Effect of interictal epileptiform discharges on EEG-based functional connectivity networks

ObjectiveFunctional connectivity networks (FCNs) based on interictal electroencephalography (EEG) can identify pathological brain networks associated with epilepsy. FCNs are altered by interictal epileptiform discharges (IEDs), but it is unknown whether this is due to the morphology of the IED or the underlying pathological activity. Therefore, we characterized the impact of IEDs on the FCN through simulations and EEG analysis.MethodsWe introduced simulated IEDs to sleep EEG recordings of eight healthy controls and analyzed the effect of IED amplitude and rate on the FCN. We then generated FCNs based on epochs with and without IEDs and compared them to the analogous FCNs from eight subjects with infantile spasms (IS), based on 1340 visually marked IEDs. Differences in network structure and strength were assessed.ResultsIEDs in IS subjects caused increased connectivity strength but no change in network structure. In controls, simulated IEDs with physiological amplitudes and rates did not alter network strength or structure.ConclusionsIncreases in connectivity strength in IS subjects are not artifacts caused by the interictal spike waveform and may be related to the underlying pathophysiology of IS.SignificanceDynamic changes in EEG-based FCNs during IEDs may be valuable for identification of pathological networks associated with epilepsy.     

Fas/FasL在各阶段婴幼儿血管瘤中的表达及意义

目的 检测Fas／FasL在各阶段婴幼儿血管瘤组织中的表达，探讨Fas／FasL在婴幼儿血管瘤细胞凋亡中的作用。方法 应用EnVision法免疫组化染色和RT-PCR检测Fas／FasL蛋白及mRNA在各阶段血管瘤组织中的表达。结果 ①增生早期和增生中期，部分血管瘤细胞表达Fas；增生晚期，阳性细胞明显增多，Fas mRNA表达最强；消退早期，仍有少量微血管内皮细胞表达Fas，之后Fas表达迅速减弱。②最早期细胞团中没有FasL（＋）细胞；增生中期，血管瘤组织中出现少量FasL（＋）细胞；增生晚期FasL（＋）细胞显著增多，FasL mRNA表达最强；消退早期之后，FasL（＋）细胞迅速减少以至消失。结论 Fas／FasL与婴幼儿血管瘤演变过程有密切联系，Fas／FasL介导的血管瘤细胞凋亡可能是婴幼儿血管瘤自行消退的重要原因。     

Eating one's words: Part III. Mentalisation‐based psychotherapy for anorexia nervosa—an outline for a treatment and training manual

This paper presents a new outline for psychotherapy with personsn with anorexia nervosa. ‘Model on mentalisation’ is the intellectual and empirical framework for this contribution. Mentalisation is defined as the ability to understand feelings, cognitions, intentions and meaning in oneself and in others. The capacity to understand oneself and others is a key determinant of self‐organisation and affect regulation, and is acquired in early attachment relationships. Impaired mentalisation is documented and described as a central psychopathological feature in anorexia nervosa. Psychotherapeutic enterprise with individuals with compromised mentalising capacity should be an activity that is specifically focused on the rehabilitation of this function, with special emphasis on how the body is representing mental states. The paper describes psychotherapeutic goals, stances and techniques. It is intended that this outline will be further developed into manuals as a basis for therapy, training and research. Copyright © 2007 John Wiley & Sons, Ltd and Eating Disorders Association.     

Second‐degree atrioventricular block (Mobitz Type I) in an adolescent with anorexia nervosa: Intrinsic or acquired conduction abnormality

Anorexia nervosa (AN) can cause both functional and structural cardiac complications, including a variety of different conduction abnormalities. This is the first case report of symptomatic diurnal second‐degree atrioventricular (AV) block (Mobitz Type I) in an adolescent with AN. We present a 12‐year‐old girl with AN, restrictor sub‐type who reported cardiac symptoms during weight gain, at the time of the initial diagnosis of AV block. Second‐degree AV block (Mobitz Type I) is discussed as a possible complication of the AN, as well as being an intrinsic conduction system disease. © 2009 by Wiley Periodicals, Inc. Int J Eat Disord 2009     

Nephrotic syndrome in a mother and her infant: relationship with cytomegalovirus infection

This case report describes infantile nephrotic syndrome (NS) in a baby girl with a clinically severe cytomegalovirus (CMV) infection. Culture of the baby's urine was positive for CMV and IgM anti-CMV antibodies were detected. After an unsuccessful course of corticosteroids, gancyclovir treatment was started and a remission of cutaneous, pulmonary, and renal symptoms was achieved. As the mother also developed NS at the end of pregnancy, a common etiology could be postulated, although there were no signs of recent CMV infection in the mother, only anti-CMV IgG. The relationship between CMV infection and glomerular disease is still unclear: NS may represent another manifestation of CMV disease.     

Morphological study of neocortical areas in Rett syndrome

Various neocortical areas from four females aged 16–24 years with Rett syndrome (RS) were investigated and compared with brains of therapy-resistant partial epilepsy (TRPE) patients (18–25 years), infantile autism (IA), and control brains (24 and 58 years). The cytoarchitecture of area 10 (frontal), area 21 (temporal), area 4 (primary motor cortex), and area 17 (primary visual cortex) was studied by the combined Klüver-Barrera (luxol fast blue and cresyl violet) standard procedure. Autofluorescence of lipofuscin, immunofluorescence of synaptic vesicle proteins [synaptophysin (p38)] and lectin-stained (Wisteria floribunda agglutinin) perineuronal nets (PNs) were studied in the cortices using dual-channel confocal laser scanning microscopy. The brains of RS females show various types of morphological/cytoarchitectonical abnormalities of single pyramidal neurons in layers II–III, and V–VII of different cortical areas. The abnormalities include mild losses of pyramidal neurons, more pronounced in layers II and III than in layers V and VII, and more evident in frontal and temporal areas than in the visual cortex. Microdysgenesis, including abnormalities due to neuronal migration disorders, was not found in RS, in contrast to the observations in TRPE patients, strongly indicating that RS is not a neuronal migration disorder. Lipofuscin distribution was normal but amounts were lower in RS cases than in control and TRPE brains. PNs were less expressed in cortices of the IA case, but were clearly overexpressed in the motor cortex of RS. Quantitative analysis of p38 showed a decrease in the area occupied by p38 immunoreactivity by 20–40% in RS compared with controls. It is concluded that RS could best be explained by a postnatal synaptogenic developmental deficiency; the basic defect, however, is still completely unknown. Received: 26 February 1996 / Revised, accepted: 11 July 1996     

女性飞行学员高空飞行生理生化耐受能力研究

为从高空缺氧耐力训练前后生理、生化角度探讨中国女飞行员驾驶战斗机的可行性,在地面模拟高空缺氧及高空飞行训练前后行心电图、心率、血氧饱和度、血压、血尿酸(BUA)、肌酸激酶(CK)、血红蛋白(Hb)、乳酸(BLA)、肌酐(Cr)和天冬氨酸转氨酶(AST)测定,并与男飞行学员的测定结果比较.发现男女飞行学员的心电图、心率、血氧饱和度、血压、Cr、CK、BUA和AST均无显著性差异;女飞行学员在高空耐缺氧Hb代偿反应高于男飞行学员(P<0.05),而BLA水平低于男飞行学员(P<0.05).认为中国女飞行学员在生理生化耐受能力上能胜任飞战斗机.     

Following Catastrophic Epilepsy Patients from Childhood to Adulthood

Summary:  As patients with catastrophic epilepsies move from childhood to adulthood, evolving and innovative therapeutic regimens are often required. However, the goal of providing the best quality of life while minimizing both seizures and side effects remains the same. Clinicians can develop appropriate care plans by being aware of patients' changing needs. Clinical symptoms of the catastrophic epilepsies may change over time; by understanding the natural history of a patient's condition, clinicians can help ease the transition from childhood to adulthood. Additionally, as children with catastrophic epilepsies become adults, medical issues (e.g., medication side effects, tolerance, and dependence) and nonmedical issues (e.g., guardian/caretaker issue, group home applications, and respite care options) must be considered when developing strategies for patient care. Regular assessment of patients, the development of emergency plans, and maintenance of consistency in the delivery of care are also important issues to consider. Finally, a multidisciplinary care plan that incorporates resources from health-care practitioners, social service professionals, and community agencies can be valuable in optimizing treatment for patients with catastrophic epilepsies.     

Infantile chronic tubulo-interstitial nephritis with cortical microcysts: variant of nephronophthisis or new disease entity?

Over a 15-year period we observed seven children (four girls, three boys) who presented within the first months of life with severe renal failure and acidosis, associated with hypertension in five patients and polyuria in four. In addition, one patient had a severe cholestatic liver disease. In two families, a similarly affected sibling had died previously. Four patients were referred with the clinical diagnosis of polycystic kidney disease because of moderate enlargement of kidneys, but renal imaging (intravenous pyelography and ultrasonography) did not confirm this diagnosis. A renal biopsy, performed in all patients, showed similar features characterized by a diffuse chronic tubulo-interstitial nephritis (TIN) and particularly by the presence of microcystic dilatation of proximal tubules and Bowman's space. Liver pathology was normal in two patients, including one with hepatomegaly. However, in the patient with cholestasis there was inflammatory portal fibrosis with mild duct proliferation. Progression of the renal disease was extremely rapid and all patients reached end-stage renal failure (ESRF) before the age of 2 years (11–22 months). Two children had successful renal transplants. Although this chronic TIN shares some features with nephronophthisis, we suggest that it represents a distinct entity both on clinical and morphological grounds. The specific clinical features of this disease are its early onset and rapid progression to ESRF. Pathologically, it differs from nephronophthisis by the absence of medullary cysts and thickened tubular basement membranes and by the presence of cortical microcysts.