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Prognostic factors in juvenile idiopathic arthritis.

Prognostic factors in juvenile arthritis are related to many variables that must be evaluated according to the different subtypes. The International League of Associations of Rheumatologists (ILAR) recently proposed six different categories referred to as the Durban criteria, under the eponym of juvenile idiopathic arthritis (JIA). The aim of this classification was to define homogeneous groups according to their clinical and biologic features. The prognostic factors were classified into the different categories of JIA. A poor outcome in the systemic form correlated with markers of disease activity, such as fever and polyarticular involvement, within the first 6 months. The risk of joint destruction in oligoarthritis correlated with the severity of arthritis within the first 2 years. Polyarthritis with positive rheumatoid factor is associated with marked disability in adulthood. In a group of psoriatic patients, the risk of developing sacroiliitis is higher in male and HLA-B27-positive patients. Patients with enthesitis-related arthritis with lower limb, knee, and tarsal involvement also are at greater risk of developing sacroiliitis. Chronic uveitis is a complication of JIA observed mainly in patients with oligoarthritis associated with positive antinuclear antibodies in serum. Secondary amyloidosis is observed mainly in children with systemic JIA. The long-term outcome must be discussed according to the various therapies. Corticosteroids contribute to growth retardation and osteoporosis, for which the use of human recombinant growth hormone and biphosphonates may be an option. Newer encouraging therapies such as anticytokines have been proposed for children with active disease. Autologous stem cell transplantation is being evaluated in some centers with promising results; however, it has a high rate of mortality. Further discussion regarding which patients should undergo autologous stem cell transplantation is needed, as is further discussion regarding the technical adaptations necessary.

Adolescent↗

Johann Friedrich Meckel, the Younger (1781-1833).

Meckel's name is well known in the medical world because of the intestinal abnormality that carries his eponym. We are less familiar with his personal life and his far-reaching achievement as a prominent anatomist. Also described is his complex personality as well as the indelible impression he made on his contemporaries.

General Surgery↗

A new identity for misidentification syndromes.

The systematic study of delusional misidentification syndromes has been hampered by the often confusing and imprecise eponymic nomenclature currently in use. We present a case report that demonstrates the inadequacies of the current system. We then propose a new classification system designed to improve research and understanding in this important area.

Aged↗

Multifocal papilloma virus epithelial hyperplasia.

Multifocal papilloma virus epithelial hyperplasia is an infection of the oral mucosa produced by human papilloma virus types 13 and 32, which primarily bilaterally affects lips, lateral borders of tongue, and buccal mucosa. The attached oral mucosa, floor of mouth, soft palate, and oropharynx are sites that appear not to be affected. This study comprises 110 patients with multifocal papilloma virus epithelial hyperplasia identified over a period of 3 years in Guatemala City and neighboring rural areas. All but four patients were younger than 18 years of age. More than one affected patient was observed in several families. All but three cases occurred in patients living in extreme poverty (annual family income less than $1,200 in U.S. dollars). Biopsies of 60 lesions demonstrated acanthosis and nuclear degeneration within the epithelium. We suggest that the eponym Heck's disease be abandoned because this disease was described in the Latin-American literature before the initial description in the American literature. We propose the term multifocal papilloma virus epithelial hyperplasia, which best describes the nature and multifocality of this disease.

Adolescent↗

Anterior chamber cleavage syndrome. A stepladder classification.

This paper classifies the abnormalities of the anterior chamber cleavage syndrome (mesodermal dysgenesis of the iris and cornea). The anatomic findings are arranged in a tabular stepladder fashion which builds from simple to more complex combinations, most of which have been previously known by eponyms. There are three groups of anomalies: 1) peripheral, 2) central, and 3) combinations of the two. 1) The peripheral anomalies consist of a prominent Schwalbe's ring, iris strands to Schwalbe's ring, and hypoplasia of the anterior iris stroma. Developmental glaucoma is commonly present. 2) The essential feature of the central anomalies is a defect in the corneal endothelium and Descemet's membrane with an overlying corneal opacity. Additional components include central iridocorneal adhesions, keratolenticular approximation with cataract, and scleralizaiton of the cornea. Chorioretinal anomalies, developmental glaucoma, and systemic malformations may be present. 3) Central and peripheral combinations may exist in the same eye, in both eyes of the same patient, or within the same family.

Abnormalities, Multiple↗

The identity of lacunar dementia and Binswanger disease.

It is postulated here that the dementia occurring in patients with lacunar strokes is due to an ischemic leukoencephalopathy. Severity of the dementia correlates, not with the volume of brain tissue lost from large-artery infarctions, but rather, with the extent of cortex disconnection resulting from demyelination. The term Lacunar Dementia is proposed, instead of the poorly known eponym "Binswanger disease" or the cumbersome descriptive name "subcortical arteriosclerotic encephalopathy." Clinically, Lacunar Dementia presents with gait difficulties, urinary incontinence, parkinsonian features, pseudobulbar palsy, emotional incontinence and dementia. High-resolution CT scan shows decrease density of frontal and periventricular white matter, without contrast enhancement; ventricular dilation, and lacunar infarcts. Small-artery disease lipohyalinosis is the cause of the lacunes and the leukoencephalopathy. Since the advent of the high-resolution CT scan, the frequency of lacunar dementia seems to be increasing, in contrast with the number of cases of multi-infarct dementia.

Cerebral Cortex↗

C.S. Campbell: Gamekeeper's thumb.

Rupture of the ulnar collateral ligament of the metacarpophalangeal joint of the thumb is an injury frequently encountered by the emergency physician. The mechanism of injury is usually a fall onto the abducted thumb. The setting is frequently one of athletic endeavor, especially skiing, in which this is the most commonly encountered upper extremity injury. The physical findings typical of collateral ligament rupture may easily be overlooked on casual examination. When not appropriately managed, the injury may result in chronic pain, weakness, and joint instability. The eponym "gamekeeper's thumb" derives from a report of similar injuries described as occurring in a series of British gamekeepers.

Finger Injuries↗

Nicotine: from molecular mechanisms to behaviour.

The addictive potential of nicotine is clearly recognized by the tenacity of tobacco smoking for most users, and has prompted extensive psychopharmacological studies in animals. In parallel, the interaction of nicotine with the many subtypes of its eponymous receptor has been the focus of molecular and cellular investigations. More recently, a convergence of these approaches has been stimulated by the generation of transgenic animals, which facilitates analysis of the impact of molecular changes on behaviour. Nicotine, like other addictive drugs including psychomotor stimulants, promotes dopamine release in the nucleus accumbens. This transmitter system has been a major focus of both neurochemical and behavioural investigations, although recently the pre-eminence of this system in nicotine dependence has been challenged. Complexities in the brain circuitry (including the subdivisions of the nucleus accumbens) and differences between behavioural models help to rationalise the current controversy.

Animals↗

Essentials of shoulder MR arthrography: a practical guide for the general radiologist.

Lesions of the glenoid labrum and ligamentous structures commonly occur secondary to shoulder trauma and are a frequent cause of shoulder joint instability. Numerous eponyms, acronyms and subclassifications are used to describe the often confusing array of bony and labro-ligamentous abnormalities of the shoulder. This aim of this review is to illustrate the relevant features of these lesions and to provide a systematic and practical approach to imaging of the shoulder using MR arthrography.

Humans↗

New techniques for the evaluation and therapeutic planning of patients with Klippel-Trénaunay syndrome.

BACKGROUND: Klippel-Trénaunay syndrome (KTS) is a well-known eponym for a capillary-lymphatic-venous malformation which is associated with soft tissue and skeletal hypertrophy, usually of one or more limbs. Plain films, sonograms, conventional venograms, and arteriograms have been employed for the evaluation of the disease. OBJECTIVE: To demonstrate the usefulness of multidetector computed tomography (MDCT) and fast 3-dimensional magnetic resonance imaging (3D-MR) venography for the assessment and therapeutic planning of patients with KTS. METHODS: A prospective study in 16 consecutive patients with KTS using MDCT and 3D-MR venography, performed between January 2004 and January 2006 in a university hospital in Pamplona, Spain. RESULTS: In nearly all patients, persistent embryologic veins were observed, and in one subject aplasia/atresia of the whole deep venous system of the affected extremity was seen. In four individuals hypoplasia of the femoral vein was observed; one subject had duplication of the femoral vein, and in three patients aplasia/atresia of this vein was found. Only half of the patients had normal popliteal veins. In one patient, aneurysmal dilatation of the popliteal vein was detected, and in six subjects, aplasia of this vein was observed. The presence of geographic stains was suggestive of hypoplasia and/or aplasia of femoral and popliteal veins. LIMITATIONS: The small size of the group of patients with KTS, which is related to low incidence of the disease. CONCLUSIONS: MDCT and 3D-MR venography are extremely helpful for the global evaluation of patients with KTS. Information regarding soft tissue and bony anatomy as well as information about superficial and deep venous systems may be obtained with a single exam.

Adolescent↗

Finkelstein's test: a biomechanical analysis.

PURPOSE: Finkelstein's test is the classic diagnostic test for de Quervain's disease. Finkelstein hypothesized that the entry of the muscle bellies of the extensor pollicis brevis (EPB) and abductor pollicis longus (APL) tendons into the first extensor compartment was responsible for the findings observed in his now eponymous test. We agree with Finkelstein's hypothesis and further hypothesize that this position would induce measurable bulk (muscle mass within the retinaculum) and tethering (stretching of synovial tissue) effects within the compartment. To test this latter hypothesis we measured the excursion and gliding resistance of the EPB and APL tendons within the first compartment. METHODS: Fifteen fresh-frozen cadavers were used. Gliding resistance and excursion were measured in 4 different wrist positions, including the wrist position of Finkelstein's test (30 degrees ulnar deviation). The bulk and tethering effect was calculated based on the mean gliding resistance over the tendon proximal/distal excursion cycle and the gliding resistance at the terminal distal excursion. RESULTS: The EPB tendon excursion was significantly more distal in 30 degrees ulnar deviation than in 60 degrees extension. Additionally the bulk and tethering resistance was significantly greater in 30 degrees ulnar deviation compared with 60 degrees extension. For the APL tendon there was no significant difference in either the tendon excursion or the bulk and tethering resistance between 30 degrees ulnar deviation and 60 degrees extension. CONCLUSIONS: We showed that in the position of Finkelstein's test the EPB tendon is significantly more distal and has significantly greater bulk and tethering effect compared with the other EPB positions. This is not the case for the APL tendon in the position of Finkelstein's test. These results suggest that an abnormal Finkelstein's test reflects differences of the EPB more than it does the APL.

Aged↗

The CH-domain of calponin does not determine the modes of calponin binding to F-actin.

Many actin-binding proteins have been observed to have a modular architecture. One of the most abundant modules is the calponin-homology (CH) domain, found as tandem repeats in proteins that cross-link actin filaments (such as fimbrin, spectrin and alpha-actinin) or link the actin cytoskeleton to intermediate filaments (such as plectin). In proteins such as the eponymous calponin, IQGAP1, and Scp1, a single CH-domain exists, but there has been some controversy over whether this domain binds to actin filaments. A previous three-dimensional reconstruction of the calponin-F-actin complex has led to the conclusion that the visualized portion of calponin bound to actin belongs to its amino-terminal homology (CH) domain. We show, using a calponin fragment lacking the CH-domain, that this domain is not bound to F-actin, and cannot be positioning calponin on F-actin as hypothesized. Further, using classification methods, we show a multiplicity in cooperative modes of binding of calponin to F-actin, similar to what has been observed for other actin-binding proteins such as tropomyosin and cofilin. Our results suggest that the form and function of the structurally conserved CH-domain found in many other actin-binding proteins have diverged. This has broad implications for inferring function from the presence of structurally conserved domains.

Actins↗

"The Epilepsies": Kinnier Wilson's landmark epileptology.

Kinnier Wilson, better known for his eponymous disease, in fact devoted much of his career to the study of epilepsy. In his long campaign to alter the general perception of epilepsy, he spent much time and effort decrying the use of "epilepsy" as a single disease, pleading for individual consideration for its sufferers. In addition, he undertook an extensive reconsideration of many of the basic principles of his mentor and friend the great John Hughlings Jackson. Eventually his status as the European expert in epilepsy earned him the signal honour of authorship (in English) of the important chapter entitled "The Epilepsies" in the 1935 flagship German language textbook of neurology that appeared immediately prior to the advent of the EEG. This chapter and the one in his posthumously published textbook of 1940 are landmarks of epileptology of that era.

Epilepsy↗

Nomenclature of the veins of the lower limb: extensions, refinements, and clinical application.

The relative deficiency of the official Terminologia Anatomica with regard to the veins of the lower limbs was responsible for a nonuniform anatomic nomenclature in the clinical literature. In 2001, an International Interdisciplinary Committee updated and refined the official Terminologia Anatomica regarding the veins of the lower limbs. Recommendations for terminology were included in an updating document that appeared in the Journal of Vascular Surgery (2002;36:416-22). To enhance further the use of a common scientific language, the committee worked on the present document, which includes (1) extensions and refinements regarding the veins of the lower limbs; (2) the nomenclature of the venous system of the pelvis; (3) the use of eponyms; and (4) the use of terms and adjectives of particular importance in clinical vascular anatomy.

Humans↗

Donald O. Hebb's synapse and learning rule: a history and commentary.

This year sees the anniversary of Donald O. Hebb's birth, in July 1904. The impact of his work, especially through his neurophysiological postulate, as described in his magnum opus, The organization of behaviour (1949), has been profound in contemporary neuroscience. Hebb's life, and the scientific milieu in psychology and neurophysiology which preceded and informed Hebb's work are described. His core postulate, which gave rise to such eponymous expressions as the Hebbian synapse and the Hebbian learning rule, is examined in some detail, as well as the part it played in his higher-order theoretical constructs concerned with neocortical structure and function. Early models which made use of the Hebbian synapse are described, and then illustrative examples are given detailing the impact of Hebb's idea in relation to learning and memory, synaptic plasticity and stability, and the question of persistent cortical activity underlying forms of short-term memory.

Animals↗

A hundred years of Alzheimer's disease research.

On the 100th anniversary of Alzheimer's lecture describing the clinicopathological entity which bears his eponym, this article reviews the major areas of progress in our understanding of the disease and outlines the many gaps still remaining. The progress toward effective mechanistic therapy is reviewed.

Alzheimer Disease↗

Ocular manifestations of familial adenomatous polyposis (Gardner syndrome).

Familial adenomatous polyposis (FAP) is a colon cancer predisposition syndrome in which hundreds to thousands of precancerous colonic polyp become evident at a mean age of 16 years (range, 7-36 years). By age 35 years, 95% of patients have polyps. Gardner syndrome is the eponym given to a subgroup of FAP with extracolonic manifestations, such as pigmented ocular fundus lesions that resemble congenital hypertrophy of the retinal pigment epithelium, among others.

Fluorescein Angiography↗

Spindle cell oncocytoma of the adenohypophysis: report of a case with a 16-year follow-up.

Spindle cell oncocytoma (SCO) is a recently described, rare neoplasm of the anterior pituitary. Clinically and radiologically simulating a non-functioning macroadenoma, its eponymous fusiform cells display a non-epithelial phenotype with conspicuous cytoplasmic accumulation of mitochondria. We report a case of SCO retrospectively identified in a biopsy specimen 16 years after transsphenoidal operation of a 48-year-old woman. Presenting symptoms were adynamia and transient decrease of visual acuity. Neuroimaging showed an isointense, enhancing, sellar-centered mass 1.8 cm in diameter without evidence of invasive growth. No postoperative adjuvant therapy was administered. The patient was left with panhypopituitarism, yet no recurrence was seen during follow-up. Initially diagnosed as a null cell adenoma of oncocytic type, repeat immunohistochemistry showed the characteristic coexpression of S100 protein, vimentin, and epithelial membrane antigen. Oncocytic granula stained intensely with antimitochondrial antibody 113-1, and were negative with the lysosomal marker CD68. Anterior pituitary hormones tested negative, and there was no evidence of neuroendocrine differentiation using antibodies to synaptophysin and chromogranin. Few cells stained for glial fibrillary acidic protein (GFAP). SCO has been proposed to represent a neoplasm of folliculo-stellate cells (FSCs). While the dynamic properties of the latter are incompletely characterized, and indeed no specific marker allows for their identification, overlapping features of SCO with look alikes, in particular pituicytoma, point to FSCs being a potential adult stem cell. The favorable outcome of the present case further argues for SCO to be considered a low-grade neoplasm. Moderate tumor size, lack of invasiveness, and low proliferation rate are likely predictors of benign behavior.

Adenoma, Oxyphilic↗