Connectivity measures suggest a sub-cortical generator of myoclonus in Angelman syndrome

ObjectiveThe clinical and neurophysiological characteristics of myoclonus in Angelman syndrome (AS) have been evaluated in single case or small cohorts, with contrasting results. We evaluated the features of myoclonus in a wide cohort of AS patients.MethodsWe performed polygraphic EEG-EMG recording in 24 patients with genetically confirmed AS and myoclonus. Neurophysiological investigations included jerk-locked back-averaging (JLBA), cortico-muscular coherence (CMC) and generalised partial directed coherence (GPDC). CMC and GPDC analyses were compared to those obtained from 10 healthy controls (HC).ResultsTwenty-four patients (aged 3–35 years, median 20) were evaluated. Sequences of quasi-continuous rhythmic jerks mostly occurred at alpha frequency or just below (mean 8.4 ± 1.4 Hz), without EEG correlate. JLBA did not show any clear transient preceding the jerks. CMC showed bilateral over-threshold CMC in alpha band that was prominent on the contralateral hemisphere in the patient group as compared to HC group. GPDC showed a significantly higher alpha outflow from both hemispheres toward activated muscles in the patient group, and a significantly higher beta outflow from contralateral hemisphere in the HC group.ConclusionsThese neurophysiological findings suggest a subcortical generator of myoclonus in AS.SignificanceMyoclonus in AS has not a cortical origin as previously hypothesised.     

Precise and limited decompression for lumbar spinal stenosis

Summary Fifty-eight consecutive patients with lumbosacral nerve root entrapment due to spinal stenosis were treated with modified microsurgical decompression. Only the clinically relevant sides and levels were decompressed while the spinous processes, the interspinous ligaments, the medial portion of ligamentum flavum and the functionally important parts of the facet joints were preserved. The reviewers rated recovery as good or excellent in 71% of patients while patient self-assessment indicated 76% good or excellent outcome. These data suggest that microsurgical decompression of spondyloarthritic changes can effectively relieve the signs and symptoms of nerve root compression and that with careful evaluation of all available data the number of nerve roots requiring decomperession is often fewer than what is suggested by diagnositic images alone.     

Acute encephalopathy associated with influenza and other viral infections

Acute encephalopathy is the most serious complication of pediatric viral infections, such as influenza and exanthem subitum. It occurs worldwide, but is most prevalent in East Asia, and every year several hundreds of Japanese children are affected by influenza-associated encephalopathy. Mortality has recently declined, but is still high. Many survivors are left with motor and intellectual disabilities, and some with epilepsy. This article reviews various syndromes of acute encephalopathy by classifying them into three major categories. The first group caused by metabolic derangement consists of various inherited metabolic disorders and the classical Reye syndrome. Salicylate is a risk factor of the latter condition. The second group, characterized by a systemic cytokine storm and vasogenic brain edema, includes Reye-like syndrome, hemorrhagic shock and encephalopathy syndrome, and acute necrotizing encephalopathy. Non-steroidal anti-inflammatory drugs, such as diclofenac sodium and mephenamic acid, may aggravate these syndromes. Severe cases are complicated by multiple organ failure and disseminated intravascular coagulation. Mortality is high, although methylprednisolone pulse therapy may be beneficial in some cases. The third group, characterized by localized edema of the cerebral cortex, has recently been termed acute encephalopathy with febrile convulsive status epilepticus, and includes hemiconvulsion-hemiplegia syndrome and acute infantile encephalopathy predominantly affecting the frontal lobes. Theophylline is a risk factor of these syndromes. The pathogenesis is yet to be clarified, but an increasing body of evidence points to excitotoxicity and delayed neuronal death.     

扩大的椎板间开窗治疗腰椎间盘突出伴侧隐窝狭窄（附140例报告）

本组采用的扩大的椎板开窗术治疗腰椎间盘突出合并侧隐窝狭窄140例。结果全部优良，与适应症严格选择和手术损伤小有关。强调切除卡压神经根的侧隐窝后壁外侧，即上关节突的冠状面．不失其脊椎三柱系统的三角结构。俯卧位头低足高，可减少硬膜囊张力避免负损伤。切除棘突下椎板时，用神经拉勾代替神经剥离器易于分离黄韧带，用90度的椎板咬骨钳斜向棘突方向，可满意地切除该部份椎板黄韧带，并可刮切椎板内层。神经根周围滴入醋酸炎舒松A2-3ml，术后疼痛锐减。减压窗口置盖明胶海绵有其争议，作者实践体会放置害处不大，要求病人有“护腰”意识。     

额叶内侧面损伤的临床特点及救治

目的 总结17例额叶内侧面损伤的临床特点及救治经验。方法 对我科1999年7月至2003年4月收治的额叶内侧面损伤患者进行回顾性分析。结果 手术治疗1例，恢复良好，保守治疗16例，其中恢复良好12例，中残2例，死亡2例，全组死亡率11．77％。结论 额叶内侧面损伤一般意识障碍较轻，但易直接发生枕骨大孔疝，严密观察病情及动态CT检查，颅内压监测，不拘泥传统手术指征，及时抓住手术时机，可有效降低致残率及死亡率。     

Outcome after traumatic frontal intracerebral haemorrhage: a comparison of unilateral and bilateral haematomas

Frontal intracerebral haemorrhage (ICH) is a common result of cranial trauma. Outcome differences between bilateral and unilateral frontal ICH are not well studied but would be valuable to predict prognosis in clinical practice. Two aims are proposed in this study: first to compare the risk of developing delayed ICH after bilateral or unilateral frontal ICH, and second to determine the variables helpful to predict outcome according to the Glasgow Outcome Scale (GOS). Between January 1993 and December 1997, 694 consecutive patients with traumatic ICH were admitted to the Chang Gung Medical Center within 24 h of the trauma. Patients with ICH in sites other than the frontal lobes were excluded. A total of 161 cases (mean age 46.3+/-20.3 years), including 57 bilateral (mean age 52.5+/-18.7 years) and 104 unilateral (mean age 42.9+/-20.5 years) traumatic frontal ICH were studied. Twenty-eight of 57 patients (49%) with bifrontal ICH versus 17 of 104 patients (16%) with unilateral frontal ICH had a further, delayed ICH. In 42 of 45 patients (93%) with delayed ICH, this occurred within 5 days of the initial trauma. Multivariate logistic regression was used to select significant predictors of outcome. We found that delayed ICH (p<0.001), age (p=0.004) and mechanism of injury (p=0.001) explained the worse outcome in patients with bifrontal ICH. The best-fitting logistic regression model included three variables: delayed ICH (p=0.011), initial GCS (p=0.023), and a sum score of clinical and radiological variables (p=0.003). Bifrontal ICH tended to occur in older patients after a fall and was associated with a higher risk of developing delayed ICH or brain stem compression compared to unilateral ICH damage. Using these three variables - delayed ICH, initial GCS, and the sum score - in a logistical regression model is useful to predict outcome in patients with traumatic frontal ICH and may aid patient management.     

Pineal atrophy and other neuroendocrine and circumventricular features of the naked mole-rat,heterocephalusglaber (Rüppell), a fossorial,equatorial rodent

Summary Quantitative aspects of the microanatomy of the pineal gland and other neuroendocrine and circumventricular structures were studied in a small, reproductively suppressed, female Naked Mole-rat from central Kenya, Africa. The atrophic pineal is the smallest in absolute size (0.002135 mm³) of any so far described in a species of rodent, and in size relative to body weight is second only to that of another tropical species. The subcommissural organ and posterior collicular recess are also relatively small and less well differentiated than those in most other examined rodent species. In contrast, the subfornical organ, OVLT and median eminence are large and well vascularized. It is concluded that the pineal in this species follows the previously described trend among rodents of relatively smaller size in species whose centers of distribution are in lower latitudes. Although the pineal is atrophic, the Naked Mole-rat still exhibits 24-hour and seasonally timed patterns of behavior and seasonal reproduction. However, in this species these events are probably cued by moisture, temperature and social factors rather than by photic information.     

Prefrontal cortex dysfunction and depression in atypical parkinsonian syndromes.

Depressive symptoms are common in patients with neurodegenerative disorders. Imaging studies suggest that a disruption of frontal-subcortical pathways may underlie depression associated with basal ganglia disease. This pilot study tested the hypothesis that frontal dysfunction contributes to depression associated with multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). Depressed patients with MSA (n = 11), PSP (n = 9), and age-matched controls (n = 25) underwent measures of cerebral glucose metabolism applying positron emission tomography with (18)F-fluorodeoxyglucose. Regional metabolism in the patient groups was compared to the normal subjects using the voxel-based statistical parametric mapping. Depressive symptom severity (Hamilton Depression Rating) and degree of locomotor disability (Hoehn & Yahr) were assessed in the patient groups. The association between prefrontal metabolism and the occurrence of depressive symptoms and the degree of locomotor disability was investigated. When compared to controls, MSA patients revealed significant metabolic decreases in bilateral frontal, parietal, and cerebellar cortex and in the left putamen. In PSP patients, significant hypometabolism was demonstrated in bilateral frontal cortex, right thalamus, and midbrain. Depression severity but not the patients' functional condition was significantly associated with dorsolateral prefrontal glucose metabolism in both patient groups. The findings of this pilot study support the hypothesis that depressive symptoms in MSA and PSP are associated with prefrontal dysfunction.     

开放手术观察腰椎间盘突出症溶核失败45例分析

目的 通过开放手术观察分析椎间盘髓核化学溶解术治疗腰椎间盘突出症失败原因。方法 收集溶核失败的腰椎间盘突出症45例行开放手术治疗。结果 术中见45例硬膜外脂肪完全消失，43例髓核未见溶解，2例髓核溶解呈糊状但未被吸收，21例伴有侧隐窝狭窄，15例突出物与神经根粘连，20例黄韧带增厚，2例椎管骨性狭窄，14例突出物钙化。结论 腰椎间盘突出症病变间隙合并有侧隐窝狭窄、神经根粘连、椎管狭窄、突出物钙化等，不是溶核治疗的适应证。     

螺旋CT对正常成人下腰椎侧隐窝的研究

目的:研究正常人下腰椎侧隐窝的形态和测量方法,探讨对侧隐窝狭窄的诊断?方法:采用尸体标本解剖与螺旋CT多层面容积重建和表面遮掩显示相结合方法对侧隐窝进行观察,选择椎体上1/4横断面作为研究平面,测量120例下腰椎双侧侧隐窝矢状径?椎管正中矢状径,计算椎管正中矢状径与双侧侧隐窝矢状径的比值,分析其相互关系?结果:下腰椎侧隐窝分三型:三角型?三叶草型和牛角型;正常成人双侧侧隐窝矢状径不对称,右侧略大于左侧(P<0.05),椎管正中矢状径与双侧侧隐窝矢状径的比率相对恒定(P>0.05),正中/右侧=3.93,正中/左侧=4.03?结论:椎体上1/4横断面为侧隐窝入口平面,利用矢状径比值的方法来判断侧隐窝狭窄具有科学性?