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Inherited knee disorders in the Medici family

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Inherited knee disorders in the Medici family
The Knee 21 (2014) 2–5
Contents lists available at ScienceDirect
The Knee
Review
Inherited knee disorders in the Medici family
Donatella Lippi a, Marco Matucci-Cerinic b, W.R. Alburyc c, George M. Weisz d,e,⁎
a
Department of Experimental and Clinical Medicine, University of Florence, Italy
Department of Rheumatology, Denothe Centre, University of Florence, Italy
c
School of Humanities, University of New England, Armidale, Australia
d
School of Humanities, University of New South Wales, Sydney, Australia
e
School of Humanities, University of New England, Armidale, Australia
b
a r t i c l e
i n f o
Article history:
Received 6 August 2012
Received in revised form 26 April 2013
Accepted 3 June 2013
Keywords:
Joints
Medici
Paleopathology
Skin
Spine
a b s t r a c t
Reconstructing a medical condition which was existent centuries ago is limited by the lack of contemporaneous
evidence-based descriptions in the accounts given by physicians and other observers. Despite these limitations
modern paleopathological evidence, supplemented by techniques of historical investigation, have led to the conclusion that males in the Medici family typically suffered from a complex clinical entity with a triple pathology of
stenotic spinal ankylosis, recurrent peripheral joint disease and erythematous skin disease; the Medici Syndrome.
Examination of the knee joint is illustrative of recurrent joint disease both in the primary and secondary lines of
the family. Pictorial and sculptural representations, if used cautiously, can assist in this retrospective process. The
six cases presented here illustrate the involvement of the knee joint where the joint destruction ultimately led to
an ankylosis.
© 2013 Published by Elsevier B.V.
Contents
1.
2.
Introduction . . . . . . . . . . . . . . . . .
Illustrative cases . . . . . . . . . . . . . . .
2.1.
Cosimo the Elder (il vecchio), 1389–1464 .
2.2.
Piero di Cosimo (il gottoso), 1416–1469 .
2.3.
The primogenito Medici dukes . . . . . .
2.4.
Carlo de' Medici, 1596–1666. . . . . . .
3.
Conclusion . . . . . . . . . . . . . . . . . .
References. . . . . . . . . . . . . . . . . . . . .
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1. Introduction
The Medici family ruled Florence for most of the period from the
1430s to the 1730s. Renowned as political figures and patrons of the
arts, and often afflicted with poor health, the Medici rulers and their
close relatives have attracted the attention of both historians and biomedical researchers [1–9].
There were two branches of the Medici family, descended from the
two sons of Giovanni di Bicci (1360–1429); the primogenito line originating with Cosimo the Elder (1389–1464) and the secondogenito line
originating with his younger brother, Lorenzo di Giovanni (1395–
⁎ Corresponding author at: P.O. Box 543, Vaucluse, 2030, Sydney, Australia.
E-mail address: [email protected] (G.M. Weisz).
0044-8486/$ – see front matter © 2013 Published by Elsevier B.V.
http://dx.doi.org/10.1016/j.knee.2013.11.006
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2
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3
3
5
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5
1440). Table 1 shows the genealogical relationship of the politically significant male members of the two branches of the family.
The Medici family's numerous medical conditions were inadequately characterized prior to the mid-twentieth century. It was for this reason that in 1947, after the Second World War, a group of
anthropologists decided to conduct a scientific study of the remains of
Medici family members who were buried within the San Lorenzo
church complex, in the center of Florence. This research excluded the
two Medici popes, Leo X and Clement VII, who were buried elsewhere.
For protection during the war, the Medici tombs in San Lorenzo and
the decorative marble sculptures by Michelangelo had been removed.
Prior to their replacement after the war, an anthropological team led
by Gaetano Pieraccini (the Mayor of Florence and President of the
Opera Medicea Laurenziana) and Giuseppe Genna inspected the remains in their tombs [2–4].
D. Lippi et al. / The Knee 21 (2014) 2–5
3
progressing into chronic stages, and possibly contributed to a decline in
longevity of those affected. As we have shown elsewhere, males of the
primary lineage experienced a sharp decline in longevity over six generations; from the time of Giovanni di Bicci (age 69 at death) and Cosimo
the Elder (age 75 at death) to that of Ippolito (age 24 at death) [13].
The traditional diagnosis of gout in the Medici family, which long
persisted as the explanation both of morbidity and of mortality, has
been excluded. The joint entity is an acute, inflammatory peripheral arthropathy, characterized by painful and immobilizing swelling in the
legs and hands. This phase alternates with a chronic one, with periods
of remission permitting normal physical activities. Eventually, a mutilating deformity of large and small joints tends to develop, rendering
the sufferer immobile and bed ridden.
2. Illustrative cases
2.1. Cosimo the Elder (il vecchio), 1389–1464
Cosimo the Elder was in his early 30s when a joint affliction, called
by his contemporaries “gout,” started to affect his hands, neck, knees
and ankles. The exhumed skeleton was observed macroscopically and
an exostosis was found above the femoral condyle at the insertion of
the adductors, with ossification of the interosseous membrane, both
conditions affecting the function of his knees [4] (Fig. 1).
2.2. Piero di Cosimo (il gottoso), 1416–1469
Fig. 1. Cosimo the elder: ossification of tendon insertion on femoral condyle.
More recently the Progetto Medici has been exhuming members of
the family and subjecting them to more modern investigations [7,8].
The accumulated paleopathological and historical evidence has led
to a reasonable conclusion that males in the Medici family typically suffered from a complex clinical entity with a triple pathology of stenotic
spinal ankylosis, recurrent peripheral joint disease and erythematous
skin disease. These components could co-exist as independent entities,
but in view of their combined presence in several generations of the
same family, they have been grouped together as a syndrome [10–12].
The three entities of the Medici syndrome were intensely symptomatic,
Piero di Cosimo suffered from a pathological condition of the joints
for almost his entire adult life, leading to eventual immobilization. The
contemporary medical description of “gout” was later interpreted as
“poliartrite anchilosante” [4]. Histological investigation documented
“l'ossificazione vera e propia di alcuni ligamentosi,” producing an ankylosis of the knees, ankles and hands. His suffering was perhaps the most
severe and prolonged of all the members of the primogenito line. X-rays
of his skeleton (Fig. 2) show that “i conchili femorali sono saldati con la
tibia” (the condyles are fused with the tibia) in a flexion contracture of
the knee, with no subluxation but fusion of almost all joints. Also found
was the ossification of the interossei-tibio-fibular-membrane [2,3].
2.3. The primogenito Medici dukes
Radiographs of the exhumed skeletons found tendinous calcification
in Giuliano, Duke of Nemours (1479–1516), Lorenzo, Duke of Urbino
(1492–1519), and Alessandro, Duke of Florence (1510/12?–1537).
This condition, no doubt, would have interfered with the mobility of
the knee joint. These findings appear to be parafemoral ossifications in
the skeletons of Giuliano, Lorenzo and Alessandro (Fig. 3), although
Fig. 2. Piero il gottoso: photo of skeleton.
4
D. Lippi et al. / The Knee 21 (2014) 2–5
Fig. 3. Femora of the primogenito Medici dukes: a. Giuliano, Duke of Nemours; b. Lorenzo, Duke of Urbino; c. Alessandro, Duke of Florence (Genna Archives, D. Lippi, Custodian).
Fig. 4. Michelangelo, sculptures of a. Giuliano, Duke Nemours; b. Lorenzo, Duke of Urbino. Capella Medici, Firenze.
Reproduced with permission of Soprintendenza Speciale al Polo Museale Fiorentino.
D. Lippi et al. / The Knee 21 (2014) 2–5
5
Fig. 5. Carlo di Ferd-9inando I, Cardinal, X-ray of fused knee joint.
the possibility that these dark parafemoral bands may be photographic
artifacts cannot be excluded.
Two of the primogenito Medici dukes, Giuliano and Lorenzo, are
depicted in the sculptural masterpieces of Michelangelo in the Capella
Medici of the San Lorenzo basilica in Florence (Fig. 4). The sculptor
worked posthumously (1526–34), both dukes having died about a decade earlier, and in all probability he had never met either of them
when they were alive. Nevertheless, one finds abnormal shapes in the
knees of these statues, indicating an unidentified pathology. Michelangelo had a detailed knowledge of normal anatomy and consummate
skill as a sculptor, so he would not have produced these abnormalities
by accident. Even if the model who posed for these statues had suffered
from deformity of the knees, Michelangelo could easily have corrected
this in his statues, showing normal joints instead. So we conjecture
that the knee problems of the two deceased Medici dukes were sufficiently well-known that Michelangelo intentionally included some abnormality in the knees of his statures.
2.4. Carlo de' Medici, 1596–1666
Carlo de' Medici, son of Grand Duke Ferdinando I, was a sickly child,
developing slowly. But he became an intellectual, dedicated to the Curia
as a Cardinal. He suffered from a number of skeletal deformities
throughout his life but stoically continued his activities, as far as possible, even while immobilized with ankylosed knees. The X-rays show fusion of all compartments of the knee joint in flexion of almost 90°
(Fig. 5) [12,14].
3. Conclusion
Although privileged by their wealth and political power, many
members of the Medici family experienced illnesses which led to permanent deformity and immobility. They appear to have suffered an
inherited disorder called the Medici Syndrome which caused an ankylotic spinal stenosis, an erythematous skin disorder and recurrent peripheral joint disease. Originally this was said to be gout. But these six
cases presented here illustrate the involvement of the knee joint
where the joint destruction ultimately led to an ankylosis.
References
[1] Pieraccini G. La Stirpe de' Medici di Cafaggiolo: Saggio di ricerce sulla trasmissione
ereditaria dei caratteri biologici. , 3. Firenze: Nardini Editore; 1986. p. 1924–5.
[2] Genna G. Richerche antropologiche sulla famiglia dei Medici. Atti dell'Accademia
Nazionalle dei Lincei 1948;4(Serie VIII):589–93.
[3] Genna G. Lorenzo il Magnifico e il fratello Giuliano dal punto di vista antropologico.
Atti dell'Accademia Nazionale dei Lincei 1953;15(Serie VIII):469–73.
[4] Costa A, Weber G. Le alterazione morbose del sistema scheletico in Cosimo dei
Medici il Vecchio, in Piero il Gottoso, in Lorenzo il Magnifico, in Guiliano Duca di
Nemours. Archivio de Vecchi Anat Patol 1955;23:1–69.
[5] Hale JR. Florence and the Medici. London: Thames and Hudson; 1977.
[6] Micheletti E. The Medici of Florence. Florence: Becocci editore; 1999.
[7] Lippi D, Fornaciari G, Gensini GF. Evidence based history of medicine. Proceedings of
the 40th International Congress of the History of Medicine. Budapest: International
Society of the, History of Medicine; 2006. p. 633–6.
[8] Lippi D. Illacrimate sepolture: Curiosità e ricerca scientific nella storia delle
riesumazioni dei Medici. Firenze: Firenze University Press; 2006.
[9] Ferri M, Lippi D. I medici: la dinastia dei misteri. Firenze: Giunti Editore; 2007.
[10] Weisz GM, Matucci-Cerinic M, Albury WR, Lippi D. The Medici Syndrome: a medicohistorical puzzle. Intern J Rheumatic Dis 2010;13:125–31.
[11] Weisz GM, Matucci-Cerinic M, Lippi D, Albury WR. The ossification diathesis in the
Medici family: DISH and other features. Rheumatol Int 2011;31:1649–52.
[12] Weisz GM, Matucci-Cerinic M, Albury WR, Lippi D. The illnesses of Carlo
di Ferdinando i de' Medici: A second opinion. Clin & Exper Rheumatol
2012;30:6–11.
[13] Lippi D, Matucci-Cerinic M, Albury WR, Weisz GM. Longevity and causes of death of
adult males in the Medici di Bicci family. J Family Hist 2009;34:243–50.
[14] Albury WR, Matucci-Cerinic M, Weisz GM. Carlo di Ferdinando i (1596–1666)—un
caso di estrema longevità nonostante la salute cagionevole, trans. In: Wieczorek A,
Rosendahl G, Mannheim Lippi D, editors. Alterauge A and Dörr F. I medici: Uomini,
potere e passione. Schnell & Steiner; 2013. p. 364–71.
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