Experts interviewed: Chen Haibo, Chief Expert of the Department of Neurology, Beijing Hospital
Global Times Health Client Reporter Niu Yulei
htmlAt noon on July 19, the agency issued a document confirming that the famous actor Zheng Peipei passed away in San Francisco, USA on the 17th at the age of 78. In 2019, she was diagnosed with neurodegenerative atypical parkinsonian syndrome - corticobasal ganglia degeneration . It is a rare disease with symptoms similar to Parkinson's disease, but current treatments do not slow its progression. It is reported that Zheng Peipei chose to donate her brain for medical research after her death.
Chen Haibo, chief expert of the Department of Neurology at Beijing Hospital, told the "Global Times Health Client" reporter that corticobasal degeneration is a chronic progressive neurodegenerative disease. Its symptoms are highly similar to Parkinson's syndrome, but it has its own particularities. Therefore, it is called "atypical parkinsonism". Parkinson's disease mainly affects the striatum, which is located in the basal ganglia, and generally does not affect the cerebral cortex. Corticobasal ganglia degeneration superimposes cortical layer lesions, so it and Parkinson's disease can be regarded as two diseases.
Chen Haibo said: "In terms of the pathogenesis of the disease, it is related to tau protein (tubulin) lesions, which lead to brain cell damage and death after deposition in the cortex and basal ganglia." The initial stage often shows the parietal cortex on one side of the brain. shrink. However, it is still unclear why tau protein accumulates abnormally in the brain and causes this disease.
After suffering from the disease, patients will show typical Parkinson's symptoms such as slow movement of limbs and inability to maintain balance, as well as typical symptoms of damage to higher-level brain functions. Specifically, human brain functions are divided into primary and advanced. Primary functions include movement, vocalization, hearing, touch, etc. Advanced functions are cognitive and application functions, that is, the ability to integrate information perceived through primary functions. For example, after seeing an apple, you can know whether it is an apple by comprehensively judging its shape, texture, color, touch, and taste. An apple can organize various sounds emitted by the human body into language to express one's thoughts and opinions. It knows that when cooking, you must wash and cut vegetables before putting them in the pot. Corticobasal ganglia degeneration can lead to the loss of high-level cortical perception. For example, if a metal key is placed in the patient's hand without looking, the patient will feel that the object is cool, hard, long and toothy, etc. However, the information cannot be combined to determine whether it is a key, or the patient can speak words but cannot organize them into sentences, or when cooking, the vegetables with soil and rope will be put directly into the pot. The patient will also have a strange phenomenon called alienation. His hands, he felt that they were not his own and would not obey his commands. They would even wave back and forth uncontrollably.
Chen Haibo said that corticobasal degeneration is irreversible once it occurs. As the cell damage in the patient's brain gradually worsens, the body will become paralyzed or even bedridden. Over time, the body's resistance will decline, making it easy for repeated infections, eventually leading to death. There are many drug and non-drug treatments for Parkinson's disease that can improve symptoms, especially for early-stage patients. However, there is basically no effective treatment for corticobasal degeneration, and all clinical treatments for it are supportive. For example, after being unable to walk, patients can use a wheelchair or be taken care of by others, or simple rehabilitation training can be carried out to allow patients to extend the time they can move as much as possible, reduce the chance of infection, and extend life expectancy. Currently, there are no medications or surgical treatments that can improve symptoms and slow disease progression. Chen Haibo said bluntly: "It's more difficult to treat than ALS."
Chen Haibo said that once abnormal symptoms such as inflexible movements and language difficulties occur, you must seek medical treatment in time. "However, you don't need to worry too much. Corticobasal degeneration is a kind of The incidence rate of rare diseases is only 0.6~0.9 per 100,000 people.” ▲
Editor-in-chief: Pan Zihu
Editor-in-chief: Zhang Mian
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