Content uploaded by Andrei Cucu
Author content
All content in this area was uploaded by Andrei Cucu on Jun 12, 2019
Content may be subject to copyright.
Historical Note
Eur Neurol 2019;81:76–78
Hydrocephalus of King Charles II of
Spain, the Bewitched King
Mihaela Dana Turliuc
a, b Andrei Ionut Cucu
a Antonio Perciaccante
c
Giulia Tosolini
d Stefano De Luca
c Bogdan Costachescu
a, b
Claudia Florida Costea
e
a 2nd Neurosurgery Clinic, “Prof. Dr. N. Oblu” Emergency Clinical Hospital, Iasi, Romania; b Department of
Neurosurgery, “Grigore. T. Popa” University of Medicine and Pharmacy, Iasi, Romania; c Department of Medicine,
“San Giovanni di Dio” Hospital, Gorizia, Italy; d Department of Foreign Languages, Literature, Education and
Communication, University of Udine, Udine, Italy; e Department of Ophthalmology, “Grigore. T. Popa” University of
Medicine and Pharmacy, Iasi, Romania
Received: April 3, 2019
Accepted: May 1, 2019
Published online: May 21, 2019
Andrei Ionut Cucu, PhD
2nd Neurosurgery Clinic
“Prof. Dr. N. Oblu” Emergency Clinical Hospital
RO–2 Ateneului Street, 700309 Iasi (Romania)
E-Mail andreiucucu @ yahoo.com
© 2019 S. Karger AG, Basel
E-Mail karger@karger.com
www.karger.com/ene
DOI: 10.1159/000500719
Keywords
Hydrocephalus · Herpetic infection · Charles II
Abstract
King of the Spanish Habsburg dynasty and sovereign of the
overseas Spanish Empire, Charles II of Spain, was physically
disabled, disfigured, mentally retarded, and he proved im-
potent. He is known in history as El Hechizado (the Bewitched)
because both him and the people believed that his mental
and physical incapacity were due to a “witchcraft act.” Al-
though several authors speculated about different diseases,
most of them genetic such as pituitary hormone deficiency,
distal renal tubular acidosis, Klinefelter syndrome, fragile X
syndrome, or male XX hermaphroditism, the hypothesis of
hydrocephalus was not taken into account. We don’t have
clear elements to hypothesize a certain etiology of Charles II’
hydrocephalus; however, we think the herpetic infection he
suffered of after his birth should not be ignored.
© 2019 S. Karger AG, Basel
Charles II of Spain, Carlos Segundo (1661–1700; Fig.1)
was the last king of the Spanish Habsburg dynasty and sov-
ereign of the overseas Spanish Empire, from Mexico up to
Philippine. Charles was the only surviving son of his pre-
decessor, Philip IV and his second wife, Mariana of Aus-
tria. His birth was awaited with enthusiasm by the Spanish
people, who were afraid of the conflict that would have
broken out if Philip IV had died without any male heirs.
La Gaceta de Madrid spread the word of his birth, de-
scribing the crown prince as “a robust man, very
handsome...a proportionate head, black hair...”. Interest-
ingly, not long after, a report sent to the French King Lou-
is XIV described a totally different situation: “the prince
seems extremely weak, he has a herpetic on both cheeks.
The head is covered with scabs, a kind of channel or
drainage that oozes underneath the right ear [1, 2].
The king was physically disabled, disfigured, mentally
retarded, and he proved impotent, since no children were
born from his 2 marriages [3]. Furthermore, throughout
Hydrocephalus of King Charles II of
Spain
77
Eur Neurol 2019;81:76–78
DOI: 10.1159/000500719
his life, he has been suffering from a number of different
diseases [4–6], and he did not speak until the age of 4 or
walk until the age of 8–10 [1, 7]. Aged 6–10 years, he had
measles, varicella, rubella, and smallpox [2]. He was treat-
ed like a baby until the age of 10 and fearing that this frag-
ile child shall be overworked, he was left completely un-
educated [7]. Due to his incapacity, the years of the reign
of Charles II were years of agony for Spain; his mother
was regent most of his reign.
As mentioned above, the king was not only mentally
retarded but also physically disabled and disfigured by a
mandibular prognathism, condition inherited from the
Habsburg family (an extreme example of the so-called
Habsburg jaw), which had been noticed in his forefather,
Charles V, who had the same prominent jaw (Fig.1). The
mandible of Charles II was so badly deformed that he
could barely speak or chew, and thus he had to swallow
his food almost entirely, which often caused him indiges-
tions and vomiting (Fig.1) [8].
He is known in history as El Hechizado (the Bewitched).
Both him and the people he ruled believed that his mental
and physical incapacity were due to a “witchcraft act” [7].
Nevertheless, further researches showed that the strong
preference for endogamy within the Spanish branch of
the Habsburg royal family led to its segregation toward
the neighbor communities and the emergence of consan-
guinity [3, 9].
From the earliest years of life, Charles II was exorcised
in the attempt to heal him, and all the exorcists from the
kingdom were summoned in order to question the de-
mons they used to expel [7]. In addition, St. Isidore’s and
St. Iago’s relics were brought in the royal palace, where
they have remained for 6 months, in the hope that the
King should be healed [10]. He partially recovered from
this disease, yet he remained in a state of mental imbecil-
ity [10], melancholy, and lethargy until the end of his life
[7]: “his mind, too, was a constant prey to a corroding
melancholy, which appears to have been in a great mea-
sure produced by the most ignoble and womanish /super-
stitions” [10].
Moreover, he suffered from increasing seizures in the
last years of his life [2, 3]. The American historians Will
and Ariel Durant described Charles II as “short, lame,
epileptic, senile, and completely bald before 35, he was
always on the verge of death, but repeatedly baffled Chris-
tendom by continuing to live” [11]. Toward the end of his
life, the frail health of Charles started deteriorating even
more, leading to his death at the age of 38, in Madrid.
As for the disease of Charles II, it had yet to be figured
out. Still, several authors speculated about different dis-
eases, most of them, genetic, such as: pituitary hormone
deficiency and distal renal tubular acidosis [3], Klinefelter
syndrome [4], fragile X syndrome [12], or male XX her-
maphroditism associated with a fragile X syndrome [1].
bc
a
Fig. 1. Charles II of Spain, Juan Carreño de Miranda (circa 1685), Museum of Art History, Vienna (a). King
Charles II, Claudio Coello (1675–1680), The Prado Museum, Madrid (b). Portrait of Charles V, Bernard van Or-
ley (circa 1515–1516), Museum of Fine Arts, Budapest (c) (public domain).
Color version available online
Turliuc/Cucu/Perciaccante/Tosolini/
DeLuca/Costachescu/Costea
Eur Neurol 2019;81:76–78
78
DOI: 10.1159/000500719
Whatever his underlying disease, we suggest that Charles’
symptoms and signs, such as macrocephaly, late growth,
and mental development, as well as the frequent episodes
of vomiting and epileptic crisis from his childhood were
related to hydrocephalus.
Our hypothesis is corroborated by the post-mortem
examination, which concluded that the emperor’s corpse
“did not contain a single drop of blood; his heart was the
size of peppercorn; his lungs corroded; his intestines rot-
ten and gangrenous; he had a single testicle, black as coal,
and his head was full of water” [4]. The necropsy noticed
that the ventricles of the brain were filled with cerebro-
spinal fluid, as in hydrocephalus [4, 13]. We don’t have
clear elements to hypothesize a certain etiology of Charles
II’ hydrocephalus; however, we think the herpetic infec-
tion he suffered of after his birth, should not be ignored,
even more as herpetic infections can cause hydrocephalus
[14, 15].
Disclosure Statement
The authors have no conflict or interests. The authors are not
having any financial interest.
References
1 García-Escudero López A, Arruza Echevarría
A, Padilla Nieva J, Puig Giró R. Carlos II: del
hechizo a su patología génito-urinaria. Arch
Esp Urol. 2009 Apr; 62(3): 179–85.
2 Calvo Poyato J. Carlos II el Hechizado. Barce-
lona: Planeta; 1996.
3 Alvarez G, Ceballos FC, Quinteiro C. The role
of inbreeding in the extinction of a European
royal dynasty. PLoS One. 2009; 4(4):e5174.
4 Gargantilla P. Enfermedades de los Reyes de
España, Los Austrias: de la locura de Juana a
la impotencia de Carlos II el Hechizado. Ma-
drid: La Esfera de Los Libros; 2005.
5 Kamen H. Spain, 1469–1714: A society of
conflict. London: Longman; 1983.
6 Bennassar B. La España de los Austrias (1516–
1700). Barcelona: Crıtica; 2000.
7 Littell E. Littell’s living age. Boston: E Littell &
Company; 1849.
8 Du Pin L-E, Abbé Vayrac J de. Histoire des
Révolutions d’Espagne, Tome 5. La Haye:
Chez Jacob Stopp; 1724.
9 Ceballos FC, Alvarez G. Royal dynasties as hu-
man inbreeding laboratories: the Habsburgs.
Heredity. 2013 Aug; 111(2): 114–21.
10 Dunlop J. Memoirs of Spain during the reigns
of Philip IV and Charles II from 1621 to 1700.
Volume II. Edinburgh: Thomas Clark; 1834.
11 Durant W, Durant A. The story of civiliza-
tion: the age of Louis XIV, 1648–1715. New
York: Simon & Schuster; 935.
12 Navalón Ramón E, Ferrando Lucas MT. La
enfermedad de Carlos II. Revista Valenciana
de Medicina de Familia. 2006; 22: 16–9.
13 de Moragas J. De Carlos I Emperador a Carlos
II El Hechizado. Barcelona: Juventud; 1970.
14 Hayashi K, Iwasaki Y, Yanagi K. Herpes sim-
plex virus type 1-induced hydrocephalus in
mice. J Virol. 1986 Mar; 57(3): 942–51.
15 Takano T, Uno M, Yamano T, Shimada M.
Congenital hydrocephalus: role of transpla-
cental myxovirus infection. Congenit Anom
(Kyoto). 1995; 35(1): 15–24.
