Vampires
These blood-sucking creatures of the night are believed
to have been inspired by the Romanian Prince Vlad, born in Transylvania in
1431. His father was named Dracul, and Dracula means “son of Dracul”. Dracula
was a “defender of the Christian faith” who, ironically, gained notoriety by
impaling his victims and dipping his bread in their blood before consuming it.
Prince Vlad before dinner (left) and after dinner (right). |
However, the symptoms of porphyria
(yes…it is pronounced poor-FEAR-e-uh!)
are likely to have contributed to several aspects of vampire lore. While it
sounds like a lost Def Leppard album,
porphyria is actually a blood disorder that arises when patients cannot make
and regulate heme properly (heme is a critical part of hemoglobin in the
blood).
Porphyria can cause excessive nail growth and receding
gums, the latter of which may make the canine teeth look more like fangs.
Moreover, porphyria can cause skin to bubble and blister just minutes following
exposure to sunlight. Look no further than 1985’s cult classic, Fright Night, to witness the awesome
power of sunlight destroying a vampire.
Finally, to make up for the compromised hemoglobin
production, the treatment for porphyria involves injecting patients with blood.
Despite this parallel with vampirism, people with the disease do not “thirst
for blood” or bite others.
Pluto from The Hills Have Eyes
Michael Berryman, best known for his portrayal of Pluto
in the 1977 cult classic, The Hills Have
Eyes, has an unforgettable appearance. While his role as a deranged desert
cannibal haunted the dreams of millions, his character did not require hours in
the make-up chair. Rather, his appearance is attributable to a rare genetic
condition known as hypohidrotic
ectodermal dysplasia. People with this syndrome have fewer sweat glands, sparse
body hair, and missing or abnormal teeth. In addition, facial features of these
individuals tend to be consistent with those seen in Berryman’s photo below.
Other than heat intolerance due to a reduced ability to sweat, people with hypohidrotic
ectodermal dysplasia are otherwise healthy.
Instantly recognizable on screen, Berryman has appeared in dozens of subsequent roles, including multiple appearances in Star Trek episodes and films. |
Werewolves
On the opposite end of the spectrum, people with
excessive hair, especially when it appears on the face, have long been attractions
at circus sideshows. Perhaps the most famous is Julia Pastrana, also
known as “the bearded lady” or “ape woman”, who caught the attention of many
onlookers during her travels with “the freak show” in the 1800s. There is a
name for this condition, which often resembles the classic appearance of a
werewolf: congenital hypertrichosis
lanuginosa, or CHL. Babies born with CHL are usually covered in hair right out
of the womb. Today, people afflicted with CHL can elect to have that hair
removed with lasers.
“Don’t worry, it’s just a little hypertrichosis flare-up!” |
Demonic Possession
Schizophrenia or multiple personality disorders are often
cited as likely explanations for people exhibiting unusual behaviors. But an autoimmune
disease called “anti-NMDA receptor encephalitis” has been recently described
that also produces striking symptoms of demonic possession. A first-hand
account of this ailment was written by Susannah Cahalan called, Brain
on Fire: My Month of Madness.
Incidentally, anti-NMDA receptor encephalitis does not
induce the green projectile vomiting made famous in The Exorcist. You have to go to Taco-Bell for that.
Zombies
There are a number of conditions that give people a
zombie persona, such as that window of time from waking up till you get some
coffee in you. While there is no disease that allows you to persist as an
undead, brain-hungry zombie, there is a famous one that can drive animals to
bite others: rabies. Rabies is caused by a virus, and,
thanks to Stephen King’s Cujo, most of us are
familiar with how rabies can transform a puppy into a hellhound. The virus is
plentiful in the salvia of infected animals and is transmitted through a bite
or scratch. Many pathogens change the behavior of their host in order to
spread. The rabies virus infects the brain in such a way that its host organism
becomes overly aggressive, increasing the odds that the virus will be
transmitted to a new victim through a bite.
Another type of disorder can lead to an eerie change in
behavior with shades of zombification. A rare mental illness called Cotard
delusion, or walking
corpse syndrome, occurs when the afflicted no longer believe they are
alive. First described in 1880 by neurologist Jules Cotard, this “delirium of
negation” can run from mild self-loathing to severe depression. In the most
extreme cases, the afflicted will deny the existence of certain body parts or
their entire body. Consequently, they will stop taking care of themselves, even
to the point of starving to death.
Marilyn Manson or ‘Cotard delusion’ support group? |
For some interesting examples of zombification in
wildlife, be sure to read Mark’s recent post, “Zombies
And The Loss Of Free Will”.
Schulenburg-Brand D, Katugampola R, Anstey AV, & Badminton MN (2014). The cutaneous porphyrias. Dermatologic clinics, 32 (3) PMID: 24891059
Contributed by: Bill Sullivan
Follow Bill on Twitter: @wjsullivanDeshmukh S, & Prashanth S (2012). Ectodermal dysplasia: a genetic review. International journal of clinical pediatric dentistry, 5 (3), 197-202 PMID: 25206167
Kaur S, Juneja M, Mishra D, & Jain S (2014). Anti-N-methyl-D-aspartate receptor encephalitis: A case report and review of the literature. Journal of pediatric neurosciences, 9 (2), 145-7 PMID: 25250071
Ramirez-Bermudez J, Aguilar-Venegas LC, Crail-Melendez D, Espinola-Nadurille M, Nente F, & Mendez MF (2010). Cotard syndrome in neurological and psychiatric patients. The Journal of neuropsychiatry and clinical neurosciences, 22 (4), 409-16 PMID: 21037126
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