hunchback of notre dame

[coconut lime]

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okay, this might sound like a weird question, but has anybody else come across something about the hunchback in their studying? i remember seeing it a while ago, but i just cant remember what it was in reference to. some neuro disorder, i think....anybody know what i'm talking about?

 

[nuclearrabbit77]

http://www.nlm.nih.gov/medlineplus/ency/article/000417.htm

here ya go

 

[coconut lime]

thank you!!

 

[quasimodo]

Quasimodo, the Hunchback of Notre Dame, was also depicted as having neurofibromatosis. He is described as having ?wens? on his skin, lumps and bumps that we know now as neurofibromas. He also had scoliosis (curvature of the spine) and a larger head circumference, both of which are identified as being a symptom or characteristic of neurofibromatosis type 1. It is also possible, though by no means certain, that Quasimodo?s creator, Victor Hugo, was also affected but, even if he was not, he did accurately describe Nf1 in his most famous character

http://www.nfauk.org/intro_to_nf.php

 

[dancelover]

I just had a question on Q-bank, and in the answer they mention that the hunchback of notre dame might have had a mucopolyosaccaroidosis (sp?) disease. Like Hurlers.

 

[bigfrank]

He could have had:

I-Cell Disease (def. Mannose-6-Phosphate addition in Golgi that targets lysosomal proteins like acid hydrosylases to lysosomes)

Hurler's Disease (iduronidase def.)

These 2 are extremely similar clinically.

 

[BrooklynDO]

wouldnt hunters be more likely (ie survivable) than hurlers?

 

[bigfrank]

Yea, that's a good point, but there can be significant variation in expression for Hurler's. Hunter's is typically less severe and does not classicly include the "gargoyle facies" that described the Hunchback.

 

[BrooklynDO]

last time i read that book was before i spoke english lol
so yea i guess youre right, i dunno what he described

 

[Stinger86]

...sure sounds like a lame-o qbank question