Life expectancy is
9 months to 29 years
, according to “Radiologic manifestations of Proteus syndrome” published in the Radiological Society of North America journal RadioGraphics.
Is Proteus syndrome fatal?
The clot can break free and travel throughout the body. If a clot becomes wedged in an artery of the lungs, called a pulmonary embolism, it can block blood flow and lead to death.
Pulmonary embolism is a leading cause of death in people
with Proteus syndrome.
Can people with Proteus syndrome have kids?
Adults with
Proteus syndrome have never had any affected children
.
Does Proteus syndrome affect the brain?
Less common findings in Proteus syndrome include malformations of the central nervous system such as
overgrowth of half of the brain
(hemimegalencephaly). In some patients, intellectual disability may be present and seizures have been reported as well.
Could the Elephant Man be cured today?
There is no cure for neurofibromatosis
, also known as the Elephant Man's Disease, so named after John Merrick, a victim who lived in the 19th century and was known as the Elephant Man.
Can Proteus syndrome be cured?
Most people with Proteus syndrome have a variant seen in the AKT1 gene in some, but not all cells of the body.
There is no cure or specific treatment for Proteus syndrome
and treatment involves medical and surgical management of symptoms.
Who is most likely to get Proteus syndrome?
Classically,
males have
been thought to be more commonly affected than females, but new studies with genetically confirmed cases have not yet been published. The genetic mutation that causes Proteus Syndrome is a somatic mutation that occurs after conception and is propagated in one or more subsets of embryonic cells.
Is Proteus Syndrome a disability?
Some people with Proteus syndrome have
neurological abnormalities
, including intellectual disability , seizures , and vision loss, as well as distinctive facial features. Proteus syndrome is caused by a change ( mutation ) in the AKT1 gene .
How does Proteus Syndrome change the body?
Proteus syndrome is a rare condition characterized by
overgrowth of the bones, skin, and other tissues
. Organs and tissues affected by the disease grow out of proportion to the rest of the body. The overgrowth is usually asymmetric, which means it affects the right and left sides of the body differently.
What are the symptoms of Proteus?
- asymmetric overgrowths, such as one side of the body having longer limbs than the other.
- raised, rough skin lesions that may have a bumpy, grooved appearance.
- a curved spine, also called scoliosis.
- fatty overgrowths, often on the stomach, arms, and legs.
Did Michael Jackson Buy Elephant Man's bones?
In 1987, pop star
Michael Jackson makes a bid to buy the bones
of Joseph Merrick, popularly known as the “Elephant Man”. In exchange for the remains, Jackson offers the London Hospital Medical College $500,000.
Is Elephant Man disease hereditary?
” Neurofibromatosis affects all races and sexes equally; a child of someone with the disease runs a 50-50 risk of developing it. Still,
half of all cases are not inherited
, but are caused by genetic mutations at conception, the researchers said.
Does Proteus syndrome hurt?
One of the features of Proteus syndrome the researchers looked at was the cerebriform connective tissue nevus (CCTN), which is a disfiguring, massive overgrowth of the skin, typically on the soles of the feet. These lesions
can cause patients pain
and make it difficult to walk and find shoes that fit.
What is the history behind Proteus syndrome?
The syndrome is
named after the Greek sea-god Proteus
, who could change his shape. The condition appears to have been first described in the American medical literature by Samia Temtamy and John Rogers in 1976. Michael Cohen described it in 1979.
