Art and Dystonia Pedro J. Garcia- Ruiz, Jaroslaw Slawek, Emilia J. Sitek, Juan Carlos Martinez Castrillo PII: DOI: Reference:
S0022-510X(15)00390-1 doi: 10.1016/j.jns.2015.06.049 JNS 13878
To appear in:
Journal of the Neurological Sciences
Received date: Revised date: Accepted date:
7 April 2015 17 June 2015 23 June 2015
Please cite this article as: Pedro J. Garcia- Ruiz, Jaroslaw Slawek, Emilia J. Sitek, Juan Carlos Martinez Castrillo, Art and Dystonia, Journal of the Neurological Sciences (2015), doi: 10.1016/j.jns.2015.06.049
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT ART AND DYSTONIA. Pedro J Garcia- Ruiz MD PhD(1) *
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Jaroslaw Slawek MD PhD (2) *
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Juan Carlos Martinez Castrillo MD PhD (3) *
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Emilia J. Sitek MA, PhD (2)
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1. Movement Disorder Unit. Department of Neurology. Fundacion Jimenez Diaz. Madrid. Spain
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2. Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Gdansk, Poland and Department of Neurology, St. Adalbert Hospital, Copernicus PL Sp. z o.o, Gdansk, Poland
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Cajal. Madrid. Spain
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3. Movement Disorder Unit. Department of Neurology. IRYCIS. Hospital Ramon y
*These authors equally contributed to this work
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Address of corresponding author: Pedro J Garcia-Ruiz
Department of Neurology Fundacion Jimenez Diaz Avda Reyes Católicos 2 Madrid 28040 Email: [email protected] Telephone: 3491 5504882
Key words: Dystonia; Art; History; Movement Disorders; Medieval Art; Brancusi; Modigliani; Wyspianski
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ACCEPTED MANUSCRIPT ABSTRACT Dystonia has a recent history in medicine. Focal dystonia was described in the 19th century by classic authors including Gowers, whilst generalized dystonia was described at the turn of the
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century. However, it is possible to find precise descriptions of dystonia in art, centuries
IP
before the medical definition. We have reviewed several pieces of art (sculpture, painting and
SC R
literature) across the history that might represent descriptions of dystonia, from ancient period to nowadays.
In classic times, the first reference to abnormal postures can be tracked back to the new
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Empire of Egypt (equinus foot), not to mention some recently described examples of dystonia from the Moche sculptures in Peru or Veracruz culture from Mexico. In Middle Ages it is possible to find many examples of sculptures in European cathedrals representing peasants
MA
with dramatic, presumably dystonic postures that coexist with amputation of limbs. This unique combination of dystonia and limb amputation probably represents ergotism. The
D
painters Brueghel, Ribera and Velazquez also represented figures with postures likely to be dystonic. Literature is also a source of precise pre-neurological
descriptions, especially
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during the 19th century. In David Copperfield, Dickens depicts characters with generalized dystonia (Uriah Heep), cervical dystonia (Mr Sharp) and spasmodic dysphonia (Mr Creakle).
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Finally, even in modern Art (19th and 20th centuries), there are dramatic descriptions of abnormal postures that are likely to be dystonic, such as painful cervical dystonia (Brancusi), cervical dystonia with sensory trick (Modigliani) and upper limb dystonia (Wyspianski).
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However some postures presented in works of art may simply be a form of artistic expression and only bear unintentional resemblance to the dystonic postures. Art may be a source of neurological information, and that includes primary and secondary dystonia
2
SC R
IP
T
ACCEPTED MANUSCRIPT
Dystonia has a relatively short history in medicine. Focal dystonia as a neurological syndrome was described in 19th century by several authors including Gowers [1] and the American
NU
Neurologist Horatio Wood [2] whilst generalized dystonia was reported at the turn of the 19th and 20th centuries by Luis Barraquer Roviralta in 1897, Marcus Walter Schwalbe in 1907; Hermann Oppenheim and Georg Theodor Ziehen in 1911; and in the same year the Jewish-
MA
Polish neurologists Edward Flatau and Wladyslaw Sterling described the condition [3-5]. However, it is possible to find precise descriptions of dystonia in art, even centuries before the
D
first medical descriptions.
The paintings or sculptures that represent people affected by various conditions usually lack
TE
the documentation of the disorder, so dystonia as the cause of unusual postures or gestures may be only hypothesized. Finally, some postures presented in works of art may simply be a
CE P
form of artistic expression and only bear unintentional resemblance to the dystonic postures. This paper presents selected pieces of art and literature dating from antiquity to modern times,
AC
that are likely to contain the representations of dystonic postures. Rather than a general review this is a personal selection of those pieces that impressed us most
ANCIENT AND CLASSICAL PERIODS The first description of an abnormal posture can be found in Egyptian art (New Empire, Fig. 1). The affected individual had a clearly equinus foot which was attributed to polio [6] (there are no descriptions of polio before the 18th century). An alternative hypothesis may be an equinus gait secondary to cerebral palsy. There are several sources commenting on historical figures affected by neurological diseases, among whom Alexander the Great is mentioned. It was suggested that Alexander the Great suffered from cervical dystonia [7]. His abnormal neck posture was described by Plutarch, who noted that Lyssipus, the emperor´s personal sculptor, modelled the first statue of
3
ACCEPTED MANUSCRIPT Alexander which represented him looking up with his face turned toward the heavens (Fig. 2). Archelasus, among others, also confirmed Alexander’s abnormal posture. In our opinion, the diagnosis of spasmodic torticollis is questionable as there are various statues representing
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Alexander and not all of them exhibit clear cervical deviation.
MIDDLE AGES
Cathedrals all over Europe represent the quintessence of medieval art. Some of the figures
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from the tomb of San Pedro de Osma in El Burgo de Osma cathedral (Soria, Spain) show clear cervical dystonic postures and some have other dystonic features or residual limbs (Figs.
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3-4).
We suggest that the statues with dystonic features plus residual limbs might represent patients
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with ergotism, or St Anthony fire [8], which was mainly characterized by peripheral vascular
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disease and gangrene resulting in limb loss [9,10]. In addition, ergotism (convulsive ergotism in particular) was also characterized by severe muscular spasms and abnormal postures [10] .
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Ergotism was due to long-term poisoning by Claviceps purpurea fungus, formed on cereals, and widespread all over Europe during XII-XVI centuries (8,9). Ergotism was graphically described in paintings by Mathias Grunewald, in some of his paintings severe dystonia can be recognized [11]. However, some authors suggest that ergotism was in fact a serotonin
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syndrome [10,12]. If this was the case, movement disorders in patients with ergotism can be easily explained, since dystonia, myoclonus and other movement disorders have been associated with serotonin syndrome [10,12].
NEW WORLD: ANCIENT PERIOD Moche artists (from pre-Columbian culture developed in Northern Peru, 100-700 AD) had exceptionally sophisticated ceramic techniques, which they often used to represent their people, particularly individuals affected by diseases or with physical deformities, as they believed that the handicapped were touched by the gods. The potter presented here (Fig. 5) represents a man with his eyes closed and facial grimacing probably in an attempt to open his eyes. The lips are horizontally tensed and the nasolabial folds are pronounced. In our opinion,
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ACCEPTED MANUSCRIPT this sculpture may be an artistic representation of blepharospasm with oromandibular dystonia [13]. A very similar representation of cranial dystonia, resembling the classical Meige's syndrome
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is shown at Fig. 6. The sculpture (Xipe-Totec Head, 300-900 AD) shows the closed eyes and
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lower face dystonia (jaw-opening), but also tongue dystonia (protrusion). This sculpture made
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of terracotta is a part of Veracruz Culture heritage from Mexico.
Sensory tricks (fr. gestes antagonistes) are characteristic features of dystonia. Figs. 7 and 8 represent examples resembling such movements. The first one was made probably somewhere
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in Tokyo graveyard and the second one represents art of Melanesia, Papua, New Guinea
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MODERN HISTORY
The great Flemish Renaissance painter Pieter Brueghel the Elder had a fascination with
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human physiognomy, both normal and pathologic. Historians of art contend that Fig. 9 depicts
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a huge yawn and have titled this work “Yawning Man” or “De Gaper”. However, Marsden credited R.E. Kelly for noting the similarity of this painting to the syndrome of
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blepharospasm-oromandibular dystonia and suggested the eponym "Brueghel's syndrome" [14]. Marsden claimed that Brueghel "clearly recognized the syndrome", but of course, it may be an overinterpretation of this undated, and untitled painting. ¨El patizambo¨ or ¨The clubfooted boy” (Jose de Rivera, 1642, Fig 10) is a representation of a
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beggar with a right hemidystonia. Velazquez painted in “The boy from Vallecas”, (1643-45, Fig. 11) depicts a boy with some degree of mental retardation and mixed cervical dystonia with a right torticollis and retrocollis.
CONTEMPORARY HISTORY The turn of the 19th and 20th centuries was the time of great transformation in art from classical figurative or descriptive forms to very modern, expressive and abstractive forms. It is possible to find descriptions of dystonia in contemporary art; this is the case of “Suffering” from Constantin Brancusi (1876-1952) (Figs. 12 and 13), which represents a boy with a very notorious painful cervical deviation. Brancusi is believed to have observed and represented a real patient with cervical dystonia [15]
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ACCEPTED MANUSCRIPT Modigliani was contemporary of Brancusi. He is known for portraits and nudes in a modern style characterized by elongated faces and figures. Some of his paintings suggest cervical dystonic features including “sensory tricks” (Fig.14). However, it is doubtful that all these
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paintings actually represented real people with dystonia.
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Stanislaw Wyspiaski, born in Cracovia (1869-1907) was also an interesting author at the turn
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of the 19th and 20th centuries. This artist practised a variety of arts including poetry, theatre and painting. He worked fast and with extraordinary intensity, his highly diverse output produced in a mere dozen years. We show one of his most realistic and beautiful artworks
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(The girl with red hat, Fig. 15), a young lady with upper limb dystonia, possibly related to cerebral palsy.
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LITERATURE AND DYSTONIA. CHARLES DICKENS. Many writers described neurological conditions across the centuries. Charles Dickens (1812-
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1870) had a special interest in individuals displaying neurological symptoms, and his novels are a source of precise neurological descriptions. Brain already quoted some Dickensian
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neurological diagnosis [16]. Besides Pickwickian syndrome, many other neurological
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descriptions can be found in Dickens´ novels [16-19] including David Copperfield [19]: 1. Generalized dystonia: Uriah Heep. This character, one of the villains of the novel is described as a very tall, strange man with Marfan-like features: "Uriah, with his long knees
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drawn up under his coffee-cup¨. Uriah Heep displayed clear dyskinesias, most probably generalized dystonia:
"He writhed himself quite off his stool in the excitement of his
feeling...” " He had a snaky undulation pervading his frame from his chin to his boots...", "...Uriah exclaimed, with a sickly, angular contortion of himself...”,"... I am sure, said Uriah, writhing himself into the silence like a Conger eel¨ 2. Spasmodic dysphonia:
Mr Creakle.
¨ The circumstances about Mr Creakle which
impressed me most was, that he had no voice, but spoke in a whisper. The exertion this cost him, or the consciousness of talking in that feeble way, made his angry face so much angry, and his thick veins so much thicker when he spoke¨ As Mr Creakle struggled to make his voice audible, he had no voice but a whisper, spasmodic dysphonia (abductor type) could be a plausible explanation of this presentation. 3. Mr Sharp: Cervical dystonia. ¨Mr Sharp was a limp, delicate-looking gentleman, with good deal of nose, and a way of carrying his head on one side, as if it were a little too heavy for
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ACCEPTED MANUSCRIPT him¨. In this paragraph, Dickens described a man with neck rotation, which may be due to
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cervical dystonia.
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SUMMARY
In summary, we have reviewed a personal selection of art (sculpture, painting and literature) that may represent real patients with dystonia or dystonic movements. Our descriptions add to the growing body of reports on the pieces of art representing movement disorders [20,21].
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Artists from ancient times throughout the centuries and up to now, from all over the world (e.g. from Europa, Asia and the New World) frequently described or depicted neurological
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diseases decades or centuries before the official reports by neurologists. Thus, history of neurology may be enriched by the history of art.
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Finally, we should like to include two recent papers related to art and dystonia: Bono et al
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recently called attention to Giovanni Agostino da Lodi (Italian renaissance painter of the Leonardeschi) who made a clear depiction of cranial-cervical dystonia [22]. Gondim et al
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reviewed neuropsychiatric phenomena described in Cantigas de Santa Maria; a collection of 427 canticles composed between 1252 and 1284 at the Court of King Alfonso X the Wise of Spain (1221-1284). In these cantigas the authors found several descriptions of dystonic
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movements [23].
Author roles:
Pedro J Garcia Ruiz: Conception and design, acquisition, analysis and interpretation of data, drafting the submitted material, administrative support Jaroslaw Slavek: Conception and design, acquisition, analysis and interpretation of data drafting part of the submitted material, critical revision and supervision Juan Carlos Martinez Castrillo: acquisition, analysis and interpretation of data; critical revision and supervision Emilia J. Sitek: acquisition of data, analysis and interpretation of data; critical revision and editorial support
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ACCEPTED MANUSCRIPT
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No conflict of interests reported
REFERENCES
Philadelphia: P Blakiston, 1888:1357
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1. Gowers W R. In: Gowers WR ed. A Manual of diseases of the nervous system.
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2. Wood HC. Nervous diseases and their diagnosis: a treatise upon the phenomena produced by diseases of the nervous system. Philadelphia; JB Lippincott,: 1887: 1937
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3. Oppenheim H. Dysbasia lordotica progressiva, Dystonia musculurum deformans.
Flatau,
W.
Sterling:
Progressiver
Torsionspasms
bei
Kindern.
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4. E.
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Neurologie Centralblatt 1911; 30: 1090-1107
Zeitschrift für die Gesamte Neurologie und Psychiatrie, Berlin, 1911, 7: 586-612.
5. Pearce JMS. Lluis Barraquer i Roviralta and the origins of torsión dystonia. J Neurol
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Neurosurg Psychiatry 2004;75:582
6. Levin S. Crippling disorders in the Bible. S Afr Med J. 1957 ;31:16-7 7. Lascaratos J, Damanakis A. Ocular torticollis: A new explanation for the abnormal head posture of Alexander the Great. Lancet 1996;347:521-522.
8. García Ruíz P, Ruíz Ezquerro JJ, García Torres A, Fanjul S. Ancient descriptions of movement disorders: Cathedral el Burgo de Osma (Soria, Spain). J Neurol 2006; 253:731-734.
9. Ayarragaray JE. Ergotism: a change of perspective. Ann Vasc Surg. 2014;28:265-8 10. Eadie MJ. Convulsive ergotism: epidemics of the serotonin syndrome? Lancet Neurol 2003 2:429-434
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ACCEPTED MANUSCRIPT 11. Garcia de Yebenes J, Garcia de Yebenes P. Dystonia in the painting of Matias Grunewald. Epidemic ergotism in the late middle ages. Arch Neurobiol 1991; 54:3740.
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12. Bodner RA, Lynch T, Lewis L, Khan D. Serotonin syndrome. Neurology
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1995;45:219-223
syndrome. Mov Disord. 2010;25:405-6
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13. Martinez-Castrillo JC, Mariscal A, Garcia-Ruiz P. Ancient representation of Meige
14. Marsden CD. Blepharospasm-oromandibular dystonia syndrome (Brueghel's
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syndrome). A variant of adult-onset torsion dystonia? J Neurol Neurosurg Psychiatry 1976;39:1204-9.
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15. Garcia Ruiz PJ, Martinez Castrillo JC. Cervical dystonia and Constantin Brancusi. Mov Disord. 2009; 24:1849-50
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16. Brain R. Dickensian diagnoses. Br Med J 1955;ii:1553-6.
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1991;54: 184-7
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17. Cosnett J E. Dickens, dystonia and dyskinesia. J of Neurol Neurosurg Psychiatry
18. Cosnett JE. Charles Dickens: Observer of sleep and its disorders. Sleep 1992;15:264267
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19. Garcia Ruiz P J, Gulliksen L. Movimientos anormales en David Copperfield. Neurología 1999; 14: 359-360
20. Smith PEM. Neurology in the National Gallery. J R Soc Med 1999; 92:649-52. 21. Clifford Rose F. The Neurology of Art: An overview. In: Clifford Rose F. ed. Neurobiology of the arts. London: Imperial College Press, 43-76 2004
22. Bono F, Morelli M, Quattrone A. Facial dystonia as depicted in art in the time of Leonardo da Vinci. Lancet Neurol. 2015;14:351
23. Gondim Fde A, Griesbach SH, Thomas FP. Neuropsychiatric phenomena in the medieval text Cantigas de Santa Maria. Neurology. 2015;84: 1991-6
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FIGURES
Figure 1. Funeral stele of the priest Ruma (XIX dynasty). Around 1500 BC, Carlsberg Museum of Copenhagen
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Figure 2. Head of Alexander the Great, Lyssipus. Istambul Archeological Museum Figure 3. Peasant with head tilt to the right and painful expression (center), note the
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amputated hands. The peasant right to the man with cervical deviation seems to have both their of his legs amputated under the knee, the naked stumps appear under the garment. El
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Burgo de Osma (Spain)
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Figure 4. A lady with a baby in her arms and cervical deviation to the left. Note the peasant just besides the woman, he has no hands, perhaps amputated. El Burgo de Osma (Spain)
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Figure 5. This potter is part of the Moche collections of the Museo Nacional de Arqueologiay Antropologıa (Lima). It represents a man with his eyes closed and facial grimacing probably in an attempt to open his eyes.
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Figure 6. A possible representation of cranial dystonia, resembling the classical Meige's Syndrome. This sculpture found in New Orleans Museum of Art (NOMA), US (Xipe-Totec Head, 300-900 AD) shows the closed eyes and lower face dystonia (jaw-opening), but also with tongue dystonia (protrusion). This sculpture made of terracotta is a part of Veracruz Culture heritage from Mexico Figures 7 and 8. The first statue was made probably somewhere in Tokyo graveyard and the second one represents the not-dated art of Melanesya, Papua, New Guinea (New Orleans Museum of Art). Figure 9. “Yawning Man” or “De Gaper”. Pieter Brueghel the Elder, around 1560. Musées Royaux des Beaux-Arts Bruxelles Figure 10. ¨El patizambo (The clubfooted boy)” from
Jose de Ribera, 1642. Louvre
Museum. A beggar with a right hemidystonia probably related with cerebral palsy.
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ACCEPTED MANUSCRIPT Figure 11. “El niño de Vallecas (The boy from Vallecas)” from Diego Velazquez, 1643-45, Museo del Prado, Madrid, depicts a boy with some degree of mental retardation, perhaps affected from cretinism, who has a mixed cervical dystonia, with right torticollis and
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retrocollis
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Figures 12 and 13. “Suffering” from Constantin Brancusi 1907. Museum of Fine Arts.
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Chicago
Figures 14. Portraits of Modigliani. A young woman with dystonic features and sensory tricks.
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Figure 15. “The girl with red hat” from Stanislaw Wyspianski (early 20th century), Upper
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Limb Dystonia (Spastic Dystonia?), Poznan, National Museum, Poznan, Poland
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ACCEPTED MANUSCRIPT HIGHLIGHTS
It is possible to find precise descriptions of dystonia in art
There are sculptures in European cathedrals representing movement disorders
Brueghel and Velazquez represented figures with presumed dystonic postures
Descriptions of dystonic postures can be found (including Brancusi) in modern art
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SC R
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S0022-510X(15)00390-1 doi: 10.1016/j.jns.2015.06.049 JNS 13878
To appear in:
Journal of the Neurological Sciences
Received date: Revised date: Accepted date:
7 April 2015 17 June 2015 23 June 2015
Please cite this article as: Pedro J. Garcia- Ruiz, Jaroslaw Slawek, Emilia J. Sitek, Juan Carlos Martinez Castrillo, Art and Dystonia, Journal of the Neurological Sciences (2015), doi: 10.1016/j.jns.2015.06.049
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT ART AND DYSTONIA. Pedro J Garcia- Ruiz MD PhD(1) *
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Jaroslaw Slawek MD PhD (2) *
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Juan Carlos Martinez Castrillo MD PhD (3) *
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Emilia J. Sitek MA, PhD (2)
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1. Movement Disorder Unit. Department of Neurology. Fundacion Jimenez Diaz. Madrid. Spain
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2. Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Gdansk, Poland and Department of Neurology, St. Adalbert Hospital, Copernicus PL Sp. z o.o, Gdansk, Poland
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Cajal. Madrid. Spain
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3. Movement Disorder Unit. Department of Neurology. IRYCIS. Hospital Ramon y
*These authors equally contributed to this work
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Address of corresponding author: Pedro J Garcia-Ruiz
Department of Neurology Fundacion Jimenez Diaz Avda Reyes Católicos 2 Madrid 28040 Email: [email protected] Telephone: 3491 5504882
Key words: Dystonia; Art; History; Movement Disorders; Medieval Art; Brancusi; Modigliani; Wyspianski
1
ACCEPTED MANUSCRIPT ABSTRACT Dystonia has a recent history in medicine. Focal dystonia was described in the 19th century by classic authors including Gowers, whilst generalized dystonia was described at the turn of the
T
century. However, it is possible to find precise descriptions of dystonia in art, centuries
IP
before the medical definition. We have reviewed several pieces of art (sculpture, painting and
SC R
literature) across the history that might represent descriptions of dystonia, from ancient period to nowadays.
In classic times, the first reference to abnormal postures can be tracked back to the new
NU
Empire of Egypt (equinus foot), not to mention some recently described examples of dystonia from the Moche sculptures in Peru or Veracruz culture from Mexico. In Middle Ages it is possible to find many examples of sculptures in European cathedrals representing peasants
MA
with dramatic, presumably dystonic postures that coexist with amputation of limbs. This unique combination of dystonia and limb amputation probably represents ergotism. The
D
painters Brueghel, Ribera and Velazquez also represented figures with postures likely to be dystonic. Literature is also a source of precise pre-neurological
descriptions, especially
TE
during the 19th century. In David Copperfield, Dickens depicts characters with generalized dystonia (Uriah Heep), cervical dystonia (Mr Sharp) and spasmodic dysphonia (Mr Creakle).
CE P
Finally, even in modern Art (19th and 20th centuries), there are dramatic descriptions of abnormal postures that are likely to be dystonic, such as painful cervical dystonia (Brancusi), cervical dystonia with sensory trick (Modigliani) and upper limb dystonia (Wyspianski).
AC
However some postures presented in works of art may simply be a form of artistic expression and only bear unintentional resemblance to the dystonic postures. Art may be a source of neurological information, and that includes primary and secondary dystonia
2
SC R
IP
T
ACCEPTED MANUSCRIPT
Dystonia has a relatively short history in medicine. Focal dystonia as a neurological syndrome was described in 19th century by several authors including Gowers [1] and the American
NU
Neurologist Horatio Wood [2] whilst generalized dystonia was reported at the turn of the 19th and 20th centuries by Luis Barraquer Roviralta in 1897, Marcus Walter Schwalbe in 1907; Hermann Oppenheim and Georg Theodor Ziehen in 1911; and in the same year the Jewish-
MA
Polish neurologists Edward Flatau and Wladyslaw Sterling described the condition [3-5]. However, it is possible to find precise descriptions of dystonia in art, even centuries before the
D
first medical descriptions.
The paintings or sculptures that represent people affected by various conditions usually lack
TE
the documentation of the disorder, so dystonia as the cause of unusual postures or gestures may be only hypothesized. Finally, some postures presented in works of art may simply be a
CE P
form of artistic expression and only bear unintentional resemblance to the dystonic postures. This paper presents selected pieces of art and literature dating from antiquity to modern times,
AC
that are likely to contain the representations of dystonic postures. Rather than a general review this is a personal selection of those pieces that impressed us most
ANCIENT AND CLASSICAL PERIODS The first description of an abnormal posture can be found in Egyptian art (New Empire, Fig. 1). The affected individual had a clearly equinus foot which was attributed to polio [6] (there are no descriptions of polio before the 18th century). An alternative hypothesis may be an equinus gait secondary to cerebral palsy. There are several sources commenting on historical figures affected by neurological diseases, among whom Alexander the Great is mentioned. It was suggested that Alexander the Great suffered from cervical dystonia [7]. His abnormal neck posture was described by Plutarch, who noted that Lyssipus, the emperor´s personal sculptor, modelled the first statue of
3
ACCEPTED MANUSCRIPT Alexander which represented him looking up with his face turned toward the heavens (Fig. 2). Archelasus, among others, also confirmed Alexander’s abnormal posture. In our opinion, the diagnosis of spasmodic torticollis is questionable as there are various statues representing
SC R
IP
T
Alexander and not all of them exhibit clear cervical deviation.
MIDDLE AGES
Cathedrals all over Europe represent the quintessence of medieval art. Some of the figures
NU
from the tomb of San Pedro de Osma in El Burgo de Osma cathedral (Soria, Spain) show clear cervical dystonic postures and some have other dystonic features or residual limbs (Figs.
MA
3-4).
We suggest that the statues with dystonic features plus residual limbs might represent patients
D
with ergotism, or St Anthony fire [8], which was mainly characterized by peripheral vascular
TE
disease and gangrene resulting in limb loss [9,10]. In addition, ergotism (convulsive ergotism in particular) was also characterized by severe muscular spasms and abnormal postures [10] .
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Ergotism was due to long-term poisoning by Claviceps purpurea fungus, formed on cereals, and widespread all over Europe during XII-XVI centuries (8,9). Ergotism was graphically described in paintings by Mathias Grunewald, in some of his paintings severe dystonia can be recognized [11]. However, some authors suggest that ergotism was in fact a serotonin
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syndrome [10,12]. If this was the case, movement disorders in patients with ergotism can be easily explained, since dystonia, myoclonus and other movement disorders have been associated with serotonin syndrome [10,12].
NEW WORLD: ANCIENT PERIOD Moche artists (from pre-Columbian culture developed in Northern Peru, 100-700 AD) had exceptionally sophisticated ceramic techniques, which they often used to represent their people, particularly individuals affected by diseases or with physical deformities, as they believed that the handicapped were touched by the gods. The potter presented here (Fig. 5) represents a man with his eyes closed and facial grimacing probably in an attempt to open his eyes. The lips are horizontally tensed and the nasolabial folds are pronounced. In our opinion,
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is shown at Fig. 6. The sculpture (Xipe-Totec Head, 300-900 AD) shows the closed eyes and
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lower face dystonia (jaw-opening), but also tongue dystonia (protrusion). This sculpture made
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of terracotta is a part of Veracruz Culture heritage from Mexico.
Sensory tricks (fr. gestes antagonistes) are characteristic features of dystonia. Figs. 7 and 8 represent examples resembling such movements. The first one was made probably somewhere
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in Tokyo graveyard and the second one represents art of Melanesia, Papua, New Guinea
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MODERN HISTORY
The great Flemish Renaissance painter Pieter Brueghel the Elder had a fascination with
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human physiognomy, both normal and pathologic. Historians of art contend that Fig. 9 depicts
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a huge yawn and have titled this work “Yawning Man” or “De Gaper”. However, Marsden credited R.E. Kelly for noting the similarity of this painting to the syndrome of
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blepharospasm-oromandibular dystonia and suggested the eponym "Brueghel's syndrome" [14]. Marsden claimed that Brueghel "clearly recognized the syndrome", but of course, it may be an overinterpretation of this undated, and untitled painting. ¨El patizambo¨ or ¨The clubfooted boy” (Jose de Rivera, 1642, Fig 10) is a representation of a
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beggar with a right hemidystonia. Velazquez painted in “The boy from Vallecas”, (1643-45, Fig. 11) depicts a boy with some degree of mental retardation and mixed cervical dystonia with a right torticollis and retrocollis.
CONTEMPORARY HISTORY The turn of the 19th and 20th centuries was the time of great transformation in art from classical figurative or descriptive forms to very modern, expressive and abstractive forms. It is possible to find descriptions of dystonia in contemporary art; this is the case of “Suffering” from Constantin Brancusi (1876-1952) (Figs. 12 and 13), which represents a boy with a very notorious painful cervical deviation. Brancusi is believed to have observed and represented a real patient with cervical dystonia [15]
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paintings actually represented real people with dystonia.
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Stanislaw Wyspiaski, born in Cracovia (1869-1907) was also an interesting author at the turn
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of the 19th and 20th centuries. This artist practised a variety of arts including poetry, theatre and painting. He worked fast and with extraordinary intensity, his highly diverse output produced in a mere dozen years. We show one of his most realistic and beautiful artworks
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(The girl with red hat, Fig. 15), a young lady with upper limb dystonia, possibly related to cerebral palsy.
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LITERATURE AND DYSTONIA. CHARLES DICKENS. Many writers described neurological conditions across the centuries. Charles Dickens (1812-
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1870) had a special interest in individuals displaying neurological symptoms, and his novels are a source of precise neurological descriptions. Brain already quoted some Dickensian
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neurological diagnosis [16]. Besides Pickwickian syndrome, many other neurological
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descriptions can be found in Dickens´ novels [16-19] including David Copperfield [19]: 1. Generalized dystonia: Uriah Heep. This character, one of the villains of the novel is described as a very tall, strange man with Marfan-like features: "Uriah, with his long knees
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drawn up under his coffee-cup¨. Uriah Heep displayed clear dyskinesias, most probably generalized dystonia:
"He writhed himself quite off his stool in the excitement of his
feeling...” " He had a snaky undulation pervading his frame from his chin to his boots...", "...Uriah exclaimed, with a sickly, angular contortion of himself...”,"... I am sure, said Uriah, writhing himself into the silence like a Conger eel¨ 2. Spasmodic dysphonia:
Mr Creakle.
¨ The circumstances about Mr Creakle which
impressed me most was, that he had no voice, but spoke in a whisper. The exertion this cost him, or the consciousness of talking in that feeble way, made his angry face so much angry, and his thick veins so much thicker when he spoke¨ As Mr Creakle struggled to make his voice audible, he had no voice but a whisper, spasmodic dysphonia (abductor type) could be a plausible explanation of this presentation. 3. Mr Sharp: Cervical dystonia. ¨Mr Sharp was a limp, delicate-looking gentleman, with good deal of nose, and a way of carrying his head on one side, as if it were a little too heavy for
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cervical dystonia.
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SUMMARY
In summary, we have reviewed a personal selection of art (sculpture, painting and literature) that may represent real patients with dystonia or dystonic movements. Our descriptions add to the growing body of reports on the pieces of art representing movement disorders [20,21].
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Artists from ancient times throughout the centuries and up to now, from all over the world (e.g. from Europa, Asia and the New World) frequently described or depicted neurological
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diseases decades or centuries before the official reports by neurologists. Thus, history of neurology may be enriched by the history of art.
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Finally, we should like to include two recent papers related to art and dystonia: Bono et al
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recently called attention to Giovanni Agostino da Lodi (Italian renaissance painter of the Leonardeschi) who made a clear depiction of cranial-cervical dystonia [22]. Gondim et al
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reviewed neuropsychiatric phenomena described in Cantigas de Santa Maria; a collection of 427 canticles composed between 1252 and 1284 at the Court of King Alfonso X the Wise of Spain (1221-1284). In these cantigas the authors found several descriptions of dystonic
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movements [23].
Author roles:
Pedro J Garcia Ruiz: Conception and design, acquisition, analysis and interpretation of data, drafting the submitted material, administrative support Jaroslaw Slavek: Conception and design, acquisition, analysis and interpretation of data drafting part of the submitted material, critical revision and supervision Juan Carlos Martinez Castrillo: acquisition, analysis and interpretation of data; critical revision and supervision Emilia J. Sitek: acquisition of data, analysis and interpretation of data; critical revision and editorial support
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No conflict of interests reported
REFERENCES
Philadelphia: P Blakiston, 1888:1357
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1. Gowers W R. In: Gowers WR ed. A Manual of diseases of the nervous system.
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2. Wood HC. Nervous diseases and their diagnosis: a treatise upon the phenomena produced by diseases of the nervous system. Philadelphia; JB Lippincott,: 1887: 1937
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3. Oppenheim H. Dysbasia lordotica progressiva, Dystonia musculurum deformans.
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6. Levin S. Crippling disorders in the Bible. S Afr Med J. 1957 ;31:16-7 7. Lascaratos J, Damanakis A. Ocular torticollis: A new explanation for the abnormal head posture of Alexander the Great. Lancet 1996;347:521-522.
8. García Ruíz P, Ruíz Ezquerro JJ, García Torres A, Fanjul S. Ancient descriptions of movement disorders: Cathedral el Burgo de Osma (Soria, Spain). J Neurol 2006; 253:731-734.
9. Ayarragaray JE. Ergotism: a change of perspective. Ann Vasc Surg. 2014;28:265-8 10. Eadie MJ. Convulsive ergotism: epidemics of the serotonin syndrome? Lancet Neurol 2003 2:429-434
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ACCEPTED MANUSCRIPT 11. Garcia de Yebenes J, Garcia de Yebenes P. Dystonia in the painting of Matias Grunewald. Epidemic ergotism in the late middle ages. Arch Neurobiol 1991; 54:3740.
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12. Bodner RA, Lynch T, Lewis L, Khan D. Serotonin syndrome. Neurology
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syndrome. Mov Disord. 2010;25:405-6
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13. Martinez-Castrillo JC, Mariscal A, Garcia-Ruiz P. Ancient representation of Meige
14. Marsden CD. Blepharospasm-oromandibular dystonia syndrome (Brueghel's
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syndrome). A variant of adult-onset torsion dystonia? J Neurol Neurosurg Psychiatry 1976;39:1204-9.
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15. Garcia Ruiz PJ, Martinez Castrillo JC. Cervical dystonia and Constantin Brancusi. Mov Disord. 2009; 24:1849-50
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16. Brain R. Dickensian diagnoses. Br Med J 1955;ii:1553-6.
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1991;54: 184-7
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17. Cosnett J E. Dickens, dystonia and dyskinesia. J of Neurol Neurosurg Psychiatry
18. Cosnett JE. Charles Dickens: Observer of sleep and its disorders. Sleep 1992;15:264267
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19. Garcia Ruiz P J, Gulliksen L. Movimientos anormales en David Copperfield. Neurología 1999; 14: 359-360
20. Smith PEM. Neurology in the National Gallery. J R Soc Med 1999; 92:649-52. 21. Clifford Rose F. The Neurology of Art: An overview. In: Clifford Rose F. ed. Neurobiology of the arts. London: Imperial College Press, 43-76 2004
22. Bono F, Morelli M, Quattrone A. Facial dystonia as depicted in art in the time of Leonardo da Vinci. Lancet Neurol. 2015;14:351
23. Gondim Fde A, Griesbach SH, Thomas FP. Neuropsychiatric phenomena in the medieval text Cantigas de Santa Maria. Neurology. 2015;84: 1991-6
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FIGURES
Figure 1. Funeral stele of the priest Ruma (XIX dynasty). Around 1500 BC, Carlsberg Museum of Copenhagen
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Figure 2. Head of Alexander the Great, Lyssipus. Istambul Archeological Museum Figure 3. Peasant with head tilt to the right and painful expression (center), note the
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amputated hands. The peasant right to the man with cervical deviation seems to have both their of his legs amputated under the knee, the naked stumps appear under the garment. El
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Burgo de Osma (Spain)
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Figure 4. A lady with a baby in her arms and cervical deviation to the left. Note the peasant just besides the woman, he has no hands, perhaps amputated. El Burgo de Osma (Spain)
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Figure 5. This potter is part of the Moche collections of the Museo Nacional de Arqueologiay Antropologıa (Lima). It represents a man with his eyes closed and facial grimacing probably in an attempt to open his eyes.
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Figure 6. A possible representation of cranial dystonia, resembling the classical Meige's Syndrome. This sculpture found in New Orleans Museum of Art (NOMA), US (Xipe-Totec Head, 300-900 AD) shows the closed eyes and lower face dystonia (jaw-opening), but also with tongue dystonia (protrusion). This sculpture made of terracotta is a part of Veracruz Culture heritage from Mexico Figures 7 and 8. The first statue was made probably somewhere in Tokyo graveyard and the second one represents the not-dated art of Melanesya, Papua, New Guinea (New Orleans Museum of Art). Figure 9. “Yawning Man” or “De Gaper”. Pieter Brueghel the Elder, around 1560. Musées Royaux des Beaux-Arts Bruxelles Figure 10. ¨El patizambo (The clubfooted boy)” from
Jose de Ribera, 1642. Louvre
Museum. A beggar with a right hemidystonia probably related with cerebral palsy.
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ACCEPTED MANUSCRIPT Figure 11. “El niño de Vallecas (The boy from Vallecas)” from Diego Velazquez, 1643-45, Museo del Prado, Madrid, depicts a boy with some degree of mental retardation, perhaps affected from cretinism, who has a mixed cervical dystonia, with right torticollis and
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retrocollis
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Figures 12 and 13. “Suffering” from Constantin Brancusi 1907. Museum of Fine Arts.
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Figures 14. Portraits of Modigliani. A young woman with dystonic features and sensory tricks.
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Figure 15. “The girl with red hat” from Stanislaw Wyspianski (early 20th century), Upper
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Limb Dystonia (Spastic Dystonia?), Poznan, National Museum, Poznan, Poland
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ACCEPTED MANUSCRIPT HIGHLIGHTS
It is possible to find precise descriptions of dystonia in art
There are sculptures in European cathedrals representing movement disorders
Brueghel and Velazquez represented figures with presumed dystonic postures
Descriptions of dystonic postures can be found (including Brancusi) in modern art
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