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Hunches on Hunchbacks Endocrinology and the Arts Hunches on hunchbacks Krishna G. Seshadri Department of Endocrinology Diabetes and Metabolism, Ramachandra University, Porur, Chennai, India ABSTRACT The hunchback has long been a symbol of revilement in art and literature. This write up tries to find the cause of the deformity into two iconic hunchbacks in literature, Manthara and Quasimodo. Key words: Chondroepithelial dysplasia, cleidocranial dysplasia, dowager’s hump, Manthara, osteoporosis, Schuerman’s disease, The Hunchback of Notre dame, Valmiki, Victor Hugo Artists and writers have the knack of harnessing the she had the hump for at least 20 years – does not sound grotesque to make a point. Thus, dysmorphic features and like an osteoporotic fracture; the caveat is that Manthara bony deformities have a special place in literature and art; had been with Kaikeyi since her birth (probably her wet none has captivated the imagination of the writer and the nurse too) and Kaikeyi did not have a child for quite a long artist more than a crook or curve in the spine. time. Manthara was probably quite old by the time Rama used her back to practice archery, leaving the osteoporosis Let us start with a familiar icon: Manthara [Figure 1], called door open. “kooni” because her spine was bent. Valmiki introduces us to Manthara in sarga 7 of the Ayodhya Kanda.[1] He uses That brings us to the most famous hunchback of them the term “Kubjayai;” kubja[2] translates as humpback or of all – Quasimodo [Figure 2]. Victor Hugo wrote his crooked (or the Latin Gibbus). Valmiki does not elaborate classic in 1839; since then, it has been immortalized in film on her illness further other than calling her “papa darshini” and stage and now endeared to children everywhere by (purveyor of sin). We have to look to other sources for Disney. The “Hunchback of Notre dame” was introduced Manthara and her deformity’s origins. The Padma Purana in epiphany of 1482 when he was crowned the “pope of tells us that she was a gandharva woman sent down fools.” Victor Hugo provides us more clues than Valmiki specifically to ensure that Rama ends up in the forest and or Kamba did: fulfills his destiny. She had accompanied Kaikeyi from her parent’s house – no mention if she had a humpback then. “…the grimace was his face. Or rather, his whole person Kamba (in his rendition of the Ramayana), while seeking was a grimace. A huge head, bristling with red hair; between to explain Manthara’s contempt for Rama, digs in to his his shoulders an enormous hump (italics are mine), a counterpart childhood and finds an episode when Rama used to hit perceptible in front; a system of thighs and legs so strangely Manthara’s hump with balls of clay. By Kamba’s rendition, astray that they could touch each other only at the knees, and, viewed from the front, resembled the crescents of two scythes joined by the handles; large feet, monstrous hands; and, with all this Access this article online deformity, an indescribable and redoubtable air of vigor, Quick Response Code: Website: agility, and courage – strange exception to the eternal rule www.ijem.in which wills that force as well as beauty shall be the result of harmony.”[3] DOI: 10.4103/2230-8210.93772 He further describes. “…this species of cyclops appeared on the threshold of the chapel, motionless, squat, and Corresponding Author: Prof. Krishna G. Seshadri, Department of Endocrinology Diabetes and Metabolism, Room #2 A1, Private Clinic, Sri Ramachandra Medical Center, Sri Ramachandra University, Porur, Chennai - 600 116, India. E-mail: [email protected] 292 Indian Journal of Endocrinology and Metabolism / Mar-Apr 2012 / Vol 16 | Issue 2 Seshadri: Hunches on hunchbacks almost as broad as he was tall; squared on the base.” To top it all, he was deaf, whether from birth or from ringing the chapel’s bell no one could tell. This is manna for the medical detective. Medical mythology clubs Quasimodo with the elephant man as having neurofibromatosis I (the elephant man probably had Proteus syndrome).[4] But this thought process assumes that the kyphosis is not due to a skeletal anomaly. What if it were? Given the accompanying features, one can exclude Schuermann’s disease or osteoporosis, even tuberculosis; clearly the condition is congenital. Key in kyphosis genu valgumat OMIM and you get 25 different congenital syndromes (too wide to fit into a two-page column); cyclops does not help as it could be just a fold Figure 1: Manthara and Kaikeyi of skin caused by the deformities. If we take a leap of faith and add deafness as a congenital accompaniment, we are left with three conditions: (1) spondyloepiphyseal dysplasia (SED) with congenital joint dislocations (10q22.1), (2) a variant of the Stickler syndrome, and (3) cleidocranial dysplasia (CCD).[5] The red hair does not narrow it any further. Many of the features of Quasimodo fit SED, especially an Omani kindred that was described.[6] Disorders of collagen including the Stickler syndrome and Kniest dysplasia may explain some, if not all, of the features of Quasimodo. CCD appears to be a closer fit. The main clinical features of CCD include persistently open skull sutures with bulging calvaria, hypoplasia or aplasia of the clavicles permitting abnormal facility in apposing the shoulders, wide pubic Figure 2: Quasimodo (1939 rendition by Charles Laughton) symphysis, short middle phalanx of the fifth fingers, dental anomalies, and often vertebral malformation. Other features include genuavalga, scoliosis, pesplanus, sinus infections, upper respiratory complications, recurrent otitis media, and hearing loss.[7] Hugo does not tell us how many fingers Quasimodo had; that secret unfortunately will remain with Esmerelda – or so we thought. It appears that Victor Hugo’s Quasimodo was not just a figment of the great writer’s imagination – he was inspired by a real life Quasimodo. The memoirs of Henry Sibson, a 19th century sculptor and contemporary of Victor Hugo working at the Notre Dame Cathedral, describe a hunched back stone mason who lived there. Sibson writes of a stone sculptor with a humpback, called Trajan. He was called by his coworkers as “Mon. Le Bossu.” Le Bossu is Figure 3: Olemec Hunchback. Ceramic. Metropolitan Museum of Art French for “the hunchback.” The Almanach de Paris from 1833 – which gives a list of all professionals working in the city – names a sculptor “Trajin” as living in Saint Germain- Oscar Wilde once said, “Life imitates art far more than des-Pres, where Hugo also lived at the time.[8] So, it is quite art imitates life.”[9] In Hugo’s case, he may have missed the possible that the Quasimodo was not a figment of Victor mark. For the endocrinologist interested in the esoteric, life Hugo’s imagination, but a real person immortalized in word! and art together make good copy. Indian Journal of Endocrinology and Metabolism / Mar-Apr 2012 / Vol 16 | Issue 2 293 Seshadri: Hunches on hunchbacks It is important to remember that the Quasimodos of the 5. Available from: http://www.omim.org. [Last accessed 2012 Jan]. ancient world were subject to much abuse and societal 6. Tuysuz B, Mizumoto S, Sugahara K, Celebi A, Mundlos S, Turkmen S. Omani-type spondyloepiphyseal dysplasia with cardiac involvement discrimination. I did find one exception though – in caused by a missense mutation in CHST3.Clin Genet 2009;75:375-83. Mesoamerica. It appears that among the Olemecs who 7. Cooper SC, Flaitz CM, Johnston DA, Lee B, Hecht JT. A natural inhabited Mexico, hunchbacks were considered to have history of cleidocranial dysplasia. Am J Med Genet 2001;104:1-6. 8. Nikkah R. Real-life Quasimodo uncovered in Tate archives. The special abilities because of their deformity, including Telegraph, Aug 10, 2010.Available from: http://www.telegraph. access to special realms; consequently, they enjoyed royal co.uk/culture/books/artsandentertainmentbooksreview/7945634/ patronage [Figure 3].[10] Real-life-Quasimodo-uncovered-in-Tate-archives.html.[Last accessed 2012 Jan]. 9. Wilde O. The decay of lying: An observation. Available from: http:// REFERENCES www.online-literature.com/wilde/1307/. [Last accessed 2012 Jan]. 10. Hunchback [Mexico; Olmec] (1989.392)”. In Heilbrunn Timeline 1. Valmiki. Ramayana. Ayodhya Kanda Sarga 7 Verse 17. of Art History. New York: The Metropolitan Museum of Art;2000. 2. Williams M. Sanskrit – English Dictionary. Available from: http://www. Available from: http://www.metmuseum.org/toah/works-of- sanskrit-lexicon.uni-koeln.de/monier/.[Last accessed 2012 Jan]. art/1989.392. [Last accessed 2012 Jan] 3. Victor H. The hunchback of notredame. Chapter 5.Available from: http://www.online-literature.com/victor_hugo/hunchback_notre_ Cite this article as: Seshadri KG. Hunches on hunchbacks. Indian J Endocr dame/6/.[Last accessed 2012 Jan]. Metab 2012;16:292-4. 4. Morse RP. Neurofibromatosis Type 1. Arch Neurol 1999;56:364-5. Source of Support: Nil, Conflict of Interest: None declared. 294 Indian Journal of Endocrinology and Metabolism / Mar-Apr 2012 / Vol 16 | Issue 2 Copyright of Indian Journal of Endocrinology & Metabolism is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use..
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