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Luigi Luciani and the earliest graphic demonstration of Wenckebach periodicity.

Using an isolated frog heart preparation with ligatures around the atria, Luigi Luciani, an Italian physiologist working in 1873 in Carl Ludwig's famous laboratory in Leipzig, was the first to demonstrate cardiac group beating, which he named periodic rhythm. He attributed this to increased resistance to impulse propagation between the atria and the ventricle. Karel F. Wenckebach, in his 1899 landmark report of group beating in a patient in which he also used pulse tracings, credited Luciani with this discovery. Wenckebach referred to the phenomena as "Luciani periods." With the advent of electrocardiography in the early 20th century, this form of group beating became known as Wenckebach periodicity and then as Mobitz type I atrioventricular block. We reanalyzed Luciani's original paper and pulse tracings, and we show that periodic rhythm does indeed meet the criteria of second-degree atrioventricular block as established by Wenckebach. We also reviewed the career of Luciani, who was an important investigator, outstanding teacher and mentor, and distinguished leader of 19th-century physiology. We conclude that Wenckebach still deserves to have his name eponymously attached to this type of atrioventricular block because he was the first to unravel the complicated relationship between atrial and ventricular conduction.

Animals↗

The significance of cerebral white matter abnormalities 100 years after Binswanger's report. A review.

BACKGROUND: Changes in the cerebral hemispheric white matter are detected with increasing frequency by CT and MRI among persons older than 60 years. The pathogenesis, clinical significance, and morphological substrate of these changes are incompletely understood. Patients who have such neuroimaging abnormalities are sometimes diagnosed with "Binswanger's disease," an eponym that has generated much confusion because of its imprecise meaning. The objectives of this study were to determine whether the term Binswanger's disease merits acceptance as a distinct clinicopathologic entity, to deduce the clinical significance of these white matter abnormalities from the analysis of appropriate publications, and to evaluate studies that correlate in vivo changes in the cerebral white matter with pathological features. SUMMARY OF REVIEW: We evaluated Binswanger's original case description and, after conducting a Medline search, reviewed more than 160 publications, mostly in the English language, on the subject of white matter abnormalities detectable by currently used neuroimaging methods (ie, leukoaraiosis). CONCLUSIONS: Binswanger's original description appears to be insufficient for the purpose of defining a new nosological entity. After evaluating the vaguely outlined pathological correlates described in a few of these subcortical cerebral leukoencephalopathies, we conclude that the clinical significance of leukoaraiosis remains incompletely defined. However, its frequency increases with age independent of other risk factors, and in nondemented subjects leukoaraiosis is associated with deficits in selected cognitive functions. Moreover, leukoaraiosis correlates with an increased risk for the subsequent development of strokes. We make specific suggestions for future studies that may help to clarify this topic.

Aged↗

Motor nerve conduction study in Raynaud's phenomenon.

The term Raynaud's phenomenon is the eponym of a clinical picture culminating into and dominated by accessorial vasomotor crysis which are triggered by different stimuli (cold, cigarette smoking, emotional stress); cold sensitivity however, is a rather constant feature. Little is known about peripheral nerve involvement in Raynaud's phenomenon but for cases secondary to thoracic outlet syndrome or to primary neurologic diseases, systemic or not. The aim of the present work was to evaluate non-invasively the involvement of arterial flow as well as of nerve function in subjects complaining of Raynaud's phenomenon.

Adult↗

Cervical necrotizing fasciitis: an uncommon sequela to dental infection.

Necrotizing fasciitis is a soft-tissue infection, usually polymicrobial, that causes necrosis of fascia and subcutaneous tissue while sparing skin and muscle. Although it more commonly involves the groin, abdomen, and extremities, it may also occur in the head and neck, usually secondary to dental infection. We report a case of cervical necrotizing fasciitis arising from a dental infection and review the cause, pathophysiology, diagnosis, and treatment of this potentially lethal entity. Early detection and interventions is emphasized. Extensive surgical debridement remains the mainstay of treatment. In addition, a clarification of the various eponyms it has gone under in the past is offered.

Adult↗

Disorders of sexual preference, or paraphilias: a review of the literature.

By their very nature, disorders of sexual preference (ICD-10) or paraphilias (DSM IV) are bizarre patterns of sexual behaviour that have diverse manifestations and are of complicated sexual orientation. Some are harmless, others are sad, robbing sufferers and possibly their partners of loving sexual relationships. Psychopathology within this group of disorders may lead to criminal behaviour, ranging from infringement of decency to some of the most heinous crimes known. In a lifetime of practice no professional in any sphere of law or medicine will see a full cross-section of these conditions, as some are rare and they do not all present to one professional group. The nomenclature is confusing, with its Greek, Latin, French and Portuguese origins, together with eponymous terms. At least 40 different paraphilias have been named (Money, 1988) but these do not cover the full extent of the field.

Forensic Psychiatry↗

The neurological assault on the great toe (1893-1911).

The late 19th/early 20th century heralded a period in neurology when there was claim and counter claim about the founders of new reflex movements of the great toe. The cutaneous plantar reflex is undoubtedly one of the most important signs in clinical neurology, and there have been several methods described of eliciting this sign, each with its own eponymous name. This article reviews the background to these claims and the contribution of the various authors, to the discovery, clinical relevance and ease of eliciting reflex movements of the great toe.

Germany↗

Diagnosis and management of drop attacks of vestibular origin: Tumarkin's otolithic crisis.

Erroneous signals of vestibular origin can cause sudden falls without warning. Drop attacks of vestibular origin in our experience most commonly occurred in patients with late or end-stage endolymphatic hydrops (usually idiopathic, ie, Meniere's disease). Eponymically termed Tumarkin's otolithic crisis, drop attacks of vestibular origin must be distinguished from drop attacks due to cardiovascular abnormalities, seizure disorders, vertebral basilar arterial insufficiency (transitory brain stem ischemia), multiple sclerosis, and drug-induced motor control disturbances. The treatment of choice for drop attacks of vestibular origin is either surgical ablation (labyrinthectomy) in the absence of serviceable hearing or middle fossa vestibular nerve section in order to preserve serviceable hearing.

Cardiovascular Diseases↗

A review of the special tests associated with shoulder examination. Part I: the rotator cuff tests.

Careful examination of the shoulder is an essential component in forming a diagnosis of problems in this area. A number of tests have been described that are claimed to improve diagnostic accuracy by specifically examining one component of the shoulder complex. Many of these tests are eponymous, and there is confusion about not only how to perform them but also what conclusion to draw from the results. This article attempts to clarify the tests used to examine the rotator cuff by presenting them as described by the original authors with the additional aim of providing a source for those wishing to refresh their knowledge without the need to refer to the original source material.

Humans↗

Erotomania: a conceptual history.

Four historical convergences are identified in the history of erotomania. According to the first, which lasted from Classical times to the early eighteenth century, erotomania was a 'general disease caused by unrequited love'. According to the second, erotomania was a disease of 'excessive physical love (nymphomania)'; this view remained active well into the nineteenth century. The third convergence focuses on the view that erotomania is a form of 'mental disorder', and this was held throughout the twentieth century. The fourth and current definition (a development of the third stage) sees erotomania as a 'the delusional belief of being loved by someone else'. Differences between Anglo-Saxon and French views cast doubt on the meaning or coherence of the much-abused English eponym 'de Clérambault syndrome'. Erotomania is a construct, a mirror reflecting Western views on spiritual and physical love, sex, and gender inequality and abuse. On account of this, it is unlikely that there will ever be a final, 'scientific' definition rendering erotomania into a 'natural kind' and making it susceptible to brain localization and biological treatment.

History, Ancient↗

The life and work of the Birmingham radiologist Dr James Brailsford (1888-1961).

James Brailsford went from humble beginnings to become an eminent radiologist through his ability, determination and immense diligence. He made a late start in medicine but became one of the world's leading authorities on skeletal diseases; he wrote the classic textbook The Radiology of Bones and Joints and described the eponymous Morquio-Brailsford syndrome. In 1934 he was elected first President of the British Association of Radiologists, of which he was founder. This later became the Faculty of Radiologists at the Royal College of Surgeons and is now the Royal College of Radiologists.

History, 19th Century↗

New discoveries in anatomy and new terminology of leg veins: clinical implications.

Advances in diagnosis and in endovascular and surgical treatments of both acute and chronic venous diseases have focused attention on several, previously clinically less important, details of venous anatomy. The time has come to standardize venous nomenclature so that general practitioners and specialists in the United States and abroad speak the same language. There is a need to adopt a universal terminology for the saphenous veins, to avoid the myriad of eponyms of perforators and, most importantly, to omit once and for all, the term "superficial" femoral vein when talking about a deep vein of the thigh. New anatomic names, such as the saphenous fascia and saphenous subcompartment, have to gain better recognition. To achieve the desired consensus on venous nomenclature, an international meeting was held in Rome, Italy, in 2001, under the auspices of the International Union of Phlebology. In this review on venous anatomy, we describe the most important previously published clinical recommendations from this conference, and suggest modifications based on their clinical experience using the new terminology.

Femoral Vein↗

Syndromes with congenital brittle bones.

BACKGROUND: There is no clear definition of osteogenesis imperfecta (OI). The most widely used classification of OI divides the disease in four types, although it has been suggested that there may be at least 12 forms of OI. These forms have been named with numbers, eponyms or descriptive names. Some of these syndromes can actually be considered congenital forms of brittle bones resembling OI (SROI). DISCUSSION: A review of different syndromes with congenital brittle bones published in the literature is presented. Syndromes are classified in "OI" (those secondary to mutations in the type I pro-collagen genes), and "syndromes resembling OI" (those secondary to mutations other that the type I pro-collagen genes, identified or not). A definition for OI is proposed as a syndrome of congenital brittle bones secondary to mutations in the genes codifying for pro-collagen genes (COL1A1 and COL1A2). SUMMARY: A debate about the definition of OI and a possible clinical and prognostic classification are warranted.

Collagen↗

Phosphorylation of RNA polymerase II in cardiac hypertrophy: cell enlargement signals converge on cyclin T/Cdk9.

Cardiac myocyte enlargement is the eponymous characteristic of cardiac hypertrophy, regardless of the instigating signal. Such triggers include biomechanical stress (e.g., work load, compensation for ischemic damage), sarcomeric protein mutations, cytoskeletal protein mutations, abnormal energetics, G protein-coupled receptors for ligands (including angiotensin II and endothelin-1), or their signal transducers within cells. In turn, increased myocyte size reflects increased RNA and protein content per cell as responses to these stimuli. In eukaryotic cells, the large subunit of RNA polymerase II (RNAPII) becomes extensively phosphorylated in its serine-rich C-terminal domain (CTD) during the transition from transcript initiation to transcript elongation - that is, "escape" of RNAPII from the promoter-proximal region into the open reading frame. Although this process is believed to be crucial to productive synthesis of mRNA and is known to be governed by two atypical cyclin-dependent kinases, Cdk7 and Cdk9, surprisingly little is understood of how regulatory pathways within cells intersect these RNAPII-directed protein kinases. Investigations of the CTD kinase module in cardiac hypertrophy provide a tentative initial map of a molecular circuit controlling cell size through regulated phosphorylation of RNAPII.

Animals↗

Sex-linked sudanophilic leukodystrophy with adrenocortical atrophy (so-called Schilder's disease). Report of a case and review of the literature.

We report the clinical and pathologic features of a case of sex-linked sudanophilic leukodystrophy in an 8-year-old boy. Our survey of the pertinent literature on this disease supports the current view that it is not limited to the central nervous system, but is a generalized process, probably caused by an unknown metabolic defect, with a definitive genetic pattern and varying expressions. Although the disease was originally described by Schilder as a single entity, cases like ours show that the term "sudanophilic leukodystrophy" represents a better classification than the usually misinterpreted eponym, "encephalitis periaxialis diffusa."

Adrenal Cortex↗

Slow spike-wave activity in EEG and associated clinical features: often called 'Lennox' or "Lennox-Gastaut' syndrome.

Clinical features were studied in 83 patients with slow spike-wave activity in the EEG. Epileptic seizures, usually intractable, occurred in 82 patients. The majority had the onset of seizures during the first 2 years of life. Minor motor seizures alone or in combination with other types of seizures occurred in 80 percent, and most had more than one type of seizure. The combination of tonic-clonic, minor motor, and absence seizures was the commonest, occurring in 37 percent. Sixty-six patients were definitely retarded and 49 showed definite motor impairment. Etiologic factors responsible for cerebral insult were identified in 53 patients. Serial EEG studies showed a close relationship between the EEG patterns of hypsarrhythmia, independent multifocal spike discharges, and slow spike-wave activity. The eponym "Lennox-Gastaut" syndrome is appropriate for a patient who has slow spike-wave activity in the EEG, exhibits mental retardation, and has intractable seizures of various types. However, the syndrome does not imply a pathologic entity because many diverse processes, both static and progressive, can produce this syndrome.

Adolescent↗

Benign paroxysmal positioning vertigo: classic descriptions, origins of the provocative positioning technique, and conceptual developments.

The original description of benign paroxysmal positioning vertigo (BPPV) has been variously attributed to Bárány, Adler, and others. In addition, the proper eponymic designation for the provocative positioning test used to diagnose BPPV has been unclear, because authors use a variety of different terms, including Bárány, Nylén-Bárány, Nylén, Hallpike, Hallpike-Dix, and Dix-Hallpike to refer to the procedure in current use. Based on a review of the extant medical literature, Bárány was the first to describe the condition in detail, and Dix and Hallpike were the first to clearly describe both the currently used provocative positioning technique and the essential clinical manifestations of benign paroxysmal positioning vertigo elicited by that technique. Nevertheless, despite their important contributions, neither Bárány nor Dix and Hallpike understood the pathophysiology of BPPV nor did they appreciate that the positioning techniques they used actually demonstrated pathology in the semicircular canals rather than the utricle. The modern understanding of the pathophysiology of BPPV began with Schuknecht's proposal that the dysfunction resulted from the gravity-dependent movement of loose or fixed dense material within the posterior semicircular canal ("cupulolithiasis"). Although Schuknecht's formulations were not consistent with all clinical features of the disease, they led to the modern "canalolithiasis theory" and highly effective canalith repositioning or "liberatory" maneuvers for BPPV.

History, 20th Century↗

A history of Todd and his paralysis.

OBJECTIVE: To describe the history of Robert Bentley Todd (1809-1860) and certain of his contributions to medicine, including his original and subsequent descriptions of "epileptic hemiplegia," which came to be called "Todd's paralysis." METHODS: The author conducted a comprehensive review of English-language literature, modern and historical, related to "Todd's paralysis" and examined Todd's original case histories and commentary by Todd, his contemporaries, and his successors. RESULTS: Todd held that some patients "who recover from a severe fit, or from frequently repeated fits of epilepsy, are often found to labor under hemiplegia, or other modifications of palsy." He believed that this resulted from "undue exaltation. [resulting in] a state of depression or exhaustion." Interestingly, Todd was the first to present an electrical theory of epilepsy, supported by his own animal experimentation, well before his better-known successor John Hughlings Jackson (1835-1911) (famous for his investigations of partial epilepsies and the eponymous "Jacksonian march"). CONCLUSION: Many neurologists and investigators followed Todd in acknowledging transient postictal paralysis as a distinct clinical entity. Yet whether the pathophysiology of "Todd's paralysis" is related to "neuronal exhaustion" or excessive inhibition is still controversial.

History, 19th Century↗

Retracing "Ondine's curse".

"Ondine's curse" is a term used to denote a rare neurological condition causing failure of automatic respiration. The patients are no longer capable of breathing spontaneously-they must consciously and voluntarily force themselves to do so. Ondine (also known as "Undine"), a mythological figure of European tradition, was a water nymph or sprite who could become human only when she fell in love with a mortal man. However, if the mortal was unfaithful to her, he was destined to forfeit his life. In the 16th century, Paracelsus coined the term "Undine" to describe the spirit that inhabited the element of water. Baron de la Motte-Fouque wrote the story of Undine in the late 18th century. It has since become a popular subject for theater productions. Jean Giraudoux, the French playwright, introduced the concept of the loss of automaticity of all functions as the "curse of Ondine." The legend was popularized in the form of the fairy tale "The Little Mermaid" by Hans Christian Andersen and as an animated motion picture by Walt Disney Productions. In this study, we look at the origins of this eponymous term, the personalities intertwined with its popularity, and its misrepresentations in the medical literature.

Humans↗