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At least 595 records · Page 33Linked to original sources
PubMed1991

Panniculitis: a report of four cases and literature review.

Panniculitis is a disease with many causes and associations. The classification of clinical subtypes is unsatisfactory and hampered by the use of eponyms. Four children with recurring panniculitis are described and their histology presented. Three had subcutaneous fat atrophy with lobular panniculitis on biopsy; all responded well to corticosteroids. The fourth child had a septal panniculitis with no atrophy of subcutaneous tissues and only a partial response to treatment with corticosteroids. A widely accepted precise histological classification of panniculitis is needed to enable accurate predictions of the outcome of this serious disorder.

Adipose Tissue↗
PubMed1976

Chronic spinal muscular atrophy of facioscapulohumeral type.

Chronic spinal muscular atrophy of FSH type affecting a mother and her son and daughter is reported. The relevant literature is reviewed and the relation between this conditon and Kugelberg-Welander (K-W) disease is discussed. Chronic spinal muscular atrophy of FSH type is considered to be a different entity from the eponymous K-W disease. Each type of muscular dystrophy, e.g. limb-girdle, FSH, distal, ocular, or oculopharyngeal type, has its counterpart of nuclear origin. A classification of the chronic spinal muscular atrophies is suggested following the classification of muscular dystrophy.

Action Potentials↗
PubMed2002

Raeder's syndrome [corrected]: paratrigeminal paralysis of the oculopupillary sympathetic system.

Raeder described five patients with mixed features of trigeminal nerve pathology and oculosympathetic impairment, with or without other cranial nerve lesions. This constellation of clinical features drew the original author's attention to the paratrigeminal region as a likely site for the causative lesion in this syndrome. An analysis of the anatomy of the oculosympathetic innervation supports the view that a restricted lesion in the middle cranial fossa might cause the syndrome of trigeminal nerve involvement, neuralgic pain or sensory change, with ptosis or miosis, or both, but no anhidrosis. Such a paratrigeminal oculosympathetic syndrome (POSS) usefully reminds clinicians to pursue vigorously possible lesions of the middle cranial fossa with careful, and possibly repeated, imaging studies. Attaching the eponym Raeder's syndrome or Raeder's paratrigeminal neuralgia to this syndrome adds nothing valuable to the anatomical description (POSS), which might be preferred for clarity.

Adolescent↗
PubMed2003

"Osler's phenomenon": misdiagnosing Cushing's syndrome.

The clinical manifestations of Cushing's syndrome can be quite variable and are frequently mistaken, with consequent delayed diagnosis and significant morbidity and mortality. Harvey Cushing described the typical signs and symptoms of Cushing's syndrome but unfortunately attributed the features to myxoedema. The first typical description of a patient with Cushing's syndrome was probably made by Sir William Osler in 1898. Thus delay or misdiagnosis with consequent high morbidity and mortality exemplifies the history of Cushing's syndrome. Four cases of Cushing's syndrome are described that were associated with deteriorating morbidity because of the considerable delay from first presentation to a secondary care physician to eventual diagnosis. The clinical diagnosis was delayed in all the four patients, although they had symptoms and signs that were missed by a number of primary and secondary care physicians. Trans-sphenoidal surgery resulted in biochemical cure as well as improvement in the accompanying co-morbidity. Although still rare, the prevalence of Cushing's syndrome is increasing. Increasing clinical awareness and the use of appropriate screening tests should facilitate earlier diagnosis with reduced morbidity and mortality. Although the syndrome is named after Harvey Cushing, Sir William Osler was probably the first to describe it. Therefore, in deference to Osler's contribution to Cushing's syndrome and the work of Harvey Cushing, it is suggested that to the list of the other eponymous conditions of Osler-Weber-Rendu and Osler's nodes, should be added the delay or misdiagnosis of Cushing's syndrome-"Osler's phenomenon".

Cushing Syndrome↗
PubMed1981

Joseph Skoda 1805-81: a centenary tribute to a pioneer of thoracic medicine.

Joseph Skoda was born on 5 December 1805 in Pilsen, Bohemia, and studied medicine in Vienna, where he became professor of medicine in 1846. His clinical researches into cardiopulmonary disease at the Allgemeine Krankenhaus were complementary to the pathological anatomical studies carried out there by Karl von Rokitansky. Skoda had been stimulated by the pioneer work on percussin and auscultation of the Paris school of medicine, but his own researches simplified the classification of physical signs taught by the French masters, Laënnec and Piorry. Skoda's findings were collected in his magnum opus, Abhandlung über Perkussion und Auskultation (1839), in which he described his famous eponymous physical sign, "Skodaic resonance." Skoda was sceptical of the medical treatment then available and was labelled a "therapeutic nihilist." Together with Rokitansky, he helped to make Vienna, for a time, the focal point of medical teaching in Europe. He died on 13 June 1881.

Anniversaries and Special Events↗
PubMed2002

Classification of hand splinting.

The terminology in describing splint or orthosis has been reviewed but there is no one single system adopted universally. Joint efforts by doctors, therapists and orthotists had been set up to review the classification of splint. Four ways of classifying hand splints have been introduced: namely, eponym, acronym, descriptive classification system and the classification system proposed by the American Society of Hand Therapists. These systems include the use of rote memory or logical deduction in grouping of splints. This paper describes the advantages and disadvantages of each classification system. Neither one of the systems stands out to be the best. A combination of the advantages of different systems, such as precision and logical deduction, may be an option for developing a new system. Moreover, communication, documentation and other environmental factors should also be considered.

Communication↗
PubMed1985

Sleeve device functions as a Starling resistor to record sphincter pressure.

In 1976 Dent (Gastroenterology 71: 263-267) introduced a sleeve-catheter device for obtaining continuous recording of lower esophageal sphincter pressure. The infused sleeve accommodates for axial sphincter movement by providing a large surface of collapsible membrane that is capable of sensing maximal sphincter pressure at any point along the sleeve. Although sleeve performance was tested previously, the precise physical principal of its function has not been delineated. This study tests the hypothesis that the sleeve device functions as a Starling resistor. The term "Starling resistor" is an eponym that designates the physics of fluid flow through collapsible tubes. When pressure at any point along an infused collapsible conduit is greater than the intraluminal pressure at the distal end of the conduit, partial collapse occurs at some axial location along the conduit where the transmural pressure equals zero. The location of zero transmural pressure is termed the "equal pressure point" (EPP). The partial collapse at the EPP causes a local change in luminal resistance that is directly related to the magnitude of the external pressure at the EPP and accompanied by a corresponding change in the pressure upstream from the EPP. A correlate to the performance of Starling resistors is that the pressure upstream to the EPP is not affected by the downstream pressure, so long as the downstream pressure is less than the external pressure. To test our hypothesis, we evaluated sleeve performance in vitro using a two-chambered model that allowed application of static or oscillatory pressures at one or two sites along the sleeve.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed2005

[Brainstem dysgenesis: Möbius, Cogan and Pierre Robin syndromes revisited].

We propose the term brainstem dysgenesis to designate patients with congenital dysfunction of the cranial nerves and muscle tone due to prenatal lesions or anomalies of the brainstem. In some patients, the dysgenesis is genetically determined and can be isolated or form part of a more extensive polymalformation syndrome (mutations of organizing or regulatory genes). In most patients with brainstem dysgenesis, however, the disorder is caused by prenatal destructive or disruptive lesions of vascular origin. Depending on the vessels involved and the magnitude of the lesion, clinical manifestations can range from intrauterine death to mild involvement of several cranial nerves. Clinical findings in some of these patients may coincide with those described in Möbius, Pierre Robin or Cogan syndromes and, if that is the case, the eponym will indicate the location of the brainstem lesion. Clinical manifestations in most patients with brainstem dysgenesis, however, do not fit into any of the aforementioned syndromes. In these circumstances the term brainstem dysgenesis should be used followed by a detailed description of each patient's clinical findings and/or the brainstem segment presumably involved. The prognosis of patients with brainstem dysgenesis due to prenatal destructive lesions depends on the magnitude of the vascular territory involved and, in most cases, is better than the initial clinical manifestations would indicate.

Brain Stem↗
PubMed2000

MR venography in children with complex craniosynostosis.

PURPOSE: Chronic venous hypertension due to jugular foramen stenosis has been proposed as an etiology for the hydrocephalus and tonsillar herniation seen in some patients with complex craniosynostosis. We report the use of MR venography (MRV) to evaluate venous outflow obstruction in this clinical setting. MATERIALS AND METHODS: We studied 17 patients, (ages 4 months to 34 years; mean 7.3 years) with complex craniosynostosis; 8 patients with Crouzon's syndrome, 2 with Apert's, 1 with Pfeiffer's and 6 patients without an eponymous classification. MR imaging included routine imaging sequences and axial 2D TOF MRV. Patterns of venous drainage and the presence of hydrocephalus and tonsillar herniation were noted. RESULTS: Jugular vein obstruction was seen in 12/17 patients; in 5/8 patients with Crouzon's, 1/2 with Apert's, the single patient with Pfeiffer's and 5/6 patients with nonsyndromic craniosynostosis. The predominant collateral drainage was via the posterior condylar veins. Nine of 12 (75%) of the patients with abnormal MRV had hydrocephalus; 3/8 patients with Crouzon's, 1/2 patients with Apert's, and 5/6 nonsyndromic patients. Two patients had hydrocephalus with normal MRV. Ten patients had tonsillar herniation, which was associated with shunted hydrocephalus in 7/10 patients, and hydrocephalus seen prior to shunt placement in 3/9. Nine of 10 patients with tonsillar herniation had an abnormal MRV, while 1 patient had a normal MRV. Venous pressures measured in 1 patient showed an 8-mm-Hg differential across the skull base. CONCLUSIONS: The posterior condylar veins appear pivotal in maintaining venous drainage when the jugular bulbs are occluded. Although the association between venous outflow obstruction, hydrocephalus and tonsillar herniation is intriguing, evidence of venous outflow obstruction by MRV may not be indicative of significant intracranial venous hypertension.

Adolescent↗
PubMedDate not supplied

Is dementia a disease?

BACKGROUND: It is customary for many neurologists to think that dementia is a disease. This view is based on the following reasons: (1) a brain disease is the cause of cognitive impairment; (2) therefore, such cognitive impairment is substituted for the disease, becoming dementia, which is then also regarded as a mental disease. OBJECTIVE: In this brief article, I take exception to such a view, contrary to the common belief in the medical field, on the ground that senile plaques and/or neurofibrillary tangles or any other factors cause neuronal apoptosis but they do not cause dementia directly. METHODS: Literature on dementia and aphasia are critically and briefly reviewed to get the historical perspective that it is the progressive neuronal losses, losing brain functions as a result, that cause dementia; that is, brain diseases cause neuronal losses which then result in the decrease of brain functions, thereby leading to dementia. RESULTS: There is no direct cause-effect relationship between brain disease, be it caused by vascular factors or not, and dementia which is the consequence or sequela of neuronal losses. CONCLUSIONS: It is concluded that dementia is not a disease and yet it occurs not only in Parkinson's disease, Alzheimer's disease (AD), Huntington's disease and Pick's disease, but also in any other neurodegenerative disease, e.g., spinocerebellar ataxia, or vascular disease, e.g., Binswanger's disease, as part of the process of aging; in fact, AD is now regarded by some as a vascular disorder with neurodegenerative consequence, rather than a neurodegenerative disorder with vascular consequence. But vascular disorder is misleading if AD includes both neurofibrillary tangles and senile plaques; on the other hand, AD cannot be a vascular disorder if it includes only neurofibrillary tangles, as it should. Dementia, in this context, is re-defined as the differential manifestation of deteriorating brain functions over time as a part of aging due to cell deaths in the brain caused by any neurodegenerative disease. Its prominent symptoms are language disorders which must be distinguished from aphasias. It is also suggested that in fairness to Fischer, senile plaques be designated as Fischer's disease separate from neurofibrillary tangles for which AD was originally named as an eponym.

Alzheimer Disease↗
PubMed1991

Schilder's diffuse sclerosis.

The natural history and the evolution of the concept of Schilder's diffuse sclerosis have been described by Poser and van Bogaert in 1956 and there is really not much to add to their analysis. The major progress made in the clinical, genetic, enzymatic and biochemical workup of many of the conditions previously grouped under the denomination of Schilder's disease confirms its heterogeneity. We will discuss (1) the myelinoclastic disorders; (2) the leukodystrophies with disorders such as adrenoleukodystrophy and the still very ill-defined sudanophilic leukodystrophies; (3) the subacute sclerosing leukoencephalitis, better known today as the subacute sclerosing panencephalitis. In conclusion, the name of Schilder is to be remembered as are all the names of the great neurologists and neuropathologists of the past but it would be better to avoid nowadays the eponym of Schilder's disease in order to prevent confusion between different disorders.

Adolescent↗
PubMed1977

Superior orbital fissure syndrome. Review of 130 cases.

Superior orbital fissure syndrome is a symptomatologic complex, consisting of retroorbital pain, paralysis of extraocular muscles, impairment of first trigeminal branches and frequent involvement of the optic nerve. From a review of 130 published cases including two personal observations, it appears that the clinical subdivisions and the several eponymic differentiations of this syndrome are unjustified on the basis of etiologic, therapeutic and prognostic elements. Only the presence--or absence--of optic signs may allow to hold the clinically useful distinction between the often 'benign' superior orbital fissure syndrome without optic nerve involvement, and the orbital apex syndrome where orbital exploration may be recommended. This review concludes that repeated neurosurgical and neuroradiologic investigations--with the exceptions of carotid angiography and orbital phlebography--can be avoided in such cases.

Aged↗
PubMed1996

Jugular foraminal stenosis in Crouzon syndrome.

We describe a 4-year-old boy with Crouzon syndrome, with associated acanthosis nigricans and Chiari-I malformation, who developed increased intracranial pressure necessitating posterior cranial expansion. Postoperatively, an arteriovenous fistula appeared over the mastoid region. Cerebral angiography demonstrated bilateral atresia of the jugular veins and occlusion of the left sigmoid and right transverse sinuses. We propose that increased intracranial venous pressure, secondary to bilateral jugular foraminal stenosis, caused hydrocephaly and venous dilation in the scalp vasculature. The latter set the stage for a traumatic arteriovenous fistula of the scalp, probably resulting from laceration of an adjacent artery and vein. Jugular atresia is a basilar malformation common to achondroplasia and certain eponymous syndromic craniosynostoses. Our patient has a mutation in fibroblast growth factor receptor 3, a different locus in the same gene mutated in achondroplasia.

Acanthosis Nigricans↗
PubMed1989

Anatomical terminology, then and now.

Anatomical terminology, which had become chaotic by the nineteenth century, was codified in the BNA of 1895, when some 5,000 terms were carefully selected from among approximately 50,000 names. The BNA and its three major revisions (BR, INA, PNA) are here reviewed and placed in historical perspective. It is emphasized that many anatomical terms are very ancient and that the various nomenclatures are not 'new terminologies' but rather, for the most part, selections of already existing names. This can be seen clearly in the naming of the cranial nerves. Another example, the carpal and tarsal bones, is analysed in detail. Of the 8 carpal bones, for instance, the current names for 7 of them are those proposed by Henle in 1855. All the nomenclatures are, as they should be, in Latin, but it is understood that translations of many terms into other languages are necessary. Although views pro and con have been expressed, current usage favours the erect posture and the anatomical position as a basis, as well as the elimination of eponyms. In both teaching and research, the Nomina has been of great benefit in reducing drastically the number of unnecessary synonyms and in providing a coherent, internationally accepted system that is now the standard in anatomical textbooks. Hence, further use of the Nomina should be encouraged.

Anatomy↗
PubMed1994

Angiodysplastic syndrome with capillary and venous malformation associated with soft tissue hypotrophy.

Angiodysplastic syndromes include a vascular malformation which may often be associated with secondary changes such as further vascular abnormalities, soft tissue and bone hypertrophy. One of the best known is the syndrome triad originally described by Klippel and Trenaunay, which includes a unilateral capillary malformation, ectatic veins and osseous and soft tissue hypertrophy. A female patient is reported who had an extensive capillary malformation, discrete ectatic veins overlying an extensive venous malformation and soft tissue hypotrophy without bone involvement. Our patient and cases from the literature illustrate the occurrence of 'atypical', hypotrophic variants of angiodysplastic syndromes. Discrete superficial angiodysplasias may overlie deep, extensive malformations, and identification of such cases is important with regard to management and prognosis. The current classification of angiodysplasias is based on the primary vascular malformation. The use of eponyms should be avoided, because they do not contribute to the diagnosis and management of such cases.

Adolescent↗
PubMed1993

A new case of isolated trichothiodystrophy.

We describe a new case of isolated trichothiodystrophy. This entity is characterized by sparse and brittle hair, low sulfur hair content, tiger tail pattern of the hair under polarizing microscopy, clean transverse fractures through the hair shaft and absent or defective hair cuticles. To our knowledge there are only two reported cases of isolated trichothiodystrophy without associated additional ectodermal or neuroectodermal dysplasias. Polarized microscopy of cut hair showed transverse fracture points (trichoschisis) and alternating dark and bright bands. Low sulfur content of the hairs was confirmed by amino acid analysis. Many acronyms and eponyms have been created to describe sulfur-deficient brittle hair associated with neuroectodermal abnormalities, leading to confusion. We suggest to use a simple classification scheme, according to increasing severity of associated features as proposed by Van Neste.

Child, Preschool↗
PubMedDate not supplied

The common cavity congenital deformity of the inner ear. An important precursor of meningitis described in 1838.

Congenital deformity of the osseous labyrinth of the inner ear is well recognized as being associated with a fistulous communication with the intracranial subarachnoid space in some cases, leading to meningitis or a spontaneous 'stapes gusher'. Such cases have been described inappropriately as 'Mondini's dysplasia'. We cite 2 patients who both died of meningitis and had an accurate postmortem anatomical appraisal of the deformed temporal bones, one recently described by us and one by Cock more than 150 years ago. The use of eponyms is to be deprecated but should faithfully reflect the original description. The 'common cavity' lesion of the inner ear is a most important precursor of otogenic meningitis readily assessed by computed tomography and needing surgery to prevent the egress of cerebrospinal fluid or ingress of pathogens causing meningitis.

Child↗
PubMed1989

Clinical characteristics of the Munchausen syndrome. A review and 3 new case histories.

The term Munchausen syndrome was introduced by Asher in 1951 for the description of patients who tell fantastic stories and deliberately seek repeated hospitalizations at different hospitals for simulated or self-induced acute illnesses. The syndrome has been repeatedly criticized and several other eponyms have been suggested. In DSM-III-R the designation chronic factitious disorder is used synonymously with Munchausen syndrome. On the basis of 3 new case reports and a statistic processing of literature case histories, this paper suggests that when using the original criteria by Asher, the syndrome constitutes a subtype of chronic factitious disorders, specially characterized by factitious illness, peregrination, pseudologia fantastica and dramatic admission circumstances. Further, most of the criticism of the syndrome may be a result of the great variability in definitions and diagnostic criteria used by different authors. Other clinical characteristics of the patients as well as psychopathologic and psychodynamic considerations are briefly discussed.

Adult↗