Search

American neurologist and neuropathologist Abraham Bert (Abe) Baker (1908-1988) was instrumental in founding the American Academy of Neurology and served as a catalyst for the emergence of neurology as a strong, independent medical discipline in the United States in the second half of the twentieth century. Baker served as the first president of the Academy from 1948 to 1951. He was also instrumental in garnering support for the National Institute of Neurological Diseases and Blindness, which was founded in 1950 and later evolved into the National Institute of Neurological Disorders and Stroke. Baker’s leadership was also essential in developing continuing medical education for neurologists at a national level and in garnering federal financial support for neurology training programs. [ABSTRACT FROM AUTHOR]

When University of Minnesota neurology resident Joseph A. Resch complained to the department director, A. B. Baker, in 1946 about the lack of a professional neurological association open to young neurologists like himself, he set into motion a chain of events that culminated in the founding of the American Academy of Neurology in June of 1948. This provided a supportive professional organization for practicing neurologists as the traditional bond between neurology and psychiatry was broken during the postwar era. Resch went on to become a pivotal figure in the practice of neurology in the Twin Cities and succeeded Baker as chairman of the University of Minnesota neurology department. [ABSTRACT FROM AUTHOR]

American neurologist and epileptologist Francis M. Forster (1912-2006) was the last survivor of the “Four Horsemen,” a nickname given to the four neurologists—Forster, Abe Baker, Russell DeJong, and Adolph Sahs—who were most instrumental in founding the American Academy of Neurology under Baker’s leadership in 1948. Forster was a consulting physician for many high-profile patients, including President Dwight Eisenhower, President Quirino and Archbishop Reyes of the Philippines, Provisional President Lonardi of Argentina, and Cardinal Albert Meyer of Chicago. Forster was also an expert witness for the prosecution in the trial of Jack Ruby, who killed Lee Harvey Oswald. Forster’s greatest legacy, though, was as a teacher: During his career as chairman of two robust academic neurology departments, he trained more than 100 residents, at least 17 of whom went on to become chairmen of neurology departments in the United States, Europe, Asia, and South America. [ABSTRACT FROM AUTHOR]

With few exceptions, neurology in Europe as well as in the United States emerged from internal medicine and psychiatry, and neurology and psychiatry in particular have long remained connected in clinical practice and teaching. When the American Board of Psychiatry and Neurology (ABPN, 1934) and the American Academy of Neurology (AAN, 1948) were founded, the emancipation of neurology as an independent specialty was still evolving. During the First International Neurological Congress (Berne, Switzerland, in September 1931), a special conference was organized on the “Relation of Neurology to General Medicine and Psychiatry in Universities and Hospitals of the Various Countries,” at which representatives from several countries described the situation in their countries. Their statements were made around the time of the founding of the ABPN and not long before that of the AAN. They show that neurology in most countries was still struggling to become independent and only in a few cities flowered as an independent specialty. In the second part of this article, specialist regulation (training, examination, and certification) in European countries (Germany, France, England, and The Netherlands) will be compared to that of the ABPN. It appears that Germany was among the early countries where this occurred, following the Bremen Ärztetag (physicians day) in 1924. Comparable to the American situation, it was professionally controlled, in contrast to the French state certification, which occurred later. The British specialist regulation was much later and more complicated. [ABSTRACT FROM AUTHOR]

Russell Nelson DeJong (1907-1990) became professor and chair of the Department of Neurology at the University of Michigan in 1950. A prolific author with more than 200 publications, DeJong wrote on virtually all areas of neurology. DeJong’s classic monograph, The Neurologic Examination (1950), grew into an encyclopedic volume from lectures he gave to junior and senior medical students; DeJong saw it through four editions. He was one of the founders of the American Academy of Neurology (AAN), serving as its vice-president from 1961 to 1963 and as the first editor-in-chief of its journal, Neurology. Abraham Baker, the driving force behind the founding of the AAN, considered DeJong “the most scholarly neurologist in the Academy.” DeJong was also president of the American Neurological Association, president of the American Board of Psychiatry and Neurology, president of the American Epilepsy Society, and chairman of the Scientific Advisory Board of the National Multiple Sclerosis. [ABSTRACT FROM AUTHOR]

Adolph Louis Sahs (1906-1986) became chair of the Department of Neurology at the University of Iowa in 1948 and retired in 1974. Through his academic neurology organizational skills, coordination of multicenter research on subarachnoid hemorrhage, and education of more than 50 neurologists, he helped bring the department to national and international prominence. Sahs was one of the founders of the American Academy of Neurology. He served as president of the Academy, the American Neurological Association, and the American Board of Psychiatry and Neurology. Sahs was known as a neurologist who could always take on yet another job in the interest of the specialty. [ABSTRACT FROM AUTHOR]

Background and Purpose: Incomplete hippocampal inversion (IHI) is an atypical anatomical pattern presented by the hippocampus. It is associated with several neuropathological conditions and is thought to be a factor of susceptibility to hippocampal sclerosis and loss of volume. The volume loss of hippocampus is an inevitable consequence of aging, and when accelerated it is commonly considered an imaging biomarker of Alzheimer's disease dementia.Methods: We have studied the relationship between IHI and hippocampal subfield volumes in a cohort of 60 healthy participants of 49-87 years of age. The presence and severity of IHI and hippocampal subfield volumes were quantified from T2 magnetic resonance (MR) images acquired at 3T.Results: It was found that IHI presented in 23.3% of participants. Right unilateral IHI was rare (two cases, 3.3%) in comparison to left unilateral IHI (nine cases, 15%), with three (5%) of participants showing bilateral IHI. No significant relationships between the whole hippocampal volumes and IHI were observed. Instead, significant relationships between the volumes of the left and right cornu ammonis subfield-1 (CA1) and IHI scores were evident.Conclusions: The rates of IHI prevalence in the current cohort are similar to those previously reported in healthy cohorts. The IHI severity is related to hippocampal subfield volumes, most notably the CA1, which is a novel finding with potential implications in research on aging and dementia. [ABSTRACT FROM AUTHOR]

Traditional diagnostic neuropathology relies on subjective interpretation of visual data obtained from a brightfield microscopy. This approach causes high variability, unsatisfactory reproducibility, and inability for multiplexing even among experts. These problems may affect patient outcomes and confound clinical decision-making. Also, standard histological processing of pathological specimens leads to auto-fluorescence and other artifacts, a reason why fluorescent microscopy is not routinely implemented in diagnostic pathology. To overcome these problems, objective and quantitative methods are required to help neuropathologists in their clinical decision-making. Therefore, we propose a computerized image analysis method to validate anti-PTBP1 antibody for its potential use in diagnostic neuropathology. Images were obtained from standard neuropathological specimens stained with anti-PTBP1 antibody. First, the noise characteristics of the images were modeled and images are de-noised according to the noise model. Next, images are filtered with sigma-adaptive Gaussian filtering for normalization, and cell nuclei are detected and segmented with a k-means-based deterministic approach. Experiments on 29 data sets from 3 cases of brain tumor and reactive gliosis show statistically significant differences between the number of positively stained nuclei in images stained with and without anti-PTBP1 antibody. The experimental analysis of specimens from 3 different brain tumor groups and 1 reactive gliosis group indicates the feasibility of using anti-PTBP1 antibody in diagnostic neuropathology, and computerized image analysis provides a systematic and quantitative approach to explore feasibility. [ABSTRACT FROM AUTHOR]

The professional regulatory cases of the psychiatrist Dr Richard Pool and the neuropathologist Dr Waney Squier have given rise to concerns among expert psychiatric witnesses, and indeed medical experts in general. Here we restate the law on expert evidence with particular reference to the judgment of the Supreme Court in the case of Kennedy v Cordia. We emphasise that Pool does not change the law regarding 'what is expertise'; in particular, the case does not establish restrictive, status-based tests governing the admissibility of expert evidence such as according to whether an expert psychiatric witness has undergone higher training, is on the specialist register as a specialist in a particular field or is a consultant. Rather, expertise continues to be legally defined in terms of a combination of qualification, knowledge and experience. Crucially, the test of medical expertise in legal proceedings is a legal test and not one determined within a medical paradigm. [ABSTRACT FROM AUTHOR]

The article profiles Dr. Harry M. Zimmerman, a neuropathologist. He was born in Vilnius, Lithuania to a stonemason and was brought to the U.S. in 1909. He graduated from Yale University Medical School in 1092 and initially trained under Dr. Milton Winternitz, a professor of pathology. Ultrastructure of cerebral vessels, pathology of cryptococcosis and myelin pathology in the brain based on 70 autopsies are some of seminal studies conducted by Zimmerman.

The article is a 75-year memorial tribute to the Greek neurologist, Constantin Tsiminakis (1875–1942). Coming from a family of physicians, Tsiminakis graduated from the University of Athens in 1897, and trained in Vienna under Nothnagel, Frankl-Hochwart, and Obersteiner. In 1905, he was appointed Reader in Neurology and Psychiatry at his alma mater. He published over 40 articles on topics of neurology and neuropathology, including megalencephaly, hydrocephalus, progressive paralysis, epidemic encephalitis, dengue fever, and narcolepsy. However, his main focus of interest was epilepsy, including post-encephalitic forms. To differentially diagnose true from feigned epilepsy, he devised a method of compressing the carotids, which became known as the “Tsiminakis maneuver”. A cultivated man and a talented poet, he rebutted the Freudian interpretation of artistic creativity. Finally, in the national legislative election in 1923, Tsiminakis was elected a Plenipotentiary Member of the Hellenic Parliament under the Liberal Party and served until 1925. [ABSTRACT FROM AUTHOR]

Purpose: This study examined the feasibility of using quantitation to augment interpretation of florbetapir PET amyloid imaging. Methods: A total of 80 physician readers were trained on quantitation of florbetapir PET images and the principles for using quantitation to augment a visual read. On day 1, the readers completed a visual read of 96 scans (46 autopsy-verified and 50 from patients seeking a diagnosis). On day 2, 69 of the readers reinterpreted the 96 scans augmenting their interpretation with quantitation (VisQ method) using one of three commercial software packages. A subset of 11 readers reinterpreted all scans on day 2 based on a visual read only (VisVis control). For the autopsy-verified scans, the neuropathologist's modified CERAD plaque score was used as the truth standard for interpretation accuracy. Because an autopsy truth standard was not available for scans from patients seeking a diagnosis, the majority VisQ interpretation of the three readers with the best accuracy in interpreting autopsy-verified scans was used as the reference standard. Results: Day 1 visual read accuracy was high for both the autopsy-verified scans (90%) and the scans from patients seeking a diagnosis (87.3%). Accuracy improved from the visual read to the VisQ read (from 90.1% to 93.1%, p < 0.0001). Importantly, access to quantitative information did not decrease interpretation accuracy of the above-average readers (>90% on day 1). Accuracy in interpreting the autopsy-verified scans also increased from the first to the second visual read (VisVis group). However, agreement with the reference standard (best readers) for scans from patients seeking a diagnosis did not improve with a second visual read, and in this cohort the VisQ group was significantly improved relative to the VisVis group (change 5.4% vs. −1.1%, p < 0.0001). Conclusion: These results indicate that augmentation of visual interpretation of florbetapir PET amyloid images with quantitative information obtained using commercially available software packages did not reduce the accuracy of readers who were already performing with above average accuracy on the visual read and may improve the accuracy and confidence of some readers in clinically relevant cases. [ABSTRACT FROM AUTHOR]

Objective Intraoperative distinction of brain tumor from surrounding brain is a crucial challenge in neuro-oncologic surgery. We directly compared confocal laser endomicroscopy (CLE) findings with intraoperative instantaneous sections by the neuropathologist in a blinded fashion. Methods The imaging device comprises a rigid endoscope with Hopkins rod lenses and a red wave length laser with a scanning depth of 80 μm. Brain tumor samples of 100 patients were investigated. Tissue samples were simultaneously investigated by the neuropathologist and with CLE. The tissue was not prepared or stained before CLE analysis. Results CLE could be performed in all cases. Sensitivity for detection of a correct final diagnosis by CLE on site was 82%–90% for high-grade gliomas (26/32), low-grade gliomas (9/10), schwannomas (7/8), and meningiomas (28/34). Sensitivity of only 37% (6/16) was achieved for metastasis (6/16). Conclusions With intraoperative CLE, it is possible to obtain an on-site histologic diagnosis with a high sensitivity in many tumors. Although definitive histologic classification requires further neuropathologic investigation, these results show that CLE could fill the gap between tissue resection and microscopic analysis. This could ultimately help neurosurgeons to scan brain tissue for tumor remnants on a microscopic scale without having to resect it first. Further development of the device and further investigations are needed before this technique can become part of the neurosurgical routine in specific cases. [ABSTRACT FROM AUTHOR]

Background: Parkinson's disease is the second most common neurodegenerative disorder for which old age is the best known risk. The proportion of elderly in the world is increasing, resulting in larger pool of people at risk for Parkinson's disease. Several other neurodegenerative disorders also produce Parkinson syndrome. Distinguishing between those variants is only possible with pathological examination of brain. No autopsy confirmed study of 80 years and older onset in parkinsonism cases has been reported. Clinical features of different PS variants, response to treatment and progression of disease in this age group remain to be determined.Methods: Patients evaluated at Movement Disorders Clinic Saskatchewan are offered a choice of autopsy at no cost. The brain is studied by board certified neuropathologist.Results: Thirty cases with clinical diagnosis of parkinsonism (onset ≥80 years) came to autopsy. Twenty-one (70%) had Parkinson's disease alone and two (6.7%) had an additional movement disorder. The progression of Parkinson's disease was accelerated, and dementia evolved earlier than reported in the younger onset cases. Most cases that tolerated an adequate dose improved on levodopa.Conclusion: Parkinson's disease is the most common variant in the octogenarian population. Most patients benefit from levodopa, and should be tried on the drug when diagnosis of parkinsonism is made. [ABSTRACT FROM AUTHOR]

In 1933 the Turkish Republic formally offered university positions to 30 German-speaking academics who were dismissed with the coming to power of the National Socialist Government. That initial number went up to 56 with the inclusion of the technical assistants. By 1948 the estimated total had increased to 199. Given renewable five-year contracts with salaries substantially higher than their Turkish counterparts, the foreign émigrés were to implement the westernization program of higher education. The ten year-old secular Turkish Republic’s extensive social reforms had encompassed the adoption of the Latin alphabet, and equal rights for women, removing gender bias in hiring. Such a high concentration of émigré academics in one institution, “the highest anywhere in the world,” provides a unique opportunity to study a subject which has been neglected. In this article two cases in psychology will be examined: Wilhelm Peters (1880–1963), who came, via Britain, to Istanbul in 1936 from the University of Jena in Germany, and Muzafer Sherif (1906–1988) who went to the United States from Ankara University in 1945. The purpose of the comparative analysis is to identify the features that are specific to the German experience, and those that are shared and underlie translocation in science within the multifaceted complexity of the process of forced migration. [ABSTRACT FROM PUBLISHER]

The article discusses a study which examined the techniques used by neuropathologists in the diagnosis of brain tumors. Particular focus is given to the scope of the online survey on brain tumor diagnostics. Also discussed are response rate from the survey, use of World Health Organization guidelines on neuropathological diagnosis, immunohistochemical methods, molecular genetic/epigenetic analysis of the tumors.

An interview with Laura Manuelidis, head of neuropathology section at Yale Medical School in New Haven, Connecticut, is presented. Topics discussed include career wishes as a child, interest in science, and experiences and education at university. She mentions her mentors including neurophysiologist Jack Flynn, chairman Harry S. N. Greene, and Peyton Rous.

The Chinese Glioma Cooperative Group (CGCG) Guideline Panel for adult diffuse gliomas provided recommendations for diagnostic and therapeutic procedures. The Panel covered all fields of expertise in neuro-oncology, i.e. neurosurgeons, neurologists, neuropathologists, neuroradiologists, radiation and medical oncologists and clinical trial experts. The task made clearer and more transparent choices about outcomes considered most relevant through searching the references considered most relevant and evaluating their value. The scientific evidence of papers collected from the literature was evaluated and graded based on the Oxford Centre for Evidence-based Medicine Levels of Evidence and recommendations were given accordingly. The recommendations will provide a framework and assurance for the strategy of diagnostic and therapeutic measures to reduce complications from unnecessary treatment and cost. The guideline should serve as an application for all professionals involved in the management of patients with adult diffuse glioma and also as a source of knowledge for insurance companies and other institutions involved in the cost regulation of cancer care in China. [ABSTRACT FROM AUTHOR]

Purpose: Forking of the cerebral aqueduct is a developmental malformation that is infrequently encountered by neurosurgeons as a rare cause of hydrocephalus, sometimes with a delayed onset. The etiology of an apparently forked aqueduct might be different. However, neuroendoscopy can often be the optimal treatment. The purpose of this study was to review the literature by analyzing the anatomical, functional, diagnostic, and therapeutic features of this unusual condition and adding our personal cases. Methods: We present a case of forking of the cerebral aqueduct that was detected in vivo and treated with a flexible scope. A thorough review of the pertinent literature is also discussed. In the past years, diagnosis of forked aqueduct was possible only postmortem. Results: A forked aqueduct is occasionally encountered in patients when a delayed hydrocephalic decompensation occurs. Conclusions: Flexible neuroendoscopy enables for a direct, in vivo diagnosis and immediate treatment through a third ventriculostomy. [ABSTRACT FROM AUTHOR]

The article examines evaluation and nomenclature of aging-related tau astrogliopathy which occurs usually in individuals over 60 years of age. It states that the astrocytes include thorn-shaped astrocytes in white matter and at the glia limitans. It mentions that anatomical distribution and morphology shows the condition can be distinguished from primary tauopathies and recommends four steps to evaluate the condition.

Background: Prognosis of patients with glioblastoma with oligodendroglial component (GBM-O) is not well defined. We report our experience of patients of GBM-O treated at our center. Methods: Between January 2007 and August 2013, out of 817 consecutive patients with glioblastoma (GBM), 74 patients with GBM-O were identified in our prospectively maintained database. An experienced neuropathologist revaluated the histopathology of all these 74 patients and the diagnosis of GBM-O was eventually confirmed in 57 patients. Patients were uniformly treated with maximal safe resection followed by focal radiotherapy with concurrent and adjuvant temozolamide (TMZ). Results: At a median follow up of 16 months, median overall survival (OS) and progression free survival (PFS) of the entire cohort was 23 months and 13 months respectively. Near total excision was performed in 30/57 (52.6%). On univariate analysis, age < 50 years was a significant favourable prognostic factor for OS (p = 0.009) and PFS (p = 0.017), while patients with near total resection had a significantly better PFS (p = 0.017), patients who completed a minimum of 6 cycles of adjuvant TMZ had significantly better OS (p = 0.000) and PFS (p = 0.003). On multivariate analysis, none of the above factors were significant except for patient who had completed a minimum of 6 cycles of TMZ (OS; p = 0.000 & PFS; p = 0.015). A comparative analysis of GBM-O patients with a similarly treated cohort of 105 GBM patients during the same period revealed significantly better median OS in favour of GBM-O (p = 0.01). Conclusions: Our experience suggests patients with GBM-O have a more favourable clinical outcome as compared to GBM. [ABSTRACT FROM AUTHOR]

The article explores the contributions of German-American neuropathologist Karl Theodor Neubuerger to the field of neurology. Neubuerger is recognized for merging clinical insights from his patients with the latest neurological and psychiatric research. The influence of mentor and neuro-oncologist Walther Spielmeyer on the work of Neubuerger is discussed, as well as Neubuerger's relationships with the Rockefeller Foundation and the Kaiser Wilhelm Society.

An editorial on brain disease such as epilepsy is presented. The author mentions the neuropathology of epilepsy and how neuropathologists began to study the histopathological diagnosis of epilepsy in the 1950s. The author also discusses the treatment plan for epilepsy that includes resective surgery for certain types of epilepsies. Topics discussed include temporal lobe epilepsy, neuroimaging studies and disease progression.

The authors investigated the effects of voluntary and automatic control on the spatial variables (envelope area, maximal amplitude, and root mean square [RMS]) of center of pressure (COP) displacement during quiet standing and identified differences in their postural control strategies (mean velocity [MV], mean power frequency [MPF], and power density). COP data were recorded under relaxed (experimental control), still (voluntary control), and dual (automatic control) conditions. RMS was significantly lower in the still and dual conditions than in the relaxed condition. MV, MPF, and power density were significantly higher in the still condition than in the dual condition. These results indicate that both voluntary and automatic control decrease the spatial variables of COP displacement; however, their postural control strategies are different. [ABSTRACT FROM AUTHOR]

ABSTRACT.The aim was to verify if motor performance at second or third month of life better predicts further development. The global motor development was assessed by a neurologist and by a physiotherapist in 111 children at 2, 3, 6, and 9 months. At 2 and 3 months a physiotherapist also performed the assessment of qualitative elements. The physiotherapeutic assessment in the third month showed higher compatibility with the neurological assessment. Proper motor performance at third month could ensure the level of at least 7 months in the ninth month of life. Qualitative score above 7 of 15 points in the third month ensured proper development in ninth month. Third month of life is a better predictor of further motor development. [ABSTRACT FROM AUTHOR]

Brain metastasis is an uncommon but increasing manifestation of ovarian epithelial carcinoma and neuropathologists' collective experience with these tumors is limited. We present clinicopathological characteristics of 13 cases of brain metastases from ovarian epithelial carcinoma diagnosed at two academic institutions. The mean ages at diagnosis of the ovarian carcinoma and their subsequent brain metastases were 58.7 and 62.8 years, respectively. At the time of initial diagnosis of ovarian carcinoma the majority of patients had an advanced stage and none had brain metastases as their first manifestation of malignancy. Brain metastases tended to be multiple with ring-enhancing features on neuroimaging. Primary tumors and their brain metastases were all high-grade histologically and the histologic subtypes were: nine high-grade serous carcinoma ( HGSC) cases, two clear cell carcinoma ( CCC) cases and a single case each of carcinosarcoma and high-grade adenocarcinoma. A recommended histo- and immunopathological approach to these tumours are provided to aid neuropathologists in the recognition and classification of metastatic ovarian carcinoma to the brain. [ABSTRACT FROM AUTHOR]

The article offers information on 116th Meeting of the British Neuropathological Society Institute of Child Health, to be held March 4, 2015 in London, England.

No abstract available [ABSTRACT FROM AUTHOR]

Introduction Movement disorder specialists can achieve a high level of accuracy when clinically diagnosing parkinsonism syndromes. However, data about the diagnostic accuracy among general neurologists is limited. Objectives This study investigated the recent diagnostic accuracy of parkinsonism syndromes by general neurologists. Methods A retrospective examination of 1362 post-mortem cases diagnosed in the years 2000-2012 by neuropathologists was performed. Out of these cases, we identified 111 patients who received a clinical parkinsonism diagnosis during life and 122 patients who received a neuropathological diagnosis of a parkinsonism syndrome post-mortem including 11 incidental cases. Results Fifty-eight (75.3%) of the 77 patients who had received clinical Parkinson's disease (PD) diagnoses were confirmed after the neuropathological examination. The sensitivity of the clinical diagnosis for idiopathic Parkinson's disease (PD) was 89.2% and the specificity was 57.8%. The corresponding numbers for progressive supranuclear palsy (PSP) were 52.9% and 100%, and for multiple system atrophy (MSA) were 64.3% and 99.0%, respectively. Conclusions Parkinson's disease is heavily overdiagnosed by general neurologists, whereas parkinsonism plus syndromes are underdiagnosed. Despite improvements in the diagnostic methods during recent decades and the development of diagnostic clinical criteria for parkinsonian syndromes, the diagnostic accuracy of Parkinson's disease remains relatively low, and 1/4 of diagnoses are incorrect. [ABSTRACT FROM AUTHOR]

The article offers information on the International Society of Neuropathology (ISN). Topics discussed include the travel grants awarded by the association for traineers to visit centres of excellence, the bursaries that support trainees in neuropathology, and the website of the society located at http://www.intsocneuro pathol.com/.

Training of neuropathologists varies worldwide. Systems range from highly organized specialist and subspecialist education with national certification, to regulated training with diploma recognition, to informal apprenticeships in neurological hospitals and no formal recognition. This overview compiles and summarizes the history of regulated training systems, the status of neuropathology within various countries' medical systems and the manner in which neuropathologists are trained. Anecdotal evidence suggests that countries with regulated systems of neuropathology training and an active professional organization are more likely to have an adequate supply of diagnostic specialists and a vibrant research community. The different training systems reflect the style of medical services delivery in the respective countries. In general, the existence of formal neuropathology training systems occurs only in countries with relatively high levels of per capita health expenditures, reflecting the development of medical specialization overall. Evolving diagnostic technologies and major international research endeavors, whose goals are to understand structure and function of the human brain, demand that neuropathology training is more than simply diagnostic histopathology. [ABSTRACT FROM AUTHOR]

Inclusion body myositis is a slowly progressive myopathy affecting predominantly the middle-aged and older patient population. It is a major form of the idiopathic inflammatory myopathies which are chronic systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. Unfortunately, there is no effective therapy yet; however, the early diagnosis is essential to provide treatment options which may significantly slow the progression of the disease. In our case-based clinicopathological study the importance of the close collaboration between the clinician and the neuropathologist is emphasised. [ABSTRACT FROM AUTHOR]

Abstract: Pathologists recognize and classify cancers according to nuclear morphology, but there remains little scientific explanation of why malignant nuclei possess their characteristic features, or how those features are related to dysregulated function. This essay will discuss a basic structure–function axis that connects one central architectural motif in the nucleus–the chromatin loop–to the vital nuclear function of transcription. The loop is attached to a “transcription factory” through components of the transcription machinery (either polymerases or transcriptional activators/repressors), and the position of a gene within a loop determines how often that gene is transcribed. Then, dysregulated transcription is tightly coupled to alterations in structure, and vice versa. We also speculate on how the experimental approaches being used to analyze loops and factories might be applied to study the problems of tumour initiation and progression. [Copyright &y& Elsevier]

It is generally accepted that Alois Alzheimer, the German neuropathologist and clinician, discovered the disease that carries his name, after the clinicopathological study of a 51-year-old woman named Auguste D. who presented a dementia syndrome. The pathological study of the brain revealed the presence of neurofibrillary tangles and senile plaques. Emil Kraepelin coined the eponym Alzheimer''s disease in the 8th edition of his textbook Clinical Psychiatry. However, several critical aspects of this history have been pointed out by historians of psychiatry. This article provides a narrative of the best-known facts leading to the formation of the original concept but also presents an informed discussion of the main critical points: 1. The descriptions of senile plaques and neurofibrillary tangles in the context of dementia before Alzheimer''s report. 2. The presence or absence of arteriosclerotic changes in the brain of Auguste D. 3. The presence of noncognitive symptoms in August D. 4. The influence of social, political and economic issues in the formation and selection of medical concepts. [Copyright &y& Elsevier]

Parkinson's disease (PD) is a complex multifactorial disease marked by extensive neuropathology in the brain with selective yet prominent and progressive loss of mid-brain dopaminergic neurons. The etiological factors involved in the development of PD are still elusive, but oxidative stress arising when reactive oxygen species (ROS) exceed amounts required for normal redox signaling is considered one of the major factors. ROS cause oxidative damage to proteins, lipids, and DNA and are one of the most prominent factors related to neurodegeneration. Pre-clinical and clinical studies clearly demonstrate the effectiveness of oxidative stress in the pathogenesis of PD. Therefore, regulation of redox signaling and inhibiting excess ROS would contribute greatly not only to extend longevity but also to ameliorate the progression of dopaminergic cell death seen in patients with PD. Several herbal products are beneficial for maintaining nerve cell function and for treating various neurodegenerative disorders by reducing oxidative stress. Here, we summarize the recent knowledge concerning promising herbs that have shown significant beneficial effects based on regulation of redox status and ROS inhibition in toxin-induced PD models. [ABSTRACT FROM AUTHOR]

Purpose: An International League Against Epilepsy (ILAE) consensus classification system for focal cortical dysplasias (FCDs) has been published in 2011 specifying clinicopathologic FCD variants. The aim of the present work was to microscopically assess interobserver agreement and intraobserver reproducibility for FCD categories among an international group of neuropathologists with different levels of experience and access to epilepsy surgery tissue. Methods: Surgical FCD specimens covering a broad histopathology spectrum were retrieved from 22 patients with epilepsy. Three surgical nonepilepsy specimens served as controls. A total of 188 slides with routine or immunohistochemical stainings were digitalized with a slide scanner to allow Internet-based microscopy review. Nine experienced neuropathologists were invited to review these cases twice at a time gap of 3 months and different orders of case presentation. The 2011 ILAE FCD consensus classification served as instruction. Kappa analysis was calculated to estimate interobserver and intraobserver agreement levels. In a third evaluation round, 21 additional neuropathologists with different experience and access to epilepsy surgery reviewed the same case series. Key Findings: Interobserver agreement was good (κ = 0.6360), with 84% consensus of diagnoses during the first evaluation (21 of 25 cases). Kappa values increased to 0.6532 after reevaluation, and consensus was obtained in 24 (96%) of 25 cases. Overall intraobserver reproducibility was also good (κ = 0.7824, ranging from 0.4991 to 1.000). Fewest changes in the classification were made in the FCD type II group (2.2% of 225 original diagnoses), whereas the majority of changes occurred in FCD type III (13.7% of 225 original diagnoses). In the third evaluation round, interobserver agreement was reflected by the level of experience of each neuropathologist, with κ values ranging from moderate (0.5056; high level of experience >40 cases/year) to low (0.3265; low level of experience <10 cases/year). Significance: Our study achieved a good and reliable interobserver agreement among the group of expert neuropathologists originally involved in the ILAE FCD consensus classification system. Intraobserver reproducibility in this group was even more robust. These results showed considerable improvement compared to a previous study evaluating the 2004 Palmini FCD classification. Agreement levels were lower in our second group of neuropathologists and were related to their level of access and experience with epilepsy surgery specimens. These results suggested that the more precise ILAE definition of FCD histopathology patterns improves operational procedures in the diagnosis of FCDs. On the other hand, microscopic assessment of FCD is a challenge and requires sustained experience and teaching. The virtual slide review system allowed testing of this hypothesis and reached a widespread group of participating colleagues from different centers all over the world. We propose to further use this tool as a teaching device and also to address other epilepsy-associated entities still difficult to classify such as hippocampal sclerosis, long-term epilepsy-associated tumors, or mild malformations of cortical development (mMCDs), which were not yet covered by current ILAE classification systems. [ABSTRACT FROM AUTHOR]

Background: Depression has been associated with higher conversion rates from mild cognitive impairment (MCI) to Alzheimer''s disease (AD) and may be a marker of prodromal AD that can be used to identify individuals with MCI who are most likely to progress to AD. Thus, we examined the neuroanatomical changes associated with depressive symptoms in MCI. Methods: Two-hundred forty-three MCI subjects from the Alzheimer''s Disease Neuroimaging Initiative who had brain magnetic resonance imaging scans at baseline and 2-year follow-up were classified into depressed (n = 44), nondepressed with other neuropsychiatric symptoms (n = 93), and no-symptom (NOSYMP; n = 106) groups based on the Neuropsychiatric Inventory Questionnaire. Tensor-based morphometry was used to create individual three-dimensional maps of 2-year brain changes that were compared between groups. Results: Depressed subjects had more frontal (p = .024), parietal (p = .030), and temporal (p = .038) white matter atrophy than NOSYMP subjects. Those whose depressive symptoms persisted over 2 years also had higher conversion to AD and more decline on measures of global cognition, language, and executive functioning compared with stable NOSYMP subjects. Nondepressed with other neuropsychiatric symptoms and NOSYMP groups exhibited no differences in rates of atrophy. Conclusions: Depressive symptoms were associated with greater atrophy in AD-affected regions, increased cognitive decline, and higher rates of conversion to AD. Depression in individuals with MCI may be associated with underlying neuropathological changes, including prodromal AD, and may be a potentially useful clinical marker in identifying MCI patients who are most likely to progress to AD. [Copyright &y& Elsevier]

The article offers news briefs related to the International Society of Neuropathology (ISN) as of 2012. It says that the XVIII International Congress of Neuropathology will be held in Rio de Janeiro, Brazil from September 14-18, 2014. It says that professor Herbert Budka of the Medical University of Vienna Institute of Neurology is the president of the Austrian Society of Neuropathology. Meanwhile, there is no official organization that represents the field of neuropathology in South Africa.

Peripheral nerve sheath tumors are common neoplasms, with classic identifiable features, but on occasion, they are diagnostically challenging. Although well-defined subtypes of peripheral nerve sheath tumors were described early in the history of surgical pathology, controversies regarding the classification and grading of these tumors persist. Advances in molecular biology have provided new insights into the nature of the various peripheral nerve sheath tumors, and have begun to suggest novel targeted therapeutic approaches. In this review, we discuss current concepts and problematic areas in the pathology of peripheral nerve sheath tumors. Diagnostic criteria and differential diagnosis for the major categories of nerve sheath tumors are proposed, including neurofibroma, schwannoma, and perineurioma. Diagnostically challenging variants, including plexiform, cellular and melanotic schwannomas are highlighted. A subset of these affects the childhood population, and has historically been interpreted as malignant, although current evidence and outcome data suggest they represent benign entities. The growing current literature and the author's experience with difficult to classify borderline or 'hybrid tumors' are discussed and illustrated. Some of these classification gray zones occur with frequency in the gastrointestinal tract, an anatomical compartment that must always be entertained when examining these neoplasms. Other growing recent areas of interest include the heterogeneous group of pseudoneoplastic lesions involving peripheral nerve composed of mature adipose tissue and/or skeletal muscle, such as the enigmatic neuromuscular choristoma. Malignant peripheral nerve sheath tumors (MPNST) represent a diagnostically controversial group; difficulties in grading and guidelines to separate 'atypical neurofibroma' from MPNST are provided. There is an increasing literature of MPNST mimics which neuropathologists must be aware of, including synovial sarcoma and ossifying fibromyxoid tumor. Finally, we discuss entities that are lacking from the section on cranial and paraspinal nerves in the current WHO classification, and that may warrant inclusion in future classifications. In summary, although the diagnosis and classification of most conventional peripheral nerve sheath tumors are relatively straightforward for the experienced observer, yet borderline and difficult-to-classify neoplasms continue to be problematic. In the current review, we attempt to provide some useful guidelines for the surgical neuropathologist to help navigate these persistent, challenging problems. [ABSTRACT FROM AUTHOR]

Background: There is significant inter-observer variation amongst the neuro-pathologists in the typing, subtyping, and grading of glial neoplasms for diagnosis. Centralized pathology review has been proposed to minimize this inter-observer variation and is now almost mandatory for accrual into multicentric trials. We sought to assess the concordance between neuro-pathologists on histopathological diagnosis of glioblastoma. Materials and Methods: Comparison of local, institutional, and central neuro-oncopathology reporting in a cohort of 34 patients with newly diagnosed supratentorial glioblastoma accrued consecutively at a tertiary-care institution on a prospective trial testing the addition of a new agent to standard chemo-radiation regimen. Results: Concordance was sub-optimal between local histological diagnosis and central review, fair between local diagnosis and institutional review, and good between institutional and central review, with respect to histological typing/subtyping. Twelve (39%) of 31 patients with local histological diagnosis had identical tumor type, subtype and grade on central review. Overall agreement was modestly better (52%) between local diagnosis and institutional review. In contrast, 28 (83%) of 34 patients had completely concordant histopathologic diagnosis between institutional and central review. The inter-observer reliability test showed poor agreement between local and central review (kappa statistic=0.12, 95% confidence interval (CI): -0.03-0.32, P=0.043), but moderate agreement between institutional and central review (kappa statistic=0.51, 95%CI: 0.17-0.84, P=0.00003). Agreement between local diagnosis and institutional review was fair. Conclusions: There exists significant interobserver variation regarding histopathological diagnosis of glioblastoma with significant implications for clinical research and practice. There is a need for more objective, quantitative, robust, and reproducible criteria for better subtyping for accurate diagnosis. [ABSTRACT FROM AUTHOR]