Charles II of Spain  

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Charles II was the last of the Spanish Habsburg dynasty, physically disabled, mentally retarded and disfigured (possibly through affliction with mandibular prognathism — he was unable to chew). His tongue was so large that his speech could barely be understood, and he frequently drooled. He may also have suffered from the bone disease acromegaly.

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Train wreck at Montparnasse (October 22, 1895) by Studio Lévy and Sons.
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Train wreck at Montparnasse (October 22, 1895) by Studio Lévy and Sons.

Charles II (6 November 1661, Madrid – 1 November 1700, Madrid) was the last Habsburg King of Spain and the ruler of nearly all of Italy (except Piedmont, the Papal States, The Republic of Lucca and the Republic of Venice), the Spanish territories in the Southern Low Countries, and Spain's overseas Empire, stretching from Mexico to the Philippines. He is noted for his extensive physical, intellectual, and emotional problems – along with the consequent ineffectual rule – as well as his role in the developments preceding the War of Spanish Succession.

Charles was physically and mentally disabled and infertile, possibly due to this massive inbreeding. Due to the deaths of his half brothers, he was the last member of the male Spanish Habsburg line.

By the time of Charles's birth there had been many generations of inbreeding within the Spanish royal house; his physical and mental disabilities are widely attributed to this inbreeding. The practice of first-cousin and uncle-niece marriages was common among 17th-century European nobility, intended to preserve prosperous families' properties. The Habsburgs were an extreme case of this; they had won their extensive holdings mostly through marriages and were determined to keep others from turning the tables on them. Charles's own immediate pedigree was almost exclusively populated with close relative relationships: Charles's mother, Mariana of Austria, herself a Habsburg, was a niece of his father, Philip. Mariana was a daughter of Empress Maria Anna of Spain (1606–46) and Emperor Ferdinand III. Thus Maria Anna was simultaneously his aunt and grandmother while Margaret of Austria, Maria Anna's mother, was both his grandmother and great-grandmother. The inbreeding was so widespread in his case that all of his eight great-grandparents were descendants of Joanna and Philip I of Castile. This inbreeding had given many in the family hereditary weaknesses. That Habsburg generation was more prone to still-births than were peasants in Spanish villages.

There was also mental illness in Charles's family. His great-great-great (-great-great, depending along which lineage one counts) grandmother, Queen Joanna, became insane early in life and became known as "Joanna the Mad."

Dating to approximately the year 1550, outbreeding in Charles II's lineage had ceased (see also pedigree collapse). From then on, all his ancestors were in one way or another descendants of Joanna and Philip I of Castile, and among these just the royal houses of Spain, Austria and Bavaria. Charles II's genome was actually more homozygous than that of a child whose parents are siblings. He was born physically and mentally disabled, and disfigured. Possibly through affliction with mandibular prognathism, he was barely able to chew. His tongue was so large that his speech could barely be understood, and he frequently drooled. It has been suggested that he suffered from the endocrine disease acromegaly, or his inbred lineage may have led to a combination of rare genetic disorders such as combined pituitary hormone deficiency and distal renal tubular acidosis.



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