[Pierre and François Mauriac, a privileged friendship between brothers]. / Pierre et François Mauriac, une amitié fraternelle privilégiée.

Pierre and François have been the key figures of the Mauriac five children. Pierre introduced his hospital service in Bordeaux to bio-clinic and was a well-known professor before he became the dean of the Faculty from 1936 to 1945. As hero during the Great War he remained loyal to the "victor of the battle of Verdun" while his brother Frangois became a fervent 'Gaullist', "each of them climbing the same mountain along another side" according to François Mauriac in 1966. Pierre was a writer too and a medical adviser for the novelist.

[Montesquieu visually impaired, then blind (January 1689- February 1755)]. / Montesquieu malvoyant, puis aveugle.

The correspondence of Montesquieu published by the Oxford Foundation informs about visual disorders of the founder of the socio-political science. The examination of his bust's face done by J.B. Lemoyne reveals a divergent squint of the left eye; the one with which he fold that he only could see big objects. This amblyopia was a premature and prolonged embarrassment. During the last ten years of his life, from 1748, date of publication of the Esprit des lois up to his death in 1755 he was blind because of the cataract of the other eye. He has not able to bust in surgery, while the French surgeon Jacques Daviel already proceeded to the extraction of the lens as we do it nowadays.

[Saint Anthony's Fire or gangrenous ergotism and its medieval iconography]. / Le feu Saint-Antoine ou ergotisme gangreneux et son iconographie médiévale.

The frequent epidemics of ergotism were called Holy Fire or st-Antony's Fire in the Middle Ages, because of the burning sensations resulting in gangrene of limbs. It was caused by eating rye bread contaminated with the fungus Claviceps purpurea. The hospitable Order of st-Antony was founded near Vienne in France with 300 establishements in Europe until 1777. In coptic and byzantine art st-Antony is the father of the monks, whereas in Occident he is the the master of fire, thaumaturgic, resulting a very important iconography in statuary and painworks in all regions, especially in Lorraine, the catholic and tridentin Lotharingia and in Corsica thanks to the franciscan pastoral. Woodcuts show not only the temptations of st-Antony, with strange and diabolic scenes, patients with gangrenous limbs. Germanic woodcuts of the 15th century show patients with different stages of ergotism and hands and feet like ex-voto. Triptycs of H. Bosch and M. Gunewald are witnesses of the frequency and seriousness of this disease still at the beginning of the 16th century.

Art and the history of medicine. The dwarf pointed by Mantegna of Montua and the Morgante of Florence.

This short article concerns two images which relate art and the history of medicine. The first is a fresco by Andrea Mantegna, from the period 1465-1474, when he was at the Gonzaga court of Ludovic III. This painting includes a female dwarf. Modern ideas on dwarfism are briefly discussed and another dwarf, Morgante, from the court of the Medicis in Florence, is described and illustrated.

[Genetic obesity in childhood]. / Les obésités génétiques en pédiatrie.

Polygenic obesity is increasingly frequent in childhood. Rare monogenic, non syndromic forms are of special physiopathological interest. They are due to recessive or dominant leptinlreceptor deficits, pro-opiomelanocortin deficit, or 4-melanocortin deficit. Genetic obesity syndromes include the Bardet-Biedl, Cohen, Albright, Alstrom, MOMA and Prader- Willi syndromes. The latter, first described in 1956, is the most frequent (1/10 000). It is associated with severe early-onset obesity, morbid hunger, and ante and postnatal hypotonia. It is due to microdeletion of paternal chromosome 15 (15qll-ql3) or to uniparental maternal disomy. This is the föirst human example of genomic imprinting and monoallelic gene expression. Early diagnosis is essential for effective therapeutic management.

[Antony's fire (gangrenous ergotism) and medieval iconography]. / Le feu Saint-Antoine ou ergotisme gangreneux et son iconographie médiévale.

Ergotism was known as Holy Fire or St Antony's Fire in the Middle Ages, because of the burning sensations and limb gangrene it entailed. It was a frequent disorder, caused by eating rye flour contaminated by the fungus Claviceps purpurea. The Hospitable Order of St Antony was founded near Vienne in France, and counted 400 establishments in Europe by 1777. Ergotism is the subject of an abundant iconography, including statues and paintings. Woodcuts show the temptations of St Antony, with strange and diabolic scenes, and individuals with gangrenous limbs. Germanic woodcuts of the XVth century show various stages of ergotism and hands and feet. The tryptics of Bosch and Grunewald bear witness to the frequency and gravity of this disorder, at the beginning of the XVIth century.

[Neurosciences in Bordeaux]. / Chronique historique Les Neurosciences à Bordeaux.

The Bordeaux Neuroscience Institute brings together all the disciplines that constitute the clinical and experimental neurosciences. Outside of the Paris region, the Institute represents the largest community of researchers working on the nervous system. The aim of this brief historical piece is to describe how neuroscientists in Bordeaux are the heirs to a long neuropsychiatric tradition established by pioneers of national and international renown. This tradition has been maintained, without interruption, through many generations. The careers and scientific work of these great neurologists and psychiatrists are briefly evoked, and particularly those of A. Pitres, E. Régis and E. Azam in the 19th century; and, in the 20th century, J. Abadie, H. Verger and R. Cruchet. The determining influence of P Delmas-Marsalet (1898-1977), Professor of Neuropsychiatry, on the development of modern neurosciences in Bordeaux is recalled through his work, his teachings, and his numerous students.

[Montesquieu, sciences and medicine in Europe]. / Montesquieu, les sciences et la médecine en Europe.

Montesquieu is the first one of his century to be fascinated by sciences, chiefly by sciences of life. He created a prize of plysics and anatomy, used the microscope and even made experiments. Despite his amblyopia and his cataract which made him blind, he was in contact with the intellectual and scientific elite of his time. He has been an innovator through his interest about public health and professional diseases.

[Autographs of physicians and famous scholars]. / Autographes de médecins et savants célèbres.

The Archives of the Society of Medicine of Bordeaux include the period of two centuries from 1796 to 1995 and many autographs and letters of medical doctors and famous scholars can be found in them such as Claude Bernard, Laennec or Pasteur. All these autographs or letters are interesting as they bear witness to the action of them who made the real scientific medicine.

X-linked dominant chondrodysplasia with platyspondyly, distinctive brachydactyly, hydrocephaly, and microphthalmia.

We describe a family with an X-linked dominant chondrodysplasia. Four males and six females were affected through four generations. Identification of skeletal abnormalities and hydrocephaly during the pregnancy of three male fetuses led to termination of the pregnancies. A fourth affected male died at 6 days of life. The four patients had chondrodysplasia, hydrocephaly, and facial features with microphthalmia. Radiographs showed severe platyspondyly and various bone abnormalities including a distinctive metaphyseal cupping of the metacarpals, metatarsals, and phalanges. The affected females were less affected and showed small stature, sometimes associated with body asymmetry and mild mental retardation. This condition appears to be a previously unrecognized X-linked dominant chondrodysplasia.

[Turner syndrome and mosaicism]. / Syndrome de Turner et mosaïcisme.

Turner's syndrome is a gonadosomatic dysgenesis of female phenotype due to a more or less complete monosomy of one of the X chromosomes leading to a haploinsufficiency of the development genes situated at the level of the pseudoautosomal region of the gonosomes. Further experience of the karyotype showed a preponderance of mosaics and considerable variability of Turner's phenotype in proportion to the number of 45, X cells. The rare cases of monozygotism discordant with variable tissular distribution mosaics show that the phenotypic expression is a genic dosage effect. In patients with TS it would thus be of interest to study a second tissue such skin fibroblasts when a discordance is observed between the phenotype and the karyotype.