Hunches on hunchbacks

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.

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 elaborate on her illness further other than calling her “papa darshini” (purveyor of sin). We have to look to other sources for Manthara and her deformity's 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 parent's house – no mention if she had a humpback then. Kamba (in his rendition of the Ramayana), while seeking to explain Manthara's contempt for Rama, digs in to his childhood and finds an episode when Rama used to hit Manthara's hump with balls of clay. By Kamba's 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.

Figure 1.

Manthara and Kaikeyi

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 in epiphany of 1482 when he was crowned the “pope of fools.” Victor Hugo provides us more clues than Valmiki or Kamba did:

Figure 2.

Quasimodo (1939 rendition by Charles Laughton)

“…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 strangely 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 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 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 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 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 19^th 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 the city – 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 Hugo's imagination, but a real person immortalized in word'

Oscar Wilde once said, “Life imitates art far more than art imitates life.”[9] In Hugo's 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 societal discrimination. 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]

Figure 3.

Olemec Hunchback. Ceramic. Metropolitan Museum of Art