Friday, April 24, 2015

Friday, April 24, 2015 12:01 am by M. in , , ,    No comments
This is a poster presented at the 67th Annual Meeting of the American Academy of Neurology; April 18-25, 2015; Washington, DC.:
Did the "Woman in the Attic" in Jane Eyre have Huntington Disease? 
Elizabeth Coon and Anhar Hassan
Neurology April 6, 2015 vol. 84 no. 14 Supplement S44.005

To describe features of Huntington disease in Charlotte Brontё’s character, Bertha Mason, in Jane Eyre. 
BACKGROUND: George Huntington’s essay “On Chorea” described adult-onset hereditary chorea in 1872. However, several decades preceding Huntington’s description, familial cases of chorea in children and adults with involuntary movements, speech disturbances, and progressive dementia were published. During this period of enhanced recognition of what is now termed Huntington disease, Charlotte Brontё published Jane Eyre in 1847.
Comparison of Charlotte Brontё’s portrayal of Bertha Mason in Jane Eyre with George Huntington’s original description of Huntington disease.
In Jane Eyre, Brontё features the enigmatic Bertha Mason, known as the “woman in the attic”. Mason had a progressive and familial neuropsychiatric disease with violent movements whose description mirrors the tenets in Huntington’s original essay. One tenet was the “tendency to insanity and suicide.” These behavioral features are prominently featured in Brontё’s text, with descriptions of Mason as a “maniac” with homicidal tendencies who later commits suicide. Mason’s cognitive decline is described as having a “cast of mind common, low, narrow and singularly incapable of being led to anything higher.” Brontё depicts Mason as having abnormal movements, described as wild and animal-like with “convulsive plunges.” Mason’s abnormal movements resemble the description in Huntington’s original essay which is of movements “which gradually increase in violence and variety.”
Brontё’s character has a familial disorder with classic motor, cognitive and behavioral features of Huntington disease. This depiction has had implications for the treatment of patients with neuropsychiatric disease. Brontё’s unsympathetic portrayal of Mason’s neuropsychiatric illness has been deplored by literary critics who brought the issue of humane treatment of neuropsychiatric patients into broader view. This insight remains important today as patients with Huntington disease and their families continue to face stigmatization and prejudicial representation.
Elizabeth Coon, MD, of the Mayo Clinic in Rochester, Minnesota, expands on the theory that Bertha had Huntington’s disease in this video published on Rare Disease Report:


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