Introduction of PSP and CBS
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PSP
Progressive Supranuclear Palsy
PSP
Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disease, classified symptoms as an atypical Parkinsonian syndrome. The onset age of typically begins between the ages of 60 and 70. Unlike Parkinson’s disease, where the pathogenic protein is α-synuclein accumulation, PSP involves the deposition of four-repeat tau protein within neurons and gial cells in the brain.
Prevalence studies have shown that for every 100 patients with parkinsonian features, 2 to 5 will have PSP.
Compared to typical Parkinson’s disease, PSP progresses more quickly. In the early stages, patients often fall due to poor balance and reflexes. Eye movement is restricted, especially when looking up or down, and other symptoms resemble those of Parkinson’s disease, including limb rigidity, coordination problems, speech disorders, and swallowing difficulties. PSP patients generally show a poorer response to Levodopa compared to people with typical Parkinson’s disease, and may experience early personality changes such as becoming withdrawn, less talkative, or conversely, emotionally unstable, with symptoms like depression, anxiety, and cognitive impairment.
The diagnosis of PSP involves clinical interviews, medical history, and neurological exams, with a particular focus on eye movement problems. Brain imaging techniques, including positron emission tomography (PET) scans for Tau, and abnormal Tau aggregation changes in cerebrospinal fluid, have become novel diagnostic tools in recent years. Currently, treatment remains limited to dopamine supplementation and regular exercise, which are essential. Clinical trials targeting the underlying mechanisms of PSP are currently ongoing.
CBS
Corticobasal Syndrome
Corticobasal Syndrome (CBS), like PSP, is a rare neurodegenerative disease and is classified as an atypical Parkinsonian syndrome. The onset age of symptoms typically begins between the ages of 60 and 70.
The disease is usually associated with degeneration of the connections between the basal ganglia (the brain region responsible for motor control) and the cerebral cortex (the brain region responsible for sensation, cognition, and higher functions). It consists of a range of symptoms related to various neurological issues, often affecting both motor and cognitive functions. Like PSP, the brain pathology in CBS is caused by the accumulation of four-repeat tau protein in nerve cells.
CBS
The symptoms often resemble those of typical Parkinson’s disease in the early stages, making it easy to confuse the two. CBS symptoms are usually more severe on one side of the body than the other. Common symptoms include: stiffness and slowness of movement in the neck, arms, and legs, unstable gait, muscle jerks, difficulty performing common arm or leg movements, loss of sensation or difficulty recognizing objects by touch on one side, the sensation that one’s hand doesn’t belong to them, and difficulties with speech and language, such as lack of motivation, personality changes, or irritability.
The cause of CBS is typically linked to the accumulation of abnormal proteins (such as Tau) in the brain, which damages the brain’s neurons, leading to functional impairments.
The diagnosis of CBS involves clinical interviews, medical history review, and neurological examinations to determine if CBS is present, with special attention given to whether one side of the body exhibits cortical symptoms such as muscle jerks, apraxia, or alien hand syndrome. Additionally, brain imaging scans can reveal cortical atrophy, and positron emission tomography (PET) scans for Tau, as well as abnormal Tau aggregation changes in cerebrospinal fluid, have emerged as novel diagnostic tools in recent years.
Currently, treatment options are limited. Dopamine supplementation and regular exercise are essential for symptom management. Maintaining an exercise routine, physical therapy, and speech therapy can help improve quality of life. Clinical trials targeting Tau protein mechanisms are currently ongoing.
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