard MRI. The true prevalence of structural abnormalities may be have been higher. Scanning did not add any information in patients with IGE or non-epileptic attacks.     

Diphenylhydantoin and pulmonary function.

Thirty-nine epileptic patients underwent pulmonary function testing. Twenty-one of these patients, ranging in age from 16 to 44 years, had taken diphenylhydantoin (DPH) for 2 to 17 years. Eighteen patients, who had taken other anticonvulsants for similar time periods served as controls. Five patients in the DPH group had lung volume abnormalities, four had abnormal airway function, and five had abnormalities of alveolar gas mixing. One patient in the control group had lung volume abnormalities, two had abnormal airway function, and five had abnormalities of alveolar gas mixing. Statistical analysis revealed no significant differences between the groups, or between either group and predicted values.     

Radiologic evaluation of the mediastinum in myasthenia gravis

We reviewed the records of 36 patients with myasthenia gravis who had CT of the anterior mediastinum before thymectomy in an effort to improve preoperative detection of thymoma. At surgery, 10 patients had thymomas, 21 had germinal center follicular hyperplasia, and 5 had histologically normal glands. CT performed on an EMI 5005 was abnormal in 23 of 36 patients; 10 had thymoma, 11 had hyperplasia, 1 had a normal gland, and 1 had a cyst. Tumors were detected by routine chest x-ray in 5 of 10 patients and by linear tomography in 7 of 8. All four patients with calcified masses on CT had thymomas (including the patient with normal linear tomograms). CT alone is too sensitive a screening test; 12 of 25 patients with hyperplasia or normal glands had abnormal scans. However, in combination with chest x-ray and linear tomography, we detected 10 of 10 patients with thymoma with reasonable certainty.     

Prognosis of optic neuritis with special reference to cerebrospinal fluid immunoglobuhs and measles virus antibodes

Forty-eight patients with optic neuritis (ON), first seen in 1970 to 1973, were neurologically and neuroophthal mologically reexamined after 7 to 10 years. Twenty-seven patients (56%) had probable MS, and 9 (19%) had possible MS. During the attack of ON, the cerebrospinal fluid (CSF) samples and serum/CSF measles antibody ratios were studied. Twenty patients had increased relative immunoglobulin G (IgG % of total protein) in their CSF; 19 of these had probable or possible MS. However, 17 of 28 patients with a normal relative IgG value had also developed MS. CSF electrophoresis was abnormal in 20 patients with ON; reexamination showed that 19 had probable or possible MS. Sixteen of 27 patients with normal electrophoresis had also developed MS. Serum/CSF measles antibody ratio had decreased in 19 patients; 13 of these had probable MS and 3 had possible MS. Of 29 patients with a normal measles antibody ratio, 14 had probable MS and 6 had possible MS. The conclusion is that examination of the CSF in ON gives valuable prognostic information because increased relative IgG, abnormal electrophoresis, or a decreased measles antibody ratio implies a high risk of developing MS. A normal CSF does not, however, rule out the possibility of dissemination.     

Relationship of childhood trauma to age of first suicide attempt and number of attempts in substance dependent patients

OBJECTIVE: To examine whether childhood trauma effect the age of first attempting suicide and the number of attempts. METHOD: One thousand twelve hundred and eighty substance dependent patients were interviewed about whether or not they had ever attempted suicide, the age of first attempt and the number of attempts. Patients completed the Childhood Trauma Questionnaire - 34 item version. RESULTS: Five hundred and thirty-eight patients (42%) had attempted suicide. Significantly more of the patients who had attempted suicide were female. Patients who had made three or more attempts had significantly higher childhood trauma scores than patients who had made two attempts, who had higher scores than patients who had made one attempt, who had higher scores than patients who had never attempted. Patients who first attempted suicide before the age of 20 years had significantly higher childhood trauma scores than patients who first attempted after 20 years of age. CONCLUSION: Childhood trauma may be a determinant of the age of onset of suicidal behavior and of the number of suicide attempts.     

The use of DSM-III axis III in recording physical illness in psychiatric patients

The randomly selected charts of 50 discharged psychiatric inpatients were reviewed for documentation of medical illness and DSM-III axis III diagnoses. Twenty-eight percent of the patients had had medical symptoms, 56% had had medical findings, 36% had had laboratory findings, and 60% had been given axis III diagnoses. In at least seven cases, the medical findings were poorly reflected in the final axis III diagnosis. In no case did the record indicate that medical factors were viewed as the cause of a patient's immediate psychiatric syndrome.     

The structural basis of moderate disability after traumatic brain damage

The objective was to discover the nature of brain damage in survivors of head injury who are left with moderate disability. Macroscopic and microscopic examination was carried out on the brains of 20 persons who had died long after a head injury that had been treated in a neurosurgical unit. All had become independent but had various disabilities (moderate disability on the Glasgow outcome scale) Most deaths had been sudden, which had led to their referral from forensic pathologists. Post-traumatic epilepsy was a feature in 75%. An intracranial haematoma had been evacuated in 75%, and in 11 of the 15 with epilepsy. Diffuse axonal injury was found in six patients, five of the mildest type (grade 1) and one of grade 2. No patient had diffuse thalamic damage but one had a small focal ischaemic lesion in the thalamus. No patient had severe ischaemic brain damage, but three had moderate lesions which were bilateral in only one. No patient had severe cortical contusions. In conclusion, the dominant lesion was focal damage from an evacuated intracranial haematoma. Severe diffuse damage was not found, with diffuse axonal injury only mild and thalamic damage in only one patient.     

Progressive supranuclear palsy and a multi-infarct state

In 58 patients with progressive supranuclear palsy (PSP), 19 (32.8%) had CT, MRI, or autopsy evidence of a multi-infarct (MI) state. The clinical findings in the infarct syndrome were similar to idiopathic PSP. Five MI-PSP patients had had a stroke, four had focal dystonia, two had hemiparesis, and one had an intention tremor of recent onset. In contrast, only 5.9% (12.9% of those with CT or MRI) of 426 Parkinson's disease patients had evidence of strokes. One case of PSP studied pathologically was attributed to cerebral amyloid angiopathy.     

THE OFFSPRING OF PEOPLE WITH CEREBRAL PALSY

To test a hypothesis that a placental deficit as a possible cause of CP might be transmitted, 88 people with congenital CP were contacted by questionnaire and letters. They had had 122 children, 93 per cent of whom were reported to be normal. The frequency of miscarriages and toxaemia was normal. 5 per cent of the mothers had had emergency caesarean sections. Three mothers also had minor malformations. The mean gestational age of the children was 38.8 weeks and their mean birthweight was 3295g. The mean birthweight of the mothers born preterm was 984g and that of their babies was 3244g. Two of the 122 children had diplegia, four had malformations, one had trisomy 18 and there was one stillbirth.