Childhood multiple sclerosis: a review

Multiple sclerosis (MS) is an autoimmune demyelinating disorder of the central nervous system (CNS) that is increasingly recognized as a disease that affects children. Similar to adult-onset MS, children present with visual and sensory complaints, as well as weakness, spasticity, and ataxia. A lumbar puncture can be helpful in diagnosing MS when CSF immunoglobulins and oligoclonal bands are present. White matter demyelinating lesions on MRI are required for the diagnosis; however, children typically have fewer lesions than adults. Many criteria have been proposed to diagnose MS that have been applied to children, mostly above 10 years of age. The recent revisions to the McDonald criteria allow for earlier diagnosis, such as after a clinically isolated event. However, children are more likely than adults to have monosymptomatic illnesses. None of the approved disease-modifying therapies used in adult-onset MS have been approved for pediatrics; however, a few studies have verified their safety and tolerability in children. Although children and adults with MS have similar neurological symptoms, laboratory (cerebrospinal fluid) data, and neuroimaging findings, the clinical course, pathogenesis, and treatment of childhood onset MS require further investigation.     

An Unusual Case of Motor and Vocal Tics With Obsessive-Compulsive Symptoms in a Young Adult With Behcet's Syndrome

Behcet's disease is a chronic relapsing-remitting, multi-system inflammatory disorder of unknown etiology, characterized by recurrent oral and genital ulcers, uveitis, and skin lesions. The most common central nervous system findings are demyelization and perivascular cell infiltration; meningoencephalitis, cerebral atrophy, and vascular thrombosis may also occur. Neuropsychiatric symptoms may include: paroxysmal dystonia, parkinsonism, delirium, hallucinations, and behavioral disturbances. We report an unusual case of explosive onset of motor and vocal tics with obsessive-compulsive symptoms in a 22-year-old male with Behcet's disease. The putative roles of infection and autoimmune mechanisms in the pathogenesis of tics and obsessive-compulsive symptoms are explored.     

Cerebrospinal fluid cytologic findings in children with borrelia infections (neuroborreliosis)

The infection of the central nervous system by Borrelia burgdorferi induces usually a mononuclear (lympho-mono-plasmocellular) cytogram of the cerebrospinal fluid in humans. The initial granulocytosis of the liquor cerebrospinalis typical for bacterial infections is transistory only. Such cytologic findings may be misleading to a diagnosis as virus infection. In case of clinical signs (Bannwarth syndrome), typical anamnesis or chronic lymphocytic meningitis the estimation of specific IgG/IgM borrelia antibodies is indicated and the antibiotic therapy should be performed.     

Meningoencephalitis in Beh?et's disease--review and clinical-morphological case report]

The paper describes the neuropathological findings in the seven-year case history of a male patient who died at the age of 29 of Beh?et's disease with initial iridocyclitis and neurological complications. In the author's opinion, the presence of any one of the three cardinal symptoms of the muco-cutaneo-ocular syndrome and its manifestation on the CNS is sufficient to suppose Beh?et's disease. Affectation of the eye is a significant and usually the first symptom. The case history and the typical morphological findings give rise to the assumption of a special ophthalmomeningoencephalic variety of Beh?et's disease.     

BDNF serum levels in subjects developing or not post-traumatic stress disorder after trauma exposure

Post-traumatic stress disorder (PTSD) is a syndrome resulting from exposure to a severe traumatic event that poses threatened death or injury and produces intense fear and helplessness. The neural structures implicated in PTSD development belong to the limbic system, an important region for emotional processing. Brain-derived neurotrophic factor (BDNF) is a neurotrophin that serves as survival factor for selected populations of central nervous system (CNS) neurons and plays a role in the limbic system by regulating synaptic plasticity, memory processes and behavior. Impaired BDNF production in the brain can lead to a variety of CNS dysfunctions including symptoms associated with PTSD. However, so far fewer studies have investigated this neurotrophin in patients with PTSD. Furthermore, given the multiple role of BDNF in various CNS disorders, it cannot be excluded that traumatic events per se may influence neurotrophin levels, without a direct association to the PTSD syndrome.     

中医疗效的确定性与不确定性的比较思考

中医对于疾病的认识带有厚重的价值论色彩,昭示了强烈的主体尺度的特色。在辨证论治方面,它强调主体对于输出信息的价值判断,而非事实性认识;辨证论治标准的非客观化、非量化,会使不同主体在直觉体悟下产生不同的辨证结果,因而也就会产生不同的论治方法;中药饮片和中成药质量的现状也使中医经验的拷贝效果往往难以得到预想的重复。中医疗效表现出复杂的确定性与不确定性的矛盾。     

Correlated symptoms and simulated medical classification

Category learning theories can be separated into those that expect judgments to be sensitive to configural information and those that expect judgments to be based on a weighted, additive summation of information. Predictions of these two classes of models were investigated in a simulated medical diagnosis task. Subjects learned about a fictitious disease or about two diseases from hypothetical case studies in which some symptoms were correlated with each other and others were independent. Following this initial training, subjects were presented either with pairs of new cases and asked to judge which was more likely to have the disease or with a single case and asked which disease was present. Across four experiments, subjects proved to be sensitive to configural information. When choosing between pairs of new cases, subjects tended to choose the case that preserved the correlation over the case that broke the correlation, even when the case with correlated symptoms contained fewer typical symptoms. When judging which disease was present in a single case, subjects' diagnoses were determined primarily by the correlated symptoms. Implications of these findings to process models of categorization are discussed.     

Central Nervous System Opportunistic Infections in HIV-1 Infection

Neurologic disease is commonly encountered in the population infected with human immunodeficiency virus type 1 (HIV-1). Although HIV-1 is responsible for many of these neurologic complications, other organisms will affect the nervous system as the immune deficiency state progresses. With the wide use of potent antiretroviral therapy, the mortality from and incidence of opportunistic infections (OIs) among persons with advanced HIV-1 infection has decreased. Nevertheless, these diseases are still seen frequently, especially among those with limited access to new antiretroviral therapies. Therefore, it remains important to recognize the most common OIs of the central nervous system (CNS) as well as primary CNS lymphoma, which will be the focus of this review.     

The diagnostic process,the diagnosis and homeostasis

In this paper I shall try to analyse appropriate logic and actual methods as a preliminary to developing expert systems that will simulate clinical diagnosis. It is doubtful that all diagnoses address the same kind of problem and hence no one logic will suffice. Sometimes the signs and symptoms manifest an underlying disorder that cannot be observed directly (“the substantialist model”); sometimes there seems to be no underlying disorder and the diagnosis is a rearrangement of the data (“the nominalist model”). In deciding their merits in the particular case, we must identify the sources of variation; what is meant by saying that the data are independent; and what can be meant by “wrong” diagnosis and misclassification. Problems arise in human diagnosis because the data may be false, even lies; because the known list of diseases is not exhaustive; and because the probability of multiple unrelated diseases increases with age. Special difficulties arise from homeostasis (which is fundamental in defining and understanding disease) because the primary disturbances may be hard to distinguish from the homeostatic responses to them.     

A Comparison of the 1988 and 1994 Diagnostic Criteria for Chronic Fatigue Syndrome

Chronic Fatigue Syndrome (CFS) is an illness that involves severe, prolonged fatigue as well as neurological, immunological, and endocrinological system pathology. Because the pathogenesis of CFS has yet to be determined, case definitions have relied on clinical observation in classifying signs and symptoms for diagnosis. In an attempt to address various criticisms and inconsistencies in diagnostic criteria, there have been several revisions of the CFS case definition. The current investigation examined the differences between 1988 and 1994 definitions as well as participants who had a psychiatric explanation for their fatigue. Dependent measures included psychiatric comorbidity, symptom frequency, and functional impairment. The 1988 criteria, compared to the 1994 criteria, appeared to select a group of participants with more symptomatology and functional impairment, but these groups did not significantly differ in psychiatric comorbidity. Implications of these findings are discussed.     

Intrathecal methotrexate--overdosing in pediatric lymphoblastic leukemia

Intrathecal methotrexate-overdosage in the meningosis therapy leads to CNS damages dependent on dose. It was given a own case report and a literature review with clinical symptoms and the therapy in this complication. Neuropathologic findings included demyelinisation und necrosis of the white matter.     

Parallel between antianxiety and cardiovascular effects of chlordiazepoxide in genetically nervous dogs

There is evidence that cardiac arrhythmias are partially of CNS origin, and that certain drugs affect arrhythmia, both positively and negatively, through CNS action. We hypothesized and confirmed that chlordiazepoxide diminished the well-established incidence of arrhythmias (A-V blocks and nodal escapes) in five nervous pointer dogs. It is known that this drug normalizes behavior and aids in the bar-pressing performance of nervous dogs. Nervous dogs tend to have low heart rates (HRs) while normals have higher HRs. Chlordiazepoxide increased HRs in our five nervous dogs, and we postulated that HR increase would be less or none in normals. This was confirmed in a later study of five normals. A further study of eight normal dogs and eight nervous dogs essentially reproduced these findings, suggesting the possibility that the slow heart rates and the behavioral abnormality have a common basis, expected to be some CNS biochemical lesion.     

301例中枢神经系统感染的临床分析

回顾性研究301例中枢神经系统感染病例的临床资料,分析其临床特点,将符合临床诊断标准的病例按病原种类分为病脑组、结脑组、化脑组及隐脑组4个组,应用相关的统计描述分析其临床特征。结果发现病毒性脑（膜）炎是最常见的中枢神经系统感染性疾病,而中枢神经系统感染性疾病一年四季均可发病,其中病毒性脑（膜）炎高发于5月及7月,结核性脑膜炎则高发于1月与3月,有明显的季节性。中枢神经系统感染的临床首发症状依次表现为发热、头痛、意识障碍、精神症状、抽搐及神经系统定位症状。     

Cholinesterase-inhibitor therapy for dementia: novel clinical substrates and mechanisms for treatment response

Historically, drugs that increase central cholinergic transmission have primarily been investigated for relieving cognitive symptoms in mild-to-moderate Alzheimer's disease. These efforts have led to the somewhat unexpected findings that cholinergic therapy has a beneficial effect on selected neuropsychiatric symptoms in AD across disease stages. In Parkinson's disease with dementia and dementia with Lewy bodies, cholinergic deficits are more severe than in AD, and there is emerging evidence that cholinesterase inhibitors are efficacious in treating core symptoms of attentional disturbance and psychosis. Recent data also suggest a rational basis for cholinergic therapy in vascular dementia. The cognitive and neuropsychiatric effects of cholinergic therapy observed in AD and other dementias form the crux of an integrative model of cholinergic therapeutic efficacy that encompasses the diverse central nervous system actions of acetylcholine and its complementary interactions with central monoamine transmitters. This heuristic framework highlights the broader therapeutic potential of cholinergic therapy for symptom-based indications in other neuropsychiatric disorders.     

Anorexia nervosa: A pastoral update

Anorexia nervosa is a disorder of unknown etiology that is a complex of physical, emotional, and behavioral changes occurring in individuals who starve themselves. The incidence of this disease has increased dramatically in the last twenty years. Most patients seem to be unaware of or unconcerned about their emaciation. After presenting a case, this study reviews the most recent findings about this disease, including the symptoms, social characteristics of its victims, increaed incidence, history, and prognosis. Suggestions are offered for pastoral care and counseling.     

发挥中药在肿瘤症状处理中的作用——兼谈结直肠癌的中医“病证结合辨治”

改善症状是中医治疗晚期恶性肿瘤的优势,但简单的对症治疗显然体现不了中医的特色;辨证论治虽然是中医治疗的核心,但在对癌病本质的把握上缺乏理论和实践的支撑;单一的辨病论治强调以毒攻毒,却不能改善症状及预后。本文作者以对结直肠癌的治疗为例,认为抓主症,将病证与辨治相结合,有望较好地解决“症”“证”“病”三者的关系,在改善症状...     

Neuropsychological Sequelae of Non-Central Nervous System Cancer and Cancer Therapy

Cancer patients report numerous adverse symptoms associated with their disease and treatment including cognitive dysfunction, fatigue, and affective distress. Cognitive dysfunction is ubiquitous in patients with primary central nervous system (CNS) cancer and recent evidence has documented similar deficits in patients with non-CNS cancer as well. Both the cancer itself and treatments including chemotherapy, biological response modifiers, and hormonal therapies have been demonstrated to adversely impact cognitive and neurobehavioral function. Neuroimaging and neurophysiological investigations have likewise revealed alterations in brain function that are helping to account for the nature of these cognitive disorders. Similarly, preclinical animal research is assisting to identify the pathophysiological mechanisms that underlie treatment-related neurotoxicities. The coalescence of multidisciplinary clinical and research efforts hold promise for the development of interventions that may offer neuroprotection in addition to currently available symptomatic therapies and cognitive rehabilitation techniques.     

重症手足口病的早期识别与处理

手足口病(hand-foot-mouth disease,HFMD)是由肠道病毒[以柯萨奇A组16型(Coxsackievirus A16,CoxA16)、肠道病毒71型(Enterovirus 71,EV71)多见]引起的急性传染病,而重症多由EV71感染引起。由于EV71的高度嗜神经性,重症者可合并中枢神经系统(central nervous system,CNS)损害、急性肺水肿/出血等。现就重症手足口病的早期识别与处理进行简要综述。     

Examining multiple feature evaluation and classification methods for improving the diagnosis of Parkinson’s disease

An accurate diagnosis of Parkinson’s disease by specialists involves many neurological, psychological and physical examinations. The specialists investigate a number of symptoms and signs when examining the nervous system conditions of a person. The diagnosis involves reviewing the medical history and genetic factor of the person. The recent diagnosis methodology to Parkinson’s disease relies on voice disorders analysis. This methodology entails extracting feature sets of a recorded person’s voice then utilizing a machine learning technique to identify the healthy and Parkinson’s cases from the voice. This paper attempts to improve the diagnoses of Parkinson’s disease by testing multiple feature evaluation and classification machine learning methods based on the voice disorders analysis. The aim of this paper is to find the optimal solution to the problem by (i) proposing a new Multiple Feature Evaluation Approach (MFEA) of a multi-agent system (ii) implementing five independent classification schemas which are Decision Tree, Naïve Bayes, Neural Network, Random Forests, and Support Vector Machine on the Parkinson’s diagnosis before and after applying the MFEA, and (iii) evaluating the diagnosis accuracy of the results. The methodology of the tests encompasses 10-fold cross-validation to evaluate the learning of methods and track variation in their performance. The test results show that the MFEA of the multi-agent system finds the best set of features and improves the performance of the classifiers. The average rate of improvement in the diagnostic accuracy of the classifiers are Decision Tree 10.51%, Naïve Bayes 15.22%, Neural Network 9.19%, Random Forests 12.75%, and Support Vector Machine 9.13%. These results show that the MFEA makes a significant improvement to the classifiers’ diagnosis results.     

Neuropsychological outcomes of pediatric demyelinating diseases: a review

Immune-mediated central nervous system (CNS) demyelinating diseases impact various areas of the brain, optic nerves, and/or spinal cord and can result in a wide range of neurologic symptoms including adverse cognitive outcomes. Neuropsychological outcomes in adult multiple sclerosis (MS) are well documented, while literature on such outcomes in pediatric cohorts is more limited. Furthermore, literature on neuropsychological outcomes in pediatric acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO), and transverse myelitis (TM) is even more limited. This paper is the first to review what is known about neuropsychological outcomes associated with immune-mediated CNS demyelinating diseases, with a focus on pediatric MS, ADEM, NMO, and TM. Additionally, this review illuminates the need to clarify differences in neuropsychological sequelae between conditions, characterize longitudinal cognitive outcomes, and investigate neuropsychological outcomes in relation to clinical variables (e.g., age of onset, disease duration, number of relapses) and psychosocial variables (e.g., fatigue, emotional problems, behavioral functioning) to better understand neuropsychological outcomes associated with these conditions.