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.|
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!”|
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.
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 SullivanFollow Bill on Twitter: @wjsullivan
Deshmukh 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