Written by Seven Kings
If you gradually lose happiness, can you afford to be less interested in anything, your sense of direction and reasoning are getting worse and worse, and you end up with an intelligence equivalent to that of a three-year-old?
Believe that everyone's answer is no. But such a terrible thing happened to a world-famous comedian whose profession is to bring happiness to everyone. To make matters worse, the severity of his condition exceeded the experience of all consulting doctors.
The actor is Robin Williams. You may have seen his representative works, such as "Death Poetry Society", "Mindcatcher", "Game of the Brave". Williams has won many heavyweight awards such as the Academy Award and the Golden Globe Award.
Robin Williams (right) starred in the film Mindcatcher, about the MIT "Sweeper," for which Williams won an Academy Award for Best Supporting Actor.
In 2014, Robin Williams committed suicide. After his death, former U.S. President Barack Obama issued a statement stating that "Robin Williams is unique and has touched countless hearts throughout his life." Many thought he was suffering from depression, but it was years after his death that his cause surfaced through an autopsy.
In 2016, his widow, Susan Schneider Williams, revealed his true cause of death in the academic journal Neurology.
In August 2014, Apple commemorated Williams on its homepage.
It turned out that Williams was suffering from a neurological disorder that made people no longer happy and the second largest dementia in the world: Dementia with Lewy bodies.
Many people know that Alzheimer's disease is a dementia that often plagues the elderly, but the second largest dementia, Lewy's dementia, is little known. Ian McKeith, a professor of psychiatry at the University of Newcastle in the United Kingdom, said that Louisian dementia is the second most common dementia in the elderly, second only to Alzheimer's disease, accounting for 10-15% of all dementias. In the United States alone, there are 1.5 million people with Lewy's dementia (about 300 million in the U.S. population).
Image credit: pixabay
Like Alzheimer's, Lewy's dementia is quite fatal. But unlike the ignorance of People with Erzheimer's disease, the latter is well aware that his brain is gradually headed for destruction.
Lewys was discovered in 1912. Discoverer Frederic Lewy, a German-American neuroscientist, accidentally found strange clumps of proteins called Lewy's while studying the brains of Parkinson's patients, which are made up of proteins α-α synucleins.
Lewys in neurons in patients with Lewy's dementia. Image source: wikipedia
α-synuclein is involved in the repair of cellular DNA and is originally a positive role. But because of the mechanisms that have not yet been clearly understood, in the brains of people with Lewysian dementia, they can gather abnormally in neurons. When the Lewyites occupy too much space in neurons, the neurons cannot perform their original work, and the patient's brain begins to slide into the abyss of irreversibility.
Early Lewy's dementia often does not have any specific symptoms, and The Mini Mental Status Exam, a common tool used to screen for Alzheimer's, is also ineffective against early-stage Lewis dementia. The initial manifestations may simply be that attention is not easy to concentrate and it is easy to wake up at night.
Image credit: pix4free
The widow recalls that Williams' problems began with inexplicable insomnia in 2013.
But gradually, patients lose interest in anything, because a big target of the Lewys body "attack" is the dopaminergic neurons in the brain.
Dopaminergic neurons release dopamine and use dopamine as a neurotransmitter. Dopamine is known to be closely linked to the brain's mood and reward systems. Addiction, such as swiping mobile phone addiction, playing games addictive is the role of dopamine.
Image source: pxfuel
Of course, as a neurotransmitter that makes people feel stimulated and happy, without dopamine, the brain feels bored, meaningless and unhappy, which is the principle of Lewy's dementia depriving happiness.
Later autopsy reports showed that Williams' brain lost 40 percent of its dopaminergic neurons. In every sense, Williams' condition is also rare among people with Lewy's dementia.
In an interview with ABC, widow Susan said Williams did have depression, but depression was only one of more than forty clinical symptoms of Lewy's dementia, and it was relatively insignificant of them. This is because people's advanced cognitive abilities and motor systems are also closely related to dopamine.
The gradual loss of dopaminergic neurons can trigger many neurological disorders, such as Parkinson's disease, and the patient's spatial perception and visual abilities will also decline.
Williams, for example, often couldn't figure out where the doors were. At one point, Susan noticed that his whole body was stiff, his head was slammed against the doorpost, and the blood was flowing. His sense of smell also began to become insensitive, and his arms began to tremble slightly. His insomnia and forgetfulness also became more frequent.
At the end of the term, dementia is visible to the naked eye, because at this point Louis's body is all over the brain. At this stage, the typical symptoms of Lewy's dementia are similar to those of Alzheimer's, and dementia is inevitable: memory decline, decreased reasoning and judgment, decreased attention and alertness.
Doctors later told Susan that some high-IQ patients with Lewy's dementia had a slow initial course of illness, but at some point it would be like a flood breaking its banks. This happened to Williams.
In early 2014, Williams had a nervous breakdown while filming Museum Night 3, because he sometimes couldn't remember a single line while shooting the film. And when he performed "The Bengal Tigers at baghdad Zoo" on Broadway just three years ago, he could say hundreds of lines a day without error.
Williams played Theodore Roosevelt in the Museum Of Fantastic Nights series.
The rapid deterioration of memory was undoubtedly a major blow to Williams, and he knew he was losing his mind, which caused double the pain. My widow recalls that during that time he always said, "I just want to restart my brain."
However, Williams did not know until his death what disease he had. He did various tests, the doctor examined his pituitary gland and heart, but found no problems. It seems that there is nothing wrong with his body except for the excessively high stress hormone, cortisol. The family thought Williams was overly worried. But in fact, only he knew that the storm was coming.
Image source: wikimedia
In May, he was diagnosed with Parkinson's disease. But Williams did not believe he had the disease. Finally, in August 2014, he chose to end the battle with his own hands with his only remaining will.
Autopsies revealed that almost all of Williams' brain had been invaded by Lewis. Susan wrote in the article: "[Williams] there are no Lewy-free neurons in the whole brain and brainstem. According to the autopsy report, Williams died of Louisian dementia.
All four doctors the widow consulted believed he was the worst Lewyian dementia they had ever seen.
One of the doctors said: "It's as if every organ of his body is suffering from cancer." Another doctor said that when Williams died, the cognitive power of the brain was equivalent to that of a three-year-old child. The doctors were also amazed that Williams had been able to hold on for so long, and they thought it was unlikely that he would be able to walk in his neuronal state.
The only thing that could give Susan a sense of relief was that the autopsy report had given an explanation for all that had gone wrong with Williams over the years. Susan pointed out that There are more than forty symptoms of Lewy's dementia, and the only thing Williams does not show is hallucinations. Of course, this may also be his elaborate cover-up. But with such a serious condition, why was no one able to make an accurate diagnosis in the first place?
In fact, the misdiagnosis rate of Lewyian dementia is very high.
According to the Lewy Body Dementia Association, because the symptoms of Lewy dementia and Parkinson's are so similar, "misdiagnosis is fairly common."
Typical symptoms of Parkinson's disease. Image source: See watermark
The Lewy Body Dementia Association further reluctantly stated that due to similar symptoms, the diagnosis of Lewys dementia and Parkinson's disease reflects "arbitrariness", in general, if the patient has a body tremor before the appearance of dementia symptoms, then it will be diagnosed with Parkinson's disease, and vice versa, Lewy's dementia.
The Alzheimer's Society points out that Lewy's dementia accounts for only 4% of all dementias in the UK, but due to the prevalence of misdiagnosis, the actual value can reach 10%-15%.
McKeith, the founder of the Consortium on Dementia with Lewy Bodies, who developed guidelines for diagnosing Lewy dementia, explains that one of the reasons Lewy dementia is difficult to diagnose is that Lewys in the cerebral cortex is difficult to detect.
Just 20 years ago, even brain scanning technology could not distinguish between Lewy's dementia and Parkinson's disease, and only autopsies could accurately judge pathology, but this was also after the 90s, because it was then that immunohistochemical staining that made The Lewys invisible appeared.
Lewys in the neurological substantia nigra neurons (as pointed by the black arrow). Image source: wikipedia
For these reasons, it was not until 1995 that the first international clinical and pathological diagnostic criteria for Lewy's dementia were available. Brain scanning techniques that distinguish between Alzheimer's and Lewyian dementia, such as the brain dopamine transporter SPECT imaging technique, have also emerged, making it possible for patients to make a diagnosis of the disease before death.
But even if Williams had been diagnosed, doctors would have been unable to do anything because Louisian dementia had no cure 7 years ago. According to the Lewy Body Dementia Association, only Japan and the Philippines in the world have now approved a drug that promises to treat Lewy dementia: Teniperazine (Aricept). Previously, donepezil was generally used to treat Alzheimer's disease.
After experiencing the purgatory that Lewy's body dementia brought to her loved ones, Susan did not want others to experience her pain. In the process of accompanying her husband on various hospitals and research institutes for many years, she also became a half-expert. She later learned about the existence of the American Brain Foundation and became a member of its board of directors, dedicated to making the silent disease known and studied by more people.
Image source: Mindcatcher
Speaking about depression, Williams once said: "The purpose of grief is to make you a different person, so people call sadness the gift of the Buddha." Grief makes you realize that what matters in the world is others, not yourself. It seems that the empathetic Susan has put the idea of a dead husband into practice.
He could make everyone laugh, except himself.