Because of this disease, the rest of his life became a losing battle

At an emergency center in Japan, a 39-year-old male patient was rushed to the hospital. He had just suffered a collapse accident and was unconscious. He was suspected of having a brain herniation and soon developed ventricular fibrillation, and his condition was very critical.

The man just encountered a collapse accident and was unconscious | Video screenshot

After emergency treatment, the patient's vital signs finally stabilized. However, the doctor was shocked by what his wife said: her husband was diagnosed with olivopontocerebellar atrophy (OPCA) five years ago.

It seems that the matter is far more than just an accident.

OPCA - a disease that causes life to wither quickly

The above story comes from the Japanese medical drama "Please Don't Chant in the Ward" in the winter of 2020. It was originally a light-hearted TV series in which the male protagonist, who has the dual professions of "monk + doctor", uses medical technology and "mouth cannon" to save patients, but in one episode, it tells such a slightly heavy story.

The patient's wife told the doctor that he had given up on life and was alienated from all the people and things he cherished in the past. It was hard to imagine that just five years ago, he was still a zoologist full of enthusiasm for his career.

Five years ago, he was still a zoologist who was passionate about his career | Video screenshot

What kind of disease is OPCA that can cause a young and healthy life to quickly wither away in just five years?

You may not have heard of OPCA, but the name of the disease gives a very straightforward definition: atrophy (A) of the inferior olive (O), pons (P), and cerebellum (C). These three areas are located in the central nervous system and are involved in maintaining body balance and coordinating voluntary movements. Once they atrophy, people will show a variety of movement disorders.

Imagine that you frequently fall down because you cannot maintain your balance, and you may even have to spend the rest of your life in a wheelchair. Because your throat muscles are out of control, it becomes difficult to speak, and the sounds you make with effort are just a bunch of meaningless mumblings to others. Even swallowing food becomes a difficult task. Since you cannot eat smoothly, you may experience various symptoms of malnutrition, and you may be infected with aspiration pneumonia at any time due to accidentally inhaling food into your trachea, which is life-threatening. This is the daily life of OPCA patients.

The time from onset to death is very short

OPCA is a rare disease with 3 to 5 cases per 100,000 people, but about 5% to 6% of patients with atypical Parkinson's disease may experience OPCA symptoms. The cause of OPCA can be genetic, usually caused by abnormal duplication of gene fragments. The age of onset of hereditary OPCA is relatively early, with an average of 28 years old. After that, the patient can survive for about 15 years.

The cause of non-hereditary (or "sporadic") OPCA is more complicated. Currently, there is only evidence that it may be related to the abnormal aggregation of a large molecule called alpha-synuclein in nerve cells. We don't even know what the function of this protein is. Non-hereditary OPCA develops later, with an average age of 53, and patients usually deteriorate rapidly within five or six years and eventually die.

Patients' conditions usually deteriorate rapidly within five or six years, eventually leading to death | Video screenshot

Back to the series, the doctor in charge sighed: "He doesn't have much time left." We don't know whether the man has hereditary or sporadic OPCA. But what is certain is that no matter which one it is, his life will be difficult.

The rest of the patient's life is a losing battle

Like Parkinson's and Alzheimer's, OPCA is classified as a "neurodegenerative disease." Neurons in certain areas of the brain are damaged or die, and as the disease progresses, the functions of the damaged brain areas gradually degenerate. The rest of an OPCA patient's life is a losing battle against their own degenerating nervous system and motor abilities.

Under such circumstances, the patients in the TV series experienced a life-threatening rescue, but they did not feel relieved at all. The first thing they did after waking up was to ask the doctor, "Why did you save me?" When they know that they have little time left to live, how many people have the courage to continue?

The patient has no sense of relief at all for surviving the disaster | Video screenshot

Since the causes and treatments of many neurodegenerative diseases are still unclear, coupled with the complexity of OPCA, the quality of life of patients can only be improved through auxiliary means. For example, accompanying care is needed to prevent patients from falling, and physical therapy is used to improve movement and language skills. After the symptoms of dysphagia worsen, many patients also need to undergo surgery to implant intestinal feeding tubes to ensure nutritional intake. Behind each of these treatment plans, family members and caregivers need to devote a huge amount of time and energy.

Even so, choking and aspiration pneumonia caused by dysphagia are still common causes of death for OPCA patients. Because of this, we may understand his intentional alienation from his wife and children. This battle will not only affect him, but may also involve his family.

After the onset of the disease, he deliberately alienated his wife and children | Video screenshot

However, Japanese dramas are Japanese dramas after all, and they are always more idealistic than reality. As a zoologist, he likes white rhinos - unlike most rhinos that fight alone, white rhinos are social creatures. After the male protagonist turned on the "mouth cannon" mode and used the white rhinoceros as an example to enlighten him, the patient realized that since death is a foregone conclusion, why should the family feel the pain of separation in advance? People live in society and always have to support each other. At the end of the series, he finally hugged his daughter in tears and decided to look forward to every tomorrow with his family.

But in the real world outside the screen, how many OPCA patients are still fighting alone?

References:

[1] Berciano J, Boesch S, Pérez-Ramos JM, and Wenning GK. Olivopontocerebellar atrophy: Toward a better nosological definition. Mov Disord 21: 1607-1613, 2006.

Author: Alulull

Editor: Muyi Yangyang

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