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Indian Journal of Endocrinology and Metabolism / Mar-Apr 2012 / Vol 16 | Issue 2242

Hunches on hunchbacks

Krishna G. Seshadri

Department of Endocrinology Diabetes and Metabolism, Ramachandra University, Porur, Chennai, India

A B S T R A C T

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, dowagers hump, Manthara, osteoporosis, Schuermans disease,

The Hunchback of Notre dame, Valmiki, Victor Hugo

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INTRODUCTION???

Artists and writers have the knack of harnessing the

grotesque to make a point. Thus, dysmorphic features and

bony deformities have a special place in literature and art;

none has captivated the imagination of the writer and the

artist more than a crook or curve in the spine.

Let us start with a familiar icon: Manthara [Figure 1], called

kooni because her spine was bent. Valmiki introduces us

to Manthara in sarga 7 of the Ayodhya Kanda.[1] He uses

the term Kubjayai; kubja[2] translates as humpback or

crooked (or the Latin Gibbus). Valmiki does not elaborateon her illness further other than calling her papa darshini

(purveyor of sin). We have to look to other sources for

Manthara and her deformitys origins. The Padma Purana

tells us that she was a gandharva woman sent down

specifically to ensure that Rama ends up in the forest and

fulfills his destiny. She had accompanied Kaikeyi from her

parents house no mention if she had a humpback then.

Kamba (in his rendition of the Ramayana), while seeking

to explain Mantharas contempt for Rama, digs in to his

EQ1

childhood and finds an episode when Rama used to hit

Mantharas hump with balls of clay. By Kambas rendition,

she had the hump for at least 20 years does not sound

like an osteoporotic fracture; the caveat is that Manthara

had been with Kaikeyi since her birth (probably her wet

nurse too) and Kaikeyi did not have a child for quite a long

time. Manthara was probably quite old by the time Rama

used her back to practice archery, leaving the osteoporosis

door open.

That brings us to the most famous hunchback of them

of all Quasimodo [Figure 2]. Victor Hugo wrote his

classic in 1839; since then, it has been immortalized in film

and stage and now endeared to children everywhere by

Disney. The Hunchback of Notre dame was introduced

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: krishnagseshadri@gmail.com

Figure 1: Manthara and Kaikeyi

No heading

needed

Endocrinology and

the Arts

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Indian Journal of Endocrinology and Metabolism / Mar-Apr 2012 / Vol 16 | Issue 2 243

Seshadri: Hunches on hunchbacks

in epiphany of 1482 when he was crowned the pope of

fools. Victor Hugo provides us more clues than Valmiki

or Kamba did:

the grimace was his face. Or rather, his whole person

was a grimace. A huge head, bristling with red hair; between

his shoulders an enormous hump (italics are mine), a counterpart

perceptible in front; a system of thighs and legs so strangelyastray that they could touch each other only at the knees, and,

viewed from the front, resembled the crescents oftwo scythes

joined by the handles; large feet, monstrous hands; and, with all this

deformity, an indescribable and redoubtable air of vigor,

agility, and courage strange exception to the eternal rule

which wills that force as well as beauty shall be the result

of harmony.[3]

He further describes. this species of cyclops appeared

on the threshold of the chapel, motionless, squat, and

almost as broad as he was tall; squared on the base. To

top it all, he was deaf, whether from birth or from ringingthe chapels bell no one could tell.

Th is is manna for the medical detect ive. 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 Schuermanns disease or osteoporosis, even

tuberculosis; clearly the condition is congenital. Key inkyphosis 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

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 Quasimodofit SED, especially anOmani 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

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 manyfingers Quasimodo had;

that secret unfortunately will remain with Esmerelda or so

we thought. It appears that Victor Hugos Quasimodo was

not just a figment of the great writers imagination he was

Figure 2: Quasimodo (1939 rendition by Charles Laughton) Figure 3: Olemec Hunchback. Ceramic. Metropolitan Museum of Art

Figure: ???1

Delete figure2 since it

may infringe

copyright

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Indian Journal of Endocrinology and Metabolism / Mar-Apr 2012 / Vol 16 | Issue 2244

Seshadri: Hunches on hunchbacks

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

French for the hunchback. The Almanach de Paris from

1833 which gives a list of all professionals working in thecity names a sculptor Trajin as living in Saint Germain-

des-Pres, where Hugo also lived at the time. [8] So, it is quite

possible that the Quasimodo was not a figment of Victor

Hugos imagination, but a real person immortalized in word!

Oscar Wilde once said, Life imitates art far more than

art imitates life.[9] In Hugos case, he may have missed the

mark. For the endocrinologist interested in the esoteric, life

and art together make good copy.

It is important to remember that the Quasimodos of the

ancient world were subject to much abuse and societaldiscrimination. I did find one exception though in

Mesoamerica. It appears that among the Olemecs who

inhabited Mexico, hunchbacks were considered to have

special abilities because of their deformity, including

access to special realms; consequently, they enjoyed royal

patronage [Figure 3].[10]

REFERENCES

1. Valmiki. Ramayana. Ayodhya Kanda Sarga 7 Verse 17.

2. Williams M. Sanskrit English Dictionary. Available from: http://www.

sanskrit-lexicon.uni-koeln.de/monier/.[Last accessed 2012 Jan].

3. Victor H. The hunchback of notredame. Chapter 5.Available from:

http://www.online-literature.com/victor_hugo/hunchback_notre_

dame/6/.[Last accessed 2012 Jan].

4. Morse RP. Neurofibromatosis Type 1. Arch Neurol 1999;56:364-5.

5. Available from: http://www.omim.org. [Last accessed 2012 Jan].

6. Tuysuz B, Mizumoto S, Sugahara K, Celebi A, Mundlos S, Turkmen S.

Omani-type spondyloepiphyseal dysplasia with cardiac involvement

caused by a missense mutation in CHST3.Clin Genet 2009;75:375-83.

7. Cooper SC, Flaitz CM, Johnston DA, Lee B, Hecht JT. A natural

history of cleidocranial dysplasia. Am J Med Genet 2001;104:1-6.

8. Nikkah R. Real-life Quasimodo uncovered in Tate archives. The

Telegraph, Aug 10, 2010.Available from: http://www.telegraph.

co.uk/culture/books/artsandentertainmentbooksreview/7945634/

Real-life-Quasimodo-uncovered-in-Tate-archives.html .[Last

accessed 2012 Jan].

9. Wilde O. The decay of lying: An observation. Available from: http://

www.online-literature.com/wilde/1307/. [Last accessed 2012 Jan].

10. Hunchback [Mexico; Olmec] (1989.392). In Heilbrunn Timeline

of Art History. New York: The Metropolitan Museum of Art;2000.

Avai lable from: ht tp:/ /www.metmuseum.org/ toah/works-of-

art/1989.392. [Last accessed 2012 Jan]

Cite this article as: Citation will be included before issue gets online***.

Source of Support: Nil, Conflict of Interest: None declared.

Author Queries???AQ1: Kindly confirm the article type.AQ2: Kindly provide year.EQ1: Introduction missing???TQ1: Please check the figure and not provide

caption and citation and citation

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