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Why is aphasia not just a language problem? What mysterious brain changes are hidden behind PPA?
Among the neuropathies, primary progressive aphasia (PPA) is a neurological syndrome in which a patient's speech ability is gradually impaired. Although similar to other types of aphasia, the symptoms of PPA are primarily determined by the damaged part of the left hemisphere of the brain, unlike most other aphasics, the symptoms of PPA gradually worsen as brain tissue continues to degenerate. Therefore, early symptoms usually have less impact than later symptoms.
Those with PPA gradually lose the ability to speak, write, read and understand language, and eventually almost all sufferers become mute and completely lose the ability to understand written and spoken language.
Primary progressive aphasia was first described by M. Marsel Mesulam in 1982, and it was gradually recognized that it was more than just a disorder of language ability. Clinically, it overlaps with and is similar to pathological features of prefrontal dementia and Alzheimer's disease. However, compared with patients with Alzheimer's disease, patients with PPA are usually able to maintain their ability to live independently.
Cause
Currently, the exact cause of PPA and other similar degenerative brain diseases remains unknown. Although pathological examination shows abnormalities in the brains of PPA patients, existing imaging techniques (such as CT scans, MRI, etc.) basically only show abnormalities in the left hemisphere.
Although epidemiological studies on PPA variants are lacking, observational retrospective studies have found that the age of onset of PPA is usually between 60 or 70 years of age.
Diagnostic criteria
The diagnostic criteria for PPA, based on Mesulam's definition, require patients to show progressive speech impairment that affects functional activities of daily living. Diagnosis requires establishing that the speech disorder is caused by a neurodegenerative process. In addition, difficulties in detecting and evaluating patients with PPA are increasingly apparent due to associated cognitive impairment.
Category
In 2011, the classification of primary progressive aphasia was updated to include three types. Patients were classified based on characteristics such as verbal expression, repetition, word and syntax comprehension, naming ability, semantic knowledge, and reading/spelling. These types are non-fluent (PNFA), semantic aphasia (SD), and syllable-fluent (LPA).
In the non-fluent form, core diagnostic criteria include grammatical errors and slow and awkward speech, while semantic aphasia is characterized by impairments in word comprehension, and naming ability worsens over the course of the disease.
Treatment
Because the course of PPA is continuous and gradual, patients have less chance of improvement. There are currently few specific drugs for PPA, and patients may be prescribed the same drugs as those for Alzheimer's disease. Most treatments focus on behavioral therapies designed to help patients find new ways to communicate and improve their quality of life.
Reports of Alzheimer's drugs using anti-tumor necrosis factor (TNF) drugs have shown that some patients improved with this therapy, but these results have not been independently confirmed and remain controversial.
As research into PPA progresses, we begin to understand more clearly how its symptoms are related to the spine, and the scientific community is hopeful about potential treatments. Aphasia is not simply a language problem, but an indicator of deeper brain changes. So, can we provide more effective treatments for this condition?
