Some diseases are so devastating that survival is almost impossible – with mortality rates reaching 100 percent.
Fortunately, because of modern medicine, they are incredibly rare.
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The few cases that do exist are mostly hereditary, such as brain wasting disease that is passed down through families.
However, others have more bizarre origins, one even stemming from cannibalistic practices.
And yes, here in the UK, a few of these diseases have left their mark.
So, let's take a closer look at these rare but devastating diseases – and specifically, why they have earned their reputation as some of the deadliest diseases known to humanity.
Creutzfeldt-Jakob disease (CJD)
The US Center for Disease Control (CDC) describes Creutzfeldt-Jakob disease (CJD) as “invariably fatal.”
It causes proteins in the brain – called prions – to fold abnormally, which is why it's called prion disease.
Folds It eventually leads to brain damage and death.
In most cases, it develops intermittently.
But the fatal condition can also be inherited if a parent carries a mutation that causes prions to form in their brain during adulthood.
The hereditary form of the disease affects only one in nine million people in the UK, according to the NHS.
In the United States, about one in every million people are diagnosed each year.
Most people die within a year of the first symptoms, which include memory loss, impaired thinking, personality changes, and sudden, jerky movements.
The Mayo Clinic likens it to Alzheimer's disease, but at a rapid rate, with additional symptoms such as blindness, speech problems, and severe coordination problems.
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Fatal familial insomnia (FFI)
Imagine a genetic condition that not only steals your sleep, but ultimately your life – that's fatal familial insomnia (FFI) in a nutshell.
This rare disease, which also belongs to the Peyrones family, affects the brain and central nervous system.
It causes sleepless nights, memory loss, and worsening muscle twitching, according to the National Organization for Rare Disorders (NORD).
It is a degenerative condition which means it gets progressively worse, and there is no cure.
Treatment focuses on relieving symptoms and keeping patients comfortable.
Globally, this disease affects between 50 and 70 families worldwide.
But only one or two people out of a million ultimately test positive.
In the United Kingdom and the United States, cases are extremely rare, with only a few reported.
What does rabies do to the body and how is it treated
Rabies is a deadly virus that is usually spread through a bite or scratch from an infected animal.
The infection is common worldwide but is mostly found in Asia, Africa, and Central and South America.
Some animals that are most likely to spread the disease are dogs, bats, foxes, skunks, and raccoons.
symptoms
After exposure to a bite or other type of rabies, it can take weeks or months for the virus to start showing symptoms.
This time frame is what doctors call the incubation period during which the virus travels through the body to the brain.
Some of the most common symptoms of rabies include:
- fever
- headache
- Excess saliva
- Muscle spasms
- paralysis
- Mental confusion
The World Health Organization describes two main manifestations of the disease: rabies rabies and paralytic rabies.
People or animals with angry rabies may appear agitated, become aggressive, and drool excessively, while other symptoms include hyperactivity, fear of water, and even fear of fresh air.
On the other hand, the symptoms of paralytic rabies are underestimated, usually causing gradual paralysis as the patient remains calm and lucid.
treatment
If you are bitten or scratched by an animal in an area at risk for rabies, you should immediately clean the wound with soap and running water for several minutes.
Next, you should disinfect the wound with an alcohol- or iodine-based antiseptic and apply a simple bandage.
After treating the wound, you should see a doctor as soon as possible and he or she will determine whether or not you need a rabies vaccination.
Post-exposure treatment is almost 100% effective if started before any rabies symptoms appear.
Some cases require immune globulin, which is given in and around the wound.
This provides short-term protection if there is a high possibility of the wound becoming infected.
Treatment should ideally begin within a few hours of being bitten, but can be delayed for up to 24 hours if necessary.
rabies
If you think rabies is just a problem for your pet, think again.
This is one of the deadliest diseases known to humanity, with fewer than 20 people reported alive worldwide, according to the CDC.
The National Health Service in Britain says that it begins mildly and deceptively, with flu-like symptoms and a strange itching sensation near the site of the bite.
But when it progresses, it causes anxiety, hallucinations, fear of water (hydrophobia) and complete brain dysfunction.
Without immediate treatment after exposure, rabies is almost always fatal.
In the United Kingdom, rabies is extremely rare, largely due to strict animal control and vaccination programmes.
The last recorded case of classic rabies in the United Kingdom was in 1902.
However, there have been isolated cases linked to animal bites abroad.
For example, in 2012, a UK resident died of rabies after being bitten by a dog in South Asia.
The United States sees an average of three cases each year.
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Kuro
Kuro, a fatal brain disorder, has one of the strangest histories.
It was widespread in the 1950s and 1960s among the people of New Guinea due to ritual cannibalism.
They would eat the brains of sick individuals who were carrying the kuru.
Like CJD and FFI, kuru is caused by an infectious protein, or prion, according to Healthline.
The name Kuru means “trembling” or “trembling with fear”, with tremors being one of the first symptoms.
People continue to develop slurred speech, followed by mood changes and an inability to eat or stand.
Death usually occurs within six months to a year.
Before their death, victims would often explode into fits of laughter, which is why it is sometimes called the “laughing disease.”
Fortunately, as cannibalism declined, so did the kuru – and it is now on the verge of extinction.
No cases of kuru have been reported in the United Kingdom or the United States, as the disease is restricted to specific cultural practices in New Guinea.
Gerstmann-Strausler-Schenker syndrome (GSS)
Another brain-folding disease, Gerstmann-Straussler-Schenker syndrome (GSS), is so rare that it has only been recognized in a few families worldwide.
Most people develop symptoms between the ages of 35 and 55, starting with coordination problems and progressing to dementia, difficulty speaking, and even blindness.
Symptoms can develop slowly for two to 10 years before leading to severe disability and death.
There are no treatments to stop its progression – instead, treatments focus on managing symptoms.
In the UK, GSS is extremely rare, with only a few recorded cases.
The National Creutzfeldt-Jakob Disease Research and Surveillance Unit monitors such cases as part of its prion disease surveillance.
In the United States, GSS is also rare, with fewer than 50 documented cases.
