Research Paper Example: Primary Progressive Aphasia
Primary Progressive Aphasia is a rare, neurological disease that impacts the ability to communicate. There are three variants of the disease, which include the semantic variant, logopenic variant, and nonfluent/agrammatic variant. (Fienberg School of Medicine) Primary Progressive Aphasia (PPA) is commonly associated with focal degeneration, which is defined as a formation or dysfunction in a particular area of the brain due to a degenerative process, such as dementia. This research paper strives for an in-depth analysis of this neurodegenerative disease, how it impacts an individual, and how to treat it.
“The underlying diseases causing PPA are called "neurodegenerative" because they cause gradually progressive brain cell death that is not similar to other causes, such as trauma, infection, stroke, or cancer” (Mandal, D. A). Several types of neurodegeneration can cause PPA. The three most common types are Frontotemporal lobar degeneration with abnormal tau protein accumulation (FTLD-tau), Frontotemporal Lobar Degeneration with abnormal TDP-43 accumulation (FTLD-TDP-43), and Alzheimer’s Disease (AD.) (Mandal, D. A). Not every case of Alzheimer's may have aphasia; however, some patients have experienced it.
FTLD-tau and FTLD-TDP-43 are caused by the tau protein, found in the central nervous system in large numbers. “Deposits of abnormal aggregates enriched with tau isoforms have also been reported in some other neurodegenerative diseases.” Dr. Ananya Mandal MD states that these proteins are abundant in nerve cells and are present to a much lesser degree in oligodendrocytes and astrocytes (Mandal, D. A). It's when these tau proteins accumulate or are found with abnormalities that they begin to cause problems in diseases such as Primary Progressive Aphasia.
Due to Primary Progressive Aphasia being developed over time, language ability continues to decline. There may be additional symptoms such as behavioral problems, memory issues, attention shortages, and the rate of degeneration varies by person. About 40-50% of people with Primary Progressive Aphasia have other family members who are affected by the disease. According to Ester Heerema MSW, “People who develop PPA show atrophy in the area of the brain where speech and language are controlled. Some cases of PPA have a genetic component found in a mutation of the GRN gene” (Ester Heerema, MSW).
Complications of Primary Progressive Aphasia can impact a person’s reading and writing skills. They may require daily care assistance, have difficulty forming sounds for words, also known as apraxia of speech, experience blunted emotions, or use inappropriate social behaviors and mannerisms. Many of those living with PPA have trouble naming objects, difficulty forming words, frequent pauses in speech, slow speech, difficulty comprehending speech, and problems with grammar.
Those affected can become mute and ultimately lose the ability to understand written or spoken language. Judgment and cognitive skills may also be affected by this disease. These traits are so similar in all three variants of the disease that it becomes challenging to diagnose. Diagnosis of PPA is very rare due to the similarities between the variant's symptoms and other neurodegenerative diseases that are closely related. Many patients are diagnosed after they pass away because of these factors. Those who can be diagnosed before death are usually tested through CT scans or MRI imaging to confirm the diagnosis (NORD).
There are a handful of therapy options; however, there is no definite cure for those with Primary Progressive Aphasia. Many of those with PPA choose speech therapy to help aid them in maintaining their ability to talk. Selective serotonin reuptake inhibitors, such as Fluoxetine, are most effective in those who have had depressive episodes with PPA. Fluoxetine has been shown with a three-month treatment to help improve recovery in post-stroke patients (Mesulam Center for Cognitive Neurology). Treatments such as Fluoxetine have not yet been approved but can completely reverse the roles of PPA.
Selective serotonin reuptake inhibitors have also been used to treat behavioral issues that become prominent in PPA. SSRI treatments are typically used to treat depression by raising the amount of serotonin in the brain. (Feinberg School of Medicine: Northwestern University). Currently, post-stroke treatments are being researched for use in primary progressive aphasia patients. “Recently, a growing body of literature of treatment in stroke-based aphasia have found promising results for pairing traditional language therapy with non-invasive neurostimulation via transcranial direct current stimulation (tDCS)” (Feinberg School of Medicine: Northwestern University).
tDCS has been significant in aiding with word retrieval and language stimuli. This treatment uses direct currents to specific locations on the brain. In research, it has been successful in treating traumatic brain injury and strokes. tDCS has also been used with symptoms such as language disorders, impaired cognition, chronic pain, and movement disorders (Toulouse Institute of Neurosciences). With Primary Progressive Aphasia, many complications become apparent. Complications such as the development of mental and social problems blunted emotions, and impairment is possibilities. Over time, one may find that they are becoming hard of hearing or have shaking, similar to Parkinson's Disease.
The impact of PPA may cause patients to require home care because of the difficulty of performing daily tasks. Some living with PPA choose to continue working if their job doesn't require a large amount of speaking or collaborating with others. Whether they will be able to continue working depends on their level of progression (NORD). The typical life expectancy after diagnosis is about three to twelve years. The complications of PPA, such as swallowing difficulty, are the main causes of fatality.
People who have Primary Progressive Aphasia may be more likely to experience complications such as injury or infections such as pneumonia (NORD). Brain cell death or damage in areas that control language, such as the left hemisphere or, more specifically, the Broca’s area, are a result of the build-up of toxic tau proteins (NORD). High levels of tau proteins are associated with rapid cognitive decline. Many studies are being conducted to help find more effective treatments for Primary Progressive Aphasia patients. Research is being performed at the University of Pennsylvania in which language testing and brain imaging before and after receiving ten partially consecutive daily sessions of real or sham high-definition transcranial direct current stimulation (HD-tDCS) are paired with modified constraint-induced language therapy (mCILT).
Language testing and brain imaging will be repeated after completion and 3 months post completion of treatment. mCILT is avoiding the use of any compensatory strategies (for example, drawing or pointing.) Patients participating in this study are required only to talk rather than use actions (University of Pennsylvania).
Another study being performed at the University of Pennsylvania will use direct current stimulation Language testing and brain imaging will be repeated immediately after completion of and up to twenty-four weeks following completion of the treatment.
In terms of clinical advancement, recent studies like those at the University of Pennsylvania have allowed a better characterization and differentiation of PPA patients based on their profiles. The improved reliability of diagnoses provides a better outlook for this neurogenerative disease. This helps develop new tests for diagnosis, treatments, and procedures that could eventually benefit others as well. Research on its own improves services and treatments not just for the patient but also for future generations.
