Fatal Familial Insomnia, When It Is Difficult to Sleep inherited from Parents to Children and Risk of Death

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You might think that the causes of insomnia that you have experienced are rooted in work stress or because you drink a lot of coffee in the afternoon. But if you are still difficult to fall asleep even though you have fixed your lifestyle and sleep patterns, you may have offspring of insomnia. In the medical world, the condition of decreased insomnia is called fatal familial insomnia. This type of sleep disorder is very risky and can cause death. Wow!

Fatal familial insomnia, causes of insomnia decrease in the family

A person is said to be insomnia when he finds it difficult to start sleeping at night and / or continue to sleep all night. These sleep disorders can come and go, also vary in how long an episode takes place and how often the problem occurs. Acute insomnia can last from one night to several weeks, while chronic insomnia recurs at least three nights in one week and runs for a month or more.

Insomnia causes you not to get the quality sleep you need to wake up in the morning feeling refreshed and energetic.

As the name implies, fatal familial insomnia (FFI) is a disorder of genetic sleep nights which decreases from generation to generation in the family tree.

What caused it?

Fatal familial insomnia (FFI) belongs to the category of prions that attack nerve cells in the brain. Some other diseases including the prion disease group are Kuru disease and aka Crazy CowCreutzfeldt-Jakob disease.

FFI is considered one of the rarest prion diseases. Only 40 families are diagnosed with this disease in the world.Usually, FFI is derived from previous family members who have a history of prion disease. If one or both parents have an FFI trigger gene mutation, the child has the chance to inherit the same condition by 50 percent.

As far as scientists can observe, the cause of insomnia decreases in families is most likely a mutation of the PRNP gene (prion protein) that causes damage to the thalamus in the brain. The thalamus is responsible for all kinds of things, including sleep patterns, body temperature, and emotional expressions. Damage to the thalamus due to prion infection occurs gradually, causing the thalamus to lose nerve cells.

Why can it cause death?

Reporting from Health Line, FFI can cause deaths from one to two years after experiencing symptoms. However, the time line for each person can differ depending on the condition of the body and the severity of the symptoms experienced.

As explained above, damage to the thalamus will interfere with the workings of the brain. That is why the symptoms of prion disease can be very diverse, ranging from heavy sweating, tremors, difficulty speaking, constipation, to impotence and dementia. However, the main symptom is lack of sleep, aka insomnia.

People with FFI cannot enter the stage of deep sleep called slow wave sleep, which helps restore your brain by rinsing toxins and cerebral-spinal fluid. The buildup of toxic fluids eventually causes further brain damage.

FFI sufferers are always trapped in a condition where they do not fully sleep or wake up. For people with FFI, no matter how hard you try to get a good night's sleep, that won't happen. Chronic sleep deprivation will progress to worsen into extreme fatigue, dementia and, finally, death.

What are the symptoms of FFI disease?

This disease tends to occur in people aged 32-62 years, very rare in people who are younger or older than that age range.

The fatal familial symptoms of insomnia in each person are different and are divided into two, namely the initial symptoms and advanced symptoms.

The initial symptoms of fatal familial insomnia include:

  • It's hard to sleep.
  • It's hard to fall asleep all night, always awake.
  • Frequent twitching or muscle spasms.
  • Often experience stiff muscles.
  • Often moving restless while sleeping, such as kicking or hitting (unable to sleep calmly).
  • Loss of appetite.
  • Often forget or have difficulty remembering things (symptoms of dementia).

Meanwhile, fatal familial insomnia symptoms include:

  • Unable to sleep.
  • Cognitive and mental functions deteriorate.
  • Loss of coordination or ataxia (unable to control body movements).
  • Excessive sweating.
  • Blood pressure and heart rate increase.
  • Weight loss due to reduced appetite.
  • Difficulty talking, even difficult to swallow.
  • Fever.

How is this disease diagnosed?

FFI is a rare hereditary disease. If no one in your family has that history, then you should not need to worry.

But if you experience the above symptoms, especially prolonged sleep difficulties, you should immediately see a doctor. After asking for a personal and family health history and conducting a basic physical examination, the doctor will recommend you to undergo a PET scan to scan your brain's functions.

Advanced genetic tests can also help doctors determine the diagnosis, because there are many diseases that can cause insomnia for a long time, such as sleep apnea.

How is this disease treated?

There is no cure for this disease. However, effective treatment can help manage and reduce symptoms. For example, by prescribing sedatives or sleeping pills for a while to sleep better.

In addition, prescription of doxycycline antibiotics can be done as a preventive measure to avoid FFI occurring in people who are at high risk of prion infection or who have family members with a PRNP gene mutation.

The most important thing is to always adopt a healthy lifestyle, such as regular exercise, eating healthy foods, managing stress well, and getting enough sleep as much as possible. Do not forget also to always be routine health officers to be able to detect the risk of disease and treat it before it gets worse.

Fatal Familial Insomnia, When It Is Difficult to Sleep inherited from Parents to Children and Risk of Death
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