The Skinner Burial of Ontario, Canada, and the Question of Paget's Disease in the Americas.

PURPOSE: To examine a possible case of Paget's disease of bone (PDB) in an Indigenous pre-contact male from Canada, individual D of the Skinner site in Ontario. METHODS: Radiographs, CT scan and histological analysis. RESULTS: The histological analysis revealed the mosaic pattern that characterizes PDB. CT scans show advanced sclerosis of the cranium and a diminished diplÓ§e with osteolytic lesions. CONCLUSIONS: The pathological features that have been identified are collectively characteristic of PDB. SIGNIFICANCE: The Skinner case advances our understanding of the global history and distribution of PDB. LIMITATIONS OF STUDY: Only two New World cases have been identified and neither has been studied in sufficient detail. SUGGESTIONS FOR FUTURE RESEARCH: The older individuals in precolonial New World skeletal series should be given CT scans, which are non-intrusive, to be followed by histological and genetic analyses when indicated.

[Visna of Egill Skallagrimsson].

Considering the changes in moral principles, human behavior and behavioral values through the ages, in Egill Skallagrimsson's Saga, Egill presents us with altered mental status. This is in terms of what at present is considered symptoms of an anti-social personality, and bipolar affective disorder. Egill Skallagrimsson is considered one of the most famous Vikings in the Icelandic Sagas. Archaeological findings mentioned in Egill's Saga indicate disfigurement of his skull, which has led many authors to suggest that Egill suffered from skeletal dysplasia. The primary assumption in the literature is that Egill Skallagrimsson was affected by Paget's disease of bone. This consideration is additionally based on the scholar's interpretation of the Saga text. The unique storytelling style in the Saga of Egill Skallagrimsson is evident; however, the question of the story's truthfulness remains open. In this article, we investigate Egill Skallagrimsson's assumed Paget's disease of bone, based on the physical and mental symptoms disclosed in the Saga of Egill Skallagrimsson. Associated with the assumption, the author's hermeneutics of Egill's Saga in the context of modern-day knowledge of Paget's disease of bone, brings forward the probability estimate to the range of permille. In Scandinavian folklore and mythology, a tale by Saxo Grammaticus of a notorious shield-maiden named Visna, reminds of Egill, as noted by Snorri Sturluson. Hence, in reference to Egill Skallagrimsson's mental status and physical appearance as listed in Egill's Saga, the authors recommend the name for his condition to be "Visna of Egill Skallagrimsson".

A microscopic evaluation of Paget's disease of bone from a Byzantine monastic crypt in Jordan.

Paget's disease of bone (PDB) is a metabolic bone disease that has been present in human populations for over 2000 years, with the earliest cases reported in Western Europe. Now present globally, PDB is one of the most common metabolic bone diseases in modern populations. This study details possible PDB of an adult male (MNR-EN Skull 3) with abnormally thickened cranial bones (17 mm). The skull was recovered from commingled skeletal remains excavated from the Robebus crypt at the Byzantine monastery of Mount Nebo, Jordan (c. late 4-7th C). Micro-CT imaging and histological sections of the bone samples revealed an abnormal pattern of bone remodeling, with atypical osteon formation, convoluted and enlarged trabeculae, and an overall pattern of highly vascularized bone. Polarized microscopy produced a mix of woven bone and lamellar bone, the mosaic pattern of atypical bone remodeling indicative of PDB. Coupled with the dense, thickened nature of the vault bones, these data suggest that the individual had PDB. To our knowledge, this represents the earliest evidence of PDB in the Middle East supported by micro-analysis, and adds to the emerging paleopathological literature involving commingled skeletal remains and the potential for identifying unique disease processes.

A case of Paget from a Northern Italy medieval necropolis.

An excavation conducted in 2002 by the Lombardy Archaeological Heritage in the St. Giulio Church (Cassano Magnago-Northern Italy) revealed an adult skeleton with an important alteration of the left femur. The femur, longer than the contralateral, exhibited an expansion of the full diaphysis; the surface was coarsened and porous. In cross section, it appeared that almost all the cortical bone had been converted into cancellous bone. Macroscopic and radiological alterations are suggestive of the monostotic form of Paget's disease, a chronic disorder, characterized by focal areas of excessive osteoclastic bone resorption followed by a secondary increase in osteoblastic bone formation. Differential diagnosis is complex for Paget's disease, requiring histological analysis, which revealed a thickened and disorganized trabecular pattern with thick cement lines. Such features, resembling to a mosaic pattern, are typical of Paget's disease.

The Paget Bicentenary: An Australian perspective.

The year 2014 marks the bicentenary of the birth of Sir James Paget (1814-1899), the celebrated English surgeon and pathologist. Although best known for his work on bone and breast disease, Paget also played an important role in the institution of Australia's first medical school. In this article, that involvement and Paget's other antipodean influences are summarised. The naming of Paget's disease of the bone is also discussed.

Enfermedad ósea de Paget: aproximación a sus orígenes históricos / Paget's disease of bone: approach to its historical origins

La enfermedad ósea de Paget es la segunda enfermedad ósea más común después de la osteoporosis. Se caracteriza por la aparición de regiones focales que presentan una remodelación ósea muy exagerada, con anormalidades en todas las fases del proceso. Este estudio tiene como objetivo investigar la hipótesis del posible origen británico de esta enfermedad estudiando su distribución geográfica mundial en esqueletos antiguos excavados en yacimientos arqueológicos. La metodología utilizada consiste en una revisión de la literatura que presenta diagnóstico de la enfermedad ósea de Paget (AU)

Paget's disease of bone is the second most common bone disease after osteoporosis. It is characterized by focal regions of highly exaggerated bone remodeling, with abnormalities in all phases of the remodeling process. This study aims to investigate the hypothesis of a possible British origin of Paget's disease of bone by studying the worldwide geographic distribution of cases identified in ancient skeletons excavated from archaeological sites. The methodology consists in reviewing cases of Paget's disease of bone described in the literature (AU)

Paget's Disease of Bone: Approach to Its Historical Origins. / Enfermedad ósea de Paget: aproximación a sus orígenes históricos.

Paget's disease of bone is the second most common bone disease after osteoporosis. It is characterized by focal regions of highly exaggerated bone remodeling, with abnormalities in all phases of the remodeling process. This study aims to investigate the hypothesis of a possible British origin of Paget's disease of bone by studying the worldwide geographic distribution of cases identified in ancient skeletons excavated from archaeological sites. The methodology consists in reviewing cases of Paget's disease of bone described in the literature.

Paget's disease of bone.

Paget's disease is an osteoclastic-mediated disorder of bone that results in abnormal bone resorption associated with inadequate remodeling that leads to mechanically weakened bone. Demonstrating variable geographic prevalence, it is becoming less frequent and age of onset is lengthening in areas of once high prevalence prior to the institution of effective medical therapies, suggesting its etiology involves both environmental as well as genetic factors. Insights into its pathophysiology are helping to clarify other inherited osteolytic disorders of bone by providing additional insights into related cellular processes.

A review of the embryological development and associated developmental abnormalities of the sternum in the light of a rare palaeopathological case of sternal clefting.

A sternal cleft or bifid sternum is a rare anterior chest wall abnormality. Although several cases have been reported in clinical literature, very little reference has been made to this anomaly in palaeopathological texts. This paper presents a case of superior sternal clefting observed in a middle-aged female with concurrent Paget's disease and congenital hyperkyphosis excavated from a 19th century Dutch psychiatric asylum cemetery in Bloemendaal, The Netherlands. The embryological development of the sternum and associated developmental abnormalities are reviewed and a differential diagnosis is performed on the suite of observed skeletal anomalies. Goltz syndrome, congenital hypothyroidism, disruption of the Hoxb-4 gene, acute excessive maternal alcohol consumption during pregnancy, Coffin-Lowry syndrome and PHACES syndrome were considered as possible causative agents, with the latter two conditions determined to be the most likely. The psychiatric asylum context, from which the individual came, supports the differential diagnosis as neurological abnormalities are common in these two syndromes. This article demonstrates that the integration of embryology, modern clinical literature and palaeopathological principles is vital in the interpretation of developmental anomalies from an archaeological context.

Egill Skallagrímsson: the first case of Van Buchem disease?

Egill Skallagrímsson, a tenth-century Viking, was a colourful warrior poet and an early anti-hero. The thickness and strength of his skull and his very ugly facial features with a prominent mandible have suggested to some authorities that Egill suffered from Paget's disease of bone. However, Paget's bone, while thickened, lacks structural integrity, infrequently involves the mandible and is prone to fractures. The more recent discoveries of sclerosing bone diseases, the elucidation of their pathophysiological abnormalities in intracellular signalling in bones and current research on the sclerostin or LRP5 genes suggest Van Buchem disease as a more probable diagnosis, although the hypothesis remains conjecture in the absence of any of his remains.

Marathon of eponyms: 16 Paget disease of bone.

The use of eponyms has long been contentious, but many remain in common use, as discussed elsewhere (Editorial: Oral Diseases. 2009: 15; 185). The use of eponyms in diseases of the head and neck is found mainly in specialties dealing with medically compromised individuals (paediatric dentistry, special care dentistry, oral and maxillofacial medicine, oral and maxillofacial pathology, oral and maxillofacial radiology and oral and maxillofacial surgery) and particularly by hospital-centred practitioners. This series has selected some of the more recognised relevant eponymous conditions and presents them alphabetically. The information is based largely on data available from MEDLINE and a number of internet websites as noted below: the authors would welcome any corrections. This document summarises data about Paget disease of bone.

Sir James Paget (1814-1899) / Sir James Paget (1814-1899)

James Paget nació en Great Yarmouth, Inglaterra, en 1814. Fue nombrado conservador del museo anatomopatológico del hospital de San Bartolomé, de Londres.Trabajador incansable, en 1874 publicó un artículo en el que relacionaba la aparición de un eccema en el pezón con el desarrollo posterior de un cáncer mamario. Fue nombrado 'Cirujano de la Corte' por la reina Victoria de Inglaterra. Murió en 1899 (AU)