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PubMed1996

Seeing the wood for the trees--the early papers of Denis Burkitt.

Denis Burkitt's was a remarkable career. A surgeon in the Colonial Medical Service in Uganda, his keen observation of the incidence of jaw tumours in children led him to describe a new form of lymphatic cancer. He confirmed, for the first time in man, that a cancer was caused by a virus and, most remarkable of all, he discovered a cure--low-dose chemotherapy. The condition was named after him--Burkitt's lymphoma--and with this came membership of a select group of eponymous Irishmen, including Graves, Colles and Corrigan. His ability to link the incidence of disease with geographical distribution brought him, first to question and then to champion Peter Cleave's theory that lack of dietary fibre contributed to many of the diseases common in western civilisation. In the process, he changed the dietary habits of the Western world. Yet Denis Burkitt's early career in Trinity College, Dublin was not auspicious for, as a Junior Freshman engineer in 1929, his tutor despaired of him, writing to his father 'Don't blame me if he gets stuck'. A happy irony then, that the College made him an Honorary Fellow half a century later.

Burkitt Lymphoma↗
PubMed1996

[J.M. Charcot 1825-1893. A life of labor].

Jean-Martin Charcot (1825-1893) is regarded as the father of clinical neurology. He was born in Paris. At the age of nineteen he began his medical studies at the University of Paris and after having passed his internship he continued his education in the Paris hospital system and successively passed from chief of clinic to physician to the hospitals of Paris and to professor at the Faculty of Medicine. Early in his academic medical career he was concerned chiefly with problems of internal medicine and produced lasting tributes to clinical and pathological understanding of especially rheumatism. His interests eventually settled to the study of disorders of the nervous system. In 1862 he was appointed physician to the Hospital of the Salpêtrière, which was to become the center of his discoveries and fame. The Salpêtrière, which had a long history as asylum and prison, was when Charcot arrived still a custodial institution for unclassified and displaced unfortunates, numbering about five thousand. Out of this hodge-podge Charcot developed in a few years the world's greatest center for clinical neurological research and introduced his famous clinico-pathological approach to the study of neurological disorders, by which he was able to classify, for the first time, a number of unknown diseases of the nervous system, the spinal cord as well as the cerebrum. Even to day his clinical descriptions of some of these disorders are unsurpassed, and they are still registrated with his name as an eponym. In 1882 a Chair of Clinical Diseases of the Nervous System, the first in medical history, was created for Charcot in the Salpêtrière. His fame as a scientist, practioner and especially as a teacher attracted students and patients from all parts of the world. He was an artist as well as a scientist, and he was the first to use projection material in medical teaching, using his own drawings as illustrations. ...

France↗
PubMed2001

[Fournier's gangrene].

Fournier's gangrene, although bearing the eponymous name of J.A. Fournier, was first reported by Baurienne in 1764 and 1883. His description was characterized by an abrupt onset of a rapidly progressive gangrene of the scrotum in otherwise healthy young males with an absence of a specific causative agent. Today, the frontiers of Fournier's gangrene have expanded to include all cases of necrotizing fasciitis of the perineum and external genitalia males and females from all age groups. All the patients were treated with antibiotics, surgical debridement and frequent wound dressings with hypertonis saline, hydrogen peroxide, colostomy and epicystostomy.

Adult↗
PubMed2001

Vulval intraepithelial neoplasia: current perspectives.

The heterogeneous clinical features of vulval intraepithelial neoplasia (VIN), its uncertain and variable natural history, difficulties in the management of certain cases and the frequency of recurrences provide a continuing challenge for gynaecologists. Patients with VIN present to a diverse range of physicians, all of whom provide differing perspectives on the multifarious issues relating to the condition. The importance of VIN relates principally to the symptoms it causes and its potential to progress to invasive vulval cancer. Both the International Society for the Study of Vulvovaginal Diseases and the International Society of Gynaecological Pathologists have stressed the importance of eliminating eponymous terminology and recommend only the term vulval intraepithelial neoplasia (VIN). This terminology includes both squamous and non-squamous varieties (Table 1). The latter includes both Paget's disease of the vulva and melanoma in situ. Non-squamous VIN will not be considered in this paper. Until 30 years ago VIN was an uncommon condition, seen principally in middle and later life. The incidence particularly in younger women has increased significantly since then [1-3]. Since that time the mean age in our unit has fallen from 52.7 years to 35.8 years (Figure 1) [2]. The increasing incidence of the condition parallels similar trends in cervical intraepithelial neoplasia (CIN) and relates at least in part to changing sexual morés, human papilloma virus (HPV) infection, and cigarette smoking.

Female↗
PubMed2002

Burckhard F. Kommerell and Kommerell's diverticulum.

Burckhard Friedrich Kommerell's scholarly description of the aortic diverticulum that bears his name was published in 1936. In the international literature, however the name of Kommerell survives only as an eponym. We present biographical information about Kommerell, as supplied by family members, and comment on the surgical relevance of his 1936 report.

Aorta, Thoracic↗
PubMedDate not supplied

[Psychiatry and the Great War].

During the World War I, the high rate of psychiatric casualties was differently tackled according to the nations: the Central Powers carried an authoritarian approach with prevailing physical treatment methods whereas the Allies' attitude reflected their democratic background. Particularly French psychiatry demonstrated a real willingness and ability to respond to the clinical realities. The conceptual problem of what DSM IV now classified as acute stress disorder was resolved so successfully that this disorder deserves the eponym "Viovenel's Syndrome". American Military Psychiatry followed the French methods of precise diagnosis and expeditious treatment close to the front and amplified them by creating effective treatment methods : brief psychotherapy methods and group psychotherapy within a therapeutic environment. Franco-American psychiatry thereby created the foundation for modern community psychiatry.

History, 20th Century↗
PubMed2002

Bell's palsy and herpesviruses.

A growing body of evidence links reactivation of herpesviruses (primarily varicella zoster virus and herpes simplex virus type 1) with the development of a large proportion of cases of acute peripheral facial palsy, a syndrome commonly known by its eponym, Bell's palsy. This article reviews the definition and natural history of the disease, its underlying anatomy and pathophysiology, the data linking herpetic reactivation with development of signs and symptoms, and therapeutic trials utilizing antiviral therapy. In addition, it poses the question, would earlier intervention with antivirals make a larger impact on outcomes?

Bell Palsy↗
PubMed2002

Royal naval physician Sir John Jamison (1776-1844), Scurvy and Sweden's medico-surgical institute.

As a direct consequence of Sweden's devastating losses in its war with Russia in 1808-9, an institute for the training of military surgeons was established in Stockholm in December 1810. This establishment soon became known as the Karolinska Institute and is the forerunner of today's eponymous institution. This paper records the nature of the British assistance that led indirectly to the founding of this institute. This aid took the form of a report into the high morbidity and mortality rates due to scurvy which were sustained by the Swedish Fleet in Carlscrona in the summer of 1808. This report, written by John Jamison, Fleet Physician to the Baltic Command of Sir James Saumarez, was used by the Stockholm medical authorities as part of their campaign for improved training of military medical personnel. Whilst Jamison's report did not in itself lead to the establishment of the Stockholm medico-surgical institute, it was undoubtedly important, and serves both as an example of Anglo-Swedish relations during the Napoleonic era and a reminder of the ravages of scurvy.

Academies and Institutes↗
PubMedDate not supplied

[Severe myoclonic epilepsy in infancy (Dravet's syndrome). Its nosological characteristics and therapeutic aspects].

AIMS: Severe myoclonic epilepsy in infancy (SMEI) is an epileptic syndrome recognised by the ICE of 1985 and 1989 and in the proposal put forward by the ILAE Task Force on Classification and Terminology in 2001. In this paper, its historical development, nosological characteristics and treatment are described. DEVELOPMENT: Although identified by Dravet in 1978, it has been called severe myoclonic epilepsy in infancy since 1981. As an alternative the name polymorphic epilepsy has also been put forward and in 2001 the ILAE recognised the eponym Dravet's syndrome. We describe how it may be mistaken for febrile convulsions in the early stages and later for Lennox Gastaut syndrome, Doose's myoclonic astatic epilepsy and certain progressive myoclonic epilepsies. We outline the risk factors, recognised in 1992, that facilitate an early diagnosis and the defining clinical criteria established in 1984. We point out the existence of atypical forms due to the absence of some of the defining criteria, which will never be above one, to formulate a diagnosis of SMEI. The frequency with which a family background of febrile convulsions and epilepsy appears seems to point to a genetic origin. Recently, de novo mutations have been found in the alpha subunit of the voltage dependent sodium channel as well as mutations in the gamma subunit of the GABAA receptor. Nosologically, it is located in group 3 of the 1989 ICE, which corresponds to epileptic syndromes without a focal determination, or which are generalised, and on the list of epilepsy/syndromes that was presented in 2001. SMEI is an epilepsy syndrome which is, in most cases, resistant to classical and new AED, and other more unusual treatment. The drugs that have proved to be more effective, although only relatively so, are topiramate, valproate and the benzodiazepines. At present another alternative that has appeared is stiripentol. Intravenous use of immunoglobulins can be useful. CONCLUSIONS: Dravet's syndrome, admitted as such by the ILAE in 2001 and probably caused by de novo mutations in the sodium channels or in the GABAA receptors, is one of the severest forms of epilepsy in infancy with very little or no response to current antiepileptic drugs. Those that have been seen to be most effective are topiramate, the benzodiazepines, valproate and, more recently, stiripentol.

Anticonvulsants↗
PubMed1992

["Preformation and prediction" of the human sex. Wilhelm Weinberg's critique of Otto Schöner's "theory"].

In 1909 Otto Schöner, a district medical officer, claimed human sex to be determined by ovarian preformation and to be precisely predicted. Up to his death in 1940 Schöner denied not only the role of sex chromosomes, but chromosomes as carrier of heritage in general. His "theory" of sex preformation in the human ovary and sex prediction was published in renowned journals, e.g. "Zentralblatt für Gynäkologie" and "Zeitschrift für Geburtshilfe und Gynäkologie". His statistics as well as the "theory" were criticized by Wilhelm Weinberg, a general practitioner, obstetrician and statistician. Weinberg's name has been firmly linked to a principle of population genetics by the eponym "Hardy-Weinberg-law". This paper is intended to give an extensive description of the controversy between Schöner and Weinberg.

Female↗
PubMedDate not supplied

[Klippel-Trenaunay-type syndromes].

Seeing the diversity of the clinical look and of the subjacent malformations of the Klippel and Trenaunay Syndrome the following questions can be put forward: must the term KT be kept up? If not how to replace it? The mesoblastic sheet includes angioblastic, lymphoblastic and osteoblastic lineages. Each of them gives rise to malformations. All the malformations and all the signs and symptoms they generate may exist alone or mixed, the diversity of association is unlimited. Hence the syndromes too. They cannot be gathered under the sole term of KT. On the other hand to give each of them an eponym would lead to confusion. Therefore the author proposes to limit himself to the use of the term "type KT" when getting in touch with. Once the syndrome is investigated accurately it must be specified by the causal malformations, the associated malformations and the secondary troubles. It is the only way to be understandable and to allow statistical research.

Arteriovenous Malformations↗
PubMed1992

Nuclear and infranuclear disorders.

Lesions of the brain stem can either affect the nuclei or the fascicles of the third, fourth or sixth cranial nerves and thus produce ocular motor disorders. Lesions of the oculomotor nuclear complex differ from lesions of the third nerve, since the motoneurones in the nucleus are specifically grouped. Similarly, a lesion of the sixth nerve nucleus results in a conjugate gaze palsy and not in an abducens palsy, because of 'interneurones' being intermingled with the abducens motoneurons. Isolated lesions of a nerve fascicle, which is the part of the cranial nerve running through the brain stem, usually cannot be distinguished clinically from lesions of the nerve outside the brain stem unless other brain stem signs are present. In the case of an isolated ocular motor nerve palsy, modern imaging techniques, particularly magnetic resonance imaging, may help to localize the lesion to the brain stem. Most often, however, brain stem lesions also involve structures surrounding the ocular motor nuclei or fascicles, sometimes leading to characteristic eponymic syndromes. In congenital eye movement disorders the pathoanatomical situation is more complex. Since the lesion takes place during intrauterine or early postnatal development, corrective misdirection of neurones occurs in addition to aplasia or hypoplasia of parts of the cranial nerves. Correspondingly, abnormal movements accompanying an attempted eye movement can be observed in some characteristic syndromes.

Abducens Nerve↗
PubMed1964

SUBJECT HEADINGS IN THE WELLCOME HISTORICAL MEDICAL LIBRARY.

The strength and unity of the medical library profession spring largely from the existence of the National Library of Medicine. Its publications promise to bring much closer the standardization and consistency still lacking to the world of medical subject headings. The Wellcome Library headings are similar to those listed in the National Library of Medicine Subject Heading Authority List of 1960. However, references and additional headings are made to cover (1) superseded terms, (2) historical phenomena with distinctive names which are peculiar to an epoch, (3) subjects from social, religious, or other branches of history, and (4) eponyms. Old texts often describe disease ambiguously or use terminology which has since been superseded. The catalogue is in three parts-topographical, biographical, and topical. The quarterly Current Work has certain simplifications (e.g., elimination of some subheadings), and has recently introduced references both to cut down needless duplication and to facilitate consultation by the reader.

England↗
PubMed1992

Pol André Bouin, MD (1870-1962). Bouin's fixative and other contributions to medicine.

Pol André Bouin was a distinguished French scientist who gained international reputation at the turn of the century for his work in histology and reproductive endocrinology. His name has been retained eponymously in the fixative he described in 1897, today known as "Bouin's fixative." The fixative that bears his name is only one of many contributions he made to academic medicine.

Endocrinology↗
PubMed1992

[Mucopolysaccharidoses. Genetics, clinical pathology, therapeutic regimes].

In recent years, several clinical and biochemical studies have been published contributing to better understanding of mucopolysaccharide storage diseases. Our purpose, therefore, is, to give an updated survey of this group of lysosomal diseases for diagnostic orientation to the pathologist. The present classification of mucopolysaccharidoses is based on a mixture of clinical symptoms, demonstration of enzyme defects, analysis of urinary excretion of glycosaminoglycans (mucopolysaccharides) and historical eponyms. A broad heterogeneity has been revealed: identical enzyme defects may lead to severe mental and physical deterioration and death during childhood or to mild forms with normal adult height, intelligence, and life expectancy. Conversely, identical phenotypes may result from mutations of different genes. Various types of mucopolysaccharidoses are mostly characterized by quantitative differences of main symptoms (coarse facial features, various skeletal abnormalities, hepatosplenomegaly, corneal clouding, mental retardation, and cardiac failure). Morphologically, storage material can be observed practically in all tissues by light and/or electron microscopy. Until recently, no more than palliative treatment could be offered. In the last decade, bone marrow transplantation has become available and may have beneficial effects in selected cases. The genes of several lysosomal enzymes have been identified, and comprehensive studies to introduce the gene into defective cells are currently undertaken. However, substantial efforts are still necessary until gene transfer will become available for patients with mucopolysaccharidoses.

Brain↗
PubMedDate not supplied

[From empiricism to neuroscience in Alzheimer's disease].

INTRODUCTION AND DEVELOPMENT: It has been almost a hundred years since neurofibrillar degeneration in the neurones of the cerebral cortex was first described by Alois Alzheimer. This finding led to the recognition of a new disease (a presenile dementia unlike senile dementia) using the clinicopathological empirical method employed at that time. Despite Alzheimer's own opinion and the subsequent findings from many neuropathological studies, general acknowledgement of the full implications of this dichotomy has taken decades. All the cases were grouped under the eponym of Alzheimer's disease and it is now being studied with the different modern scientific methods currently available to researchers. CONCLUSIONS: Alzheimer's disease has given rise to an exceptional associationist movement around the world and has become one of leading social and health problems facing the human race; its research and treatment will require the involvement of national and supranational public authorities.

Alzheimer Disease↗
PubMed2004

[Disorders of executive consciousness].

UNLABELLED: Executive function is a higher order cognitive capacity that involves memory, perception and performance of complex tasks. Disorders of the executive functions are sign of lesions in the prefrontal cortex, involving the prefrontal-striatal-thalamic networks and the parietal association areas. According to signs and localization, five basic prefrontal syndromes are recognised. 1. Damage in posterior dorsolateral prefrontal cortex and subcortical nuclei causes the dorsolateral syndrome with impaired decision making, working memory and planning. 2. The ventromedial-orbitofrontal syndrome: if lesion spares the basal forebrain, memory can be preserved, but poor social decision making develops. 3. The dorsomedial syndrome consists of attention disorder, akinesia, mutism and apathy. 4. The bilateral ventrolateral prefrontal regions serve perception of self and environment. 5. The ventral lateral (verbalizer) area of the dominant hemisphere coordinates language. Executive impairments can be found in cerebrovascular, Parkinson's and other diseases of basal ganglia, and in frontotemporal lobar degeneration. The dorsolateral syndrome can be examined by the use of Wisconsin card sorting test, self ordered pointing task and the delayed response task. Prefrontal-basal function can be assessed by Gambling-, Faux Pas-, and Emotion identification tasks. CONCLUSIONS: 1. A dysexecutive syndrome does not fulfil the criteria of dementia. 2. A "frontal syndrome" is an indefinite eponym. Focal lesions in prefrontal systems lead to localization-specific symptoms, which can be defined by psychometric tests. 3. In neurological diseases associated with multifocal damage of the brain neuropsychologic tests may help to determine strategic lesions, which are responsible for the actual syndromes.

Akinetic Mutism↗