Corticobasal degeneration is a rare neurodegenerative disease with no known cause, affecting the cerebral cortex and basal ganglia of the brain. Diagnosis is difficult and can only be done postmortem. Symptoms include muscle contractions, impaired movement, and loss of speech. Diagnosis can be done through histological examination of brain tissue. There is no cure and treatment focuses on symptom relief.
Corticobasal degeneration is a neurodegenerative disease affecting the cerebral cortex and basal ganglia of the brain. It’s rare, affecting five to seven people in 100,000, and usually doesn’t show up until age 60. Diagnosis is difficult, as corticobasal degeneration has similar symptoms to other neurodegenerative conditions and can only be definitively diagnosed postmortem. The causes of the disease are not known at the moment.
Corticobasal degeneration is a slow, progressive disease, meaning that the brain gradually degenerates, causing symptoms to worsen over time. Symptoms include uncontrolled rhythmic muscle contractions, decreased movement, muscle stiffness, impaired balance, alien hand syndrome or the inability to sense and control hand movements, apraxia or the inability to control movement in limbs, and aphasia or loss of speech. Psychological and cognitive symptoms including irritability, dementia and depression are also common. This cluster of symptoms is sometimes referred to as corticobasal syndrome (CBS) or corticobasal degeneration syndrome (CBDS), because definitive diagnosis of corticobasal degeneration is not possible while the patient is alive.
Imaging techniques including fluorodopa positron emission tomography (FDOPA PET), magnetic resonance imaging (MRI), and single photon emission computed tomography (SPECT) are sometimes used on a patient with CBS, although they are usually inconclusive . However, such techniques may allow for the diagnosis of corticobasal degeneration in the future. FDOPA PET is used to diagnose impaired dopamine uptake in affected areas of the brain. MRI can identify atrophy, or wasting, of structures in the brain, and SPECT looks at perfusion, or blood supply in the brain, which is usually reduced in patients with CBS.
Corticobasal degeneration is often misdiagnosed as progressive supranuclear palsy, Parkinson disease, and various forms of dementia, including Alzheimer disease. Frontotemporal dementia, a degenerative disease of the frontal and temporal lobes of the brain, can progress to CBS. While the symptoms of CBS don’t align exactly with any of these other conditions, allowing some of them to be ruled out as possible causes in some patients, not all possible symptoms are always present, and it is often impossible to completely rule out other diseases. There is no cure for CBS and the prognosis is very poor, with most patients dying within eight years. Treatment focuses on relieving symptoms and may include dopaminergic medications as well as speech therapy to address aphasia and methods to help the patient move and eat.
Corticobasal degeneration can be diagnosed by histological or microscopic examination of brain tissue. Unnaturally high levels of tau protein, along with astroglial inclusions or abnormal growths of astrocytes, the brain’s support cells, are indicative of the condition. The most commonly used method to detect these marks is the Gallyas-Braak stain method.
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