Slowly turning to bone may sound like a sorcerous curse, but there's a real syndrome that can do just that. Here we take a look at some of the strangest conditions to affect humans, including...
This genetic condition makes the human body highly susceptible to human papillomavirus, a common disease spread through simple skin contact. People with epidermodysplasia verruciformis may develop a chronic infection, leading to warts, discolored patches of shin or even large growths resembling chunks of bark. In the most extreme cases, the feet or hands can grow to resemble splintered wood.
This condition is usually caused by recessive genes, but it can rarely occur as a dominant mutated gene or be the result of a damaged immune system. There have been around 200 reported cases, with around 10% of sufferers being born to related parents.
Unfortunately the condition cannot be cured, only managed with a regimen of drugs and cosmetic surgery. To make matters even worse, around half of those experiencing this condition will also develop skin cancers.
As it happens, all humans develop a tail as an embryo... but in the overwhelming majority of cases, this structure is absorbed into the developing body and becomes the coccyx. In the rare occasions where it remains, the tail can develop muscles and blood flow - in some cases the bearer of the vestigial tail can learn to move it. In the case of a pseudotail, the structure may feature bones thanks to being linked to the spine or coccyx.
While tails of either kind are quite rare and can be a nuisance or even painful, there are benefits - more than one individual has found themselves associated with the deity Hanuman. This Hindu god blends the characteristics of monkey and man, so humans born with a tail may be venerated by his adherents!
SPOAN is an extremely rare condition. Around eighty cases have been recorded - a couple in Egypt, a few in southern Brazil and the rest in northern Brazil. The syndrome seems most concentrated in the town of Serrinha dos Pintos, where around 1:250 people have SPOAN and 1:15 are thought to be carriers of the responsible genetics.
Because the syndrome is a neurodegenerative disorder carried by recessive genes, it only takes effect if a person has the defective gene from both parents. SPOAN gradually cripples the sufferer, removing the ability to walk before they have reached adolescence. It also impairs vision and causes sensorimotor peripheral neuropathy, though the mental abilities of the victim remain intact.
There is a feature of Serrinha dos Pintos that may explain the overrepresentation of SPOAN. The 5000-strong town is fairly isolated and a surprisingly high proportion of people are related... including around 30% of couples.
If both members of a couple share an ancestor who carried SPOAN, they too may have the defective genetics - and if they are both carriers, their children have a chance to receive two sets of the SPOAN genes and develop the full-blown syndrome.
Sufferers of the appropriately-named foreign accent syndrome can find themselves putting together sentences in the wrong order, substituting or incorrectly using words or even changing the way they pronounce vowels. The overall results vary from person to person - Julie Matthias from Kent in England found herself speaking with an accent more similar to that of a South African or Italian.
The causes of foreign accent syndrome are equally unclear, but various forms of brain damage (including strokes, bleeds, injuries, tumors and multiple sclerosis) have been blamed. Researchers that mapped out lesions (damaged areas) from the brains of 25 people with foreign accent syndrome found they frequently included the bilateral lower and middle portions of the precentral gyrus and in the medial frontal cortex - areas of the brain heavily involved in speech.
Fibrodysplasia ossificans progressiva is a genetic disorder that causes muscle and connective tissue to ossify - or become bone. Making matters worse, any injury (including surgery or even injections) can trigger a flareup and increase the speed of the transformation.
The symptoms begin with a simple stiffness of the neck or shoulders that doesn't go away - this is caused by parts of the soft tissue hardening and becoming bone. By the age of thirty this abnormal bone growth becomes fused to the skeleton, immobilizing affected joints and partially paralyzing the victim. This process continues until it kills the sufferer at around age forty via complications - usually due to difficulty breathing.
Perhaps the scariest thing about fibrodysplasia ossificans progressiva is that many cases are the result of a "fresh" mutation of the ACVR1/ALK2 gene, meaning it is utterly unpredictable. This protein coded by this gene helps turn cartilage into bone as a normal part of skeletal maturation - but a mutation can incorrectly activate this function and ossify soft tissue. The only upside is that this condition is incredibly rare - only one in two million people across the world are thought to be affected.
- Treeman Syndrome
- Humans With a Tail
- The Town Monopolizing SPOAN Syndrome
- Foreign Accent Syndrome
- Stoneman Syndrome
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| The human body is a complicated thing... (Monoar_CGI_Artist) |
Treeman Syndrome
Perhaps an inspiration for depictions of the part-human, part plant from folklore across the world, the colorfully-named treeman syndrome is actually a bad case of epidermodysplasia verruciformis - a condition that can lead to bark-like skin.This genetic condition makes the human body highly susceptible to human papillomavirus, a common disease spread through simple skin contact. People with epidermodysplasia verruciformis may develop a chronic infection, leading to warts, discolored patches of shin or even large growths resembling chunks of bark. In the most extreme cases, the feet or hands can grow to resemble splintered wood.
This condition is usually caused by recessive genes, but it can rarely occur as a dominant mutated gene or be the result of a damaged immune system. There have been around 200 reported cases, with around 10% of sufferers being born to related parents.
Unfortunately the condition cannot be cured, only managed with a regimen of drugs and cosmetic surgery. To make matters even worse, around half of those experiencing this condition will also develop skin cancers.
Humans With a Tail
Did you know that humans can be born with a tail? These structures can be "true" vestigial tails consisting of skin and muscle, or pseudotails that are extensions of the coccyx or a deformation of the spine.As it happens, all humans develop a tail as an embryo... but in the overwhelming majority of cases, this structure is absorbed into the developing body and becomes the coccyx. In the rare occasions where it remains, the tail can develop muscles and blood flow - in some cases the bearer of the vestigial tail can learn to move it. In the case of a pseudotail, the structure may feature bones thanks to being linked to the spine or coccyx.
While tails of either kind are quite rare and can be a nuisance or even painful, there are benefits - more than one individual has found themselves associated with the deity Hanuman. This Hindu god blends the characteristics of monkey and man, so humans born with a tail may be venerated by his adherents!
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| Some humans born with tails have become associated with deities! (reinhold_embacher) |
The Town Monopolizing SPOAN Syndrome
The town of Serrinha dos Pintos in north-eastern Brazil has a dubious honor - it has by far the highest rate of SPOAN syndrome in the world.SPOAN is an extremely rare condition. Around eighty cases have been recorded - a couple in Egypt, a few in southern Brazil and the rest in northern Brazil. The syndrome seems most concentrated in the town of Serrinha dos Pintos, where around 1:250 people have SPOAN and 1:15 are thought to be carriers of the responsible genetics.
Because the syndrome is a neurodegenerative disorder carried by recessive genes, it only takes effect if a person has the defective gene from both parents. SPOAN gradually cripples the sufferer, removing the ability to walk before they have reached adolescence. It also impairs vision and causes sensorimotor peripheral neuropathy, though the mental abilities of the victim remain intact.
There is a feature of Serrinha dos Pintos that may explain the overrepresentation of SPOAN. The 5000-strong town is fairly isolated and a surprisingly high proportion of people are related... including around 30% of couples.
If both members of a couple share an ancestor who carried SPOAN, they too may have the defective genetics - and if they are both carriers, their children have a chance to receive two sets of the SPOAN genes and develop the full-blown syndrome.
Foreign Accent Syndrome
Can you imagine waking up one day with your native accent replaced by that of another country?Sufferers of the appropriately-named foreign accent syndrome can find themselves putting together sentences in the wrong order, substituting or incorrectly using words or even changing the way they pronounce vowels. The overall results vary from person to person - Julie Matthias from Kent in England found herself speaking with an accent more similar to that of a South African or Italian.
The causes of foreign accent syndrome are equally unclear, but various forms of brain damage (including strokes, bleeds, injuries, tumors and multiple sclerosis) have been blamed. Researchers that mapped out lesions (damaged areas) from the brains of 25 people with foreign accent syndrome found they frequently included the bilateral lower and middle portions of the precentral gyrus and in the medial frontal cortex - areas of the brain heavily involved in speech.
Stoneman Syndrome
Mythology features a few creatures (such as the Greek Gorgons) who kill by turning their victims to stone. As it happens, there is an extremely rare condition that slowly turns the human body to bone without supernatural aid.Fibrodysplasia ossificans progressiva is a genetic disorder that causes muscle and connective tissue to ossify - or become bone. Making matters worse, any injury (including surgery or even injections) can trigger a flareup and increase the speed of the transformation.
The symptoms begin with a simple stiffness of the neck or shoulders that doesn't go away - this is caused by parts of the soft tissue hardening and becoming bone. By the age of thirty this abnormal bone growth becomes fused to the skeleton, immobilizing affected joints and partially paralyzing the victim. This process continues until it kills the sufferer at around age forty via complications - usually due to difficulty breathing.
Perhaps the scariest thing about fibrodysplasia ossificans progressiva is that many cases are the result of a "fresh" mutation of the ACVR1/ALK2 gene, meaning it is utterly unpredictable. This protein coded by this gene helps turn cartilage into bone as a normal part of skeletal maturation - but a mutation can incorrectly activate this function and ossify soft tissue. The only upside is that this condition is incredibly rare - only one in two million people across the world are thought to be affected.
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