Causes Of A Rapidly Progressive Dementia
There are clearly other disorders which cause rapidly progressive dementia, but I am dismissing many on the basis of the normal MRI and CSF and the lack of encephalopathic features. There are peculiar chronic encephalitic illnesses, such as Rasmussens encephalitis and Landau-Kleffner syndrome, which are predominantly diseases of childhood and adolescence, although they can present in later life. Cerebral vasculitis can be a great mimic, is a great favourite in clinicopathological conferences, and can present focally. In this case, however, there are no stroke or multiple sclerosis-like episodes, and the MRI was normal with no oligoclonal bands on CSF, so I think that it is almost inconceivable.
I also considered Hashimotos encephalopathy. It tends to affect young to middle aged women. It usually begins abruptly, and typically causes an encephalopathic illness. There can be focal signs, and headaches are common. The CSF is usually abnormal. Thyroid function can be normal, so it is imperative to check for thyroid autoantibodies. The progressive unremitting course in this patient is very much against this as a diagnosis.
Whipples disease is a possibility, especially given the history of weight loss. These patients also tend to be encephalopathic. Movement disorders, including oculomasticatory myorhythmia, and brain stem symptoms, are described in this condition.
What To Tell Your Kids About Aphasia
The best thing you can help your kids keep in mind when visiting a loved one with aphasia is to show them love and respect in the same way as before they began to struggle with words. When engaging someone with aphasia in conversation, it helps to speak slowly and clearly, and only discuss one topic at a time to reduce confusion. There’s no need for your kids to try to “carry” the conversation, or pretend they can understand what’s being said. Instead, encourage your kids to relax and be themselves. While conversation is undoubtedly more difficult, there’s a good chance the person suffering from aphasia will enjoy being around the people they love.
Lost Naming And Wordpicture Matching
Statistical analysis revealed that the patient group was impaired relative to controls at naming both high- and low-familiarity items, and matching low familiarity words to target pictures with close distractors . Due to all controls performing at ceiling on the distant distractor subtest, the data were not analysed statistically. However, as shown in , there was some overlap between patient and control performance on both the high and low familiarity conditions of the distant distractor subtest. Patient scores were generally lower for naming and matching low- relative to high-familiarity items, although the same was true of controls in the close-distractor condition. Statistical analysis comparing the difference in performance between the high and low familiarity conditions revealed that the patient group showed a larger familiarity effect than the control group when the distractor pictures were semantically close to the target . See for individual performance on each wordpicture matching condition.
Fig. 3
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Charles Marshall Phd Mrcp
We need to improve awareness that dementia can affect language as well as memory to ensure that people with PPA are diagnosed promptly and accurately.
People tend to be affected with PPA in late-middle-age, meaning that it can be an early-onset dementia.
The age of onset can differ from patient to patient, however, as can the progression of the condition someone may live for over a decade after diagnosis, and keep a high level of independence for a number of years.
Why there can be so much difference between individuals with PPA is something that scientists are researching.
PPA can, in around 10% of cases, be genetic. This means that a specific faulty gene might be causing these problems, but this is only the case for a small minority of people with PPA, says Chris Hardy, PhD, a senior research fellow at the Dementia Research Centre at the University College London Queen Square Institute of Neurology.
Levels Of Specificity And Typicality
Naming
On the basis of extensive pre-testing, we selected a series of 44 coloured pictures of objects that almost all normal individuals can name at a specific level. That is, pictures of boats and of small birds are typically named at the basic level but there are some reasonably well-known types of boats and small birds that most people, if asked, can name more precisely . In the pre-tests, these 44 items had 95% name agreement at this specific level in older controls, and can be dividedon the basis of familiarity ratings from controlsinto 22 higher- and 22 lower-familiarity objects . The pictures were presented for naming to the patients and controls in this study, with the instruction to produce the most specific name they could think of for that picture.
Wordpicture matching
Each of the same 44 items from the specific naming test was presented twice in an array of seven pictures. In one condition , the target object was accompanied by six semantically close distractors in the other condition , it occurred amongst completely unrelated, distant distractors . The name of the target was spoken by the experimenter and the participant was asked to point to its picture in the array.
Fig. 1
Example of wordpicture matching from the Levels of Specificity and Typicality test. In this example, the subject is asked to point to the Kingfisher. Close semantic distractors. Unrelated distant distractors.
Colour knowledge
Fig. 2
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What Is Primary Progressive Aphasia
In most cases, this damage is caused by a group of diseases called frontotemporal dementia. Most people who develop primary progressive aphasia will be in their 50s and 60s.
The term PPA covers three separate conditions.
- Semantic dementia
- Progressive non-fluent aphasia
- Logopenic aphasia
If you would like to find out more about primary progressive aphasia including what is happening in the brain of someone with PPA you can read our blog.
Causes Of Aphasia And Dementia
We have already established that both dementia and aphasia are caused when there are damages in the brain.
What sets the two conditions apart is the section of the brain that is hurt.
For aphasia, people will get the illness when the part of the brain that controls speech is damaged. This mostly happens because of traumatic brain injury or a stroke.
Infections and brain tumors are other probable causes of aphasia.
Dementia is quite different from this.
Even though a stroke or traumatic brain injury can also cause it, it is mostly a result of amyloid plaque build-up in the brain, which leads to the development of the most common dementia type known as Alzheimers disease.
This build-up cuts off neurons in the brain that are responsible for relaying messages from one part of the brain to the next.
It ends up affecting the entire brain and not the speech part, as is the case with aphasia.
This is why a person with dementia may not know the name of a familiar face or even remember how to hold a fork because they cannot access their memories.
The person with aphasia only has problems accessing the part of the brain that deals with speech.
These differences between aphasia and dementia are instrumental when it comes to diagnosis.
A doctor can study the symptoms to know whether a person is only having trouble with speech or they are having communication problems as well as other challenges with their day to day lives to understand what the person is suffering from.
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What Role Do Genetics And Other Risk Factors Play
In the vast majority of individuals, PPA is not genetic. However, in a small number of families, PPA can be caused by hereditary forms of FTLD. The most common gene implicated in these families is the progranulin gene . Other, less-common genes implicated in FTLD include the microtubule associated protein tau and chromosome 9 open reading frame 72 .
The risk factors for PPA seem to be different than that of Alzheimer’s disease dementia. The biggest genetic risk factor for Alzheimer’s disease dementia is ApoE4. However, research evidence indicates that ApoE4 is not a risk factor for PPA caused by AD.
Personal history or family history of learning disability in a first-degree relative, especially dyslexia, has been reported as a risk factor for PPA but not for Alzheimer’s disease dementia.
What Questions Should I Ask My Doctor
If you or a loved one has suspected or diagnosed primary progressive aphasia, you may also want to ask your healthcare provider:
- What are the early signs of primary progressive aphasia?
- What tests diagnose primary progressive aphasia?
- Are there treatments to slow the progression of primary progressive aphasia?
- What can I do to increase my quality of life with primary progressive aphasia?
A note from Cleveland Clinic
Primary progressive aphasia is a gradual loss of language skills. Its a sign of an underlying neurodegenerative disease. In some people, PPA is the first sign of Alzheimers disease, while in others, its related to frontotemporal dementia. Experts dont always know what causes this condition, but its often a combination of environmental and genetic factors. There isnt a way to reverse primary progressive aphasia, but treatment may help you maintain communication skills for as long as possible. Talk to your healthcare provider about the best treatment for you.
Last reviewed by a Cleveland Clinic medical professional on 04/12/2022.
References
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Aphasia In Alzheimers Disease And Other Dementias : Evidence From Chinese
- The SAGE Encyclopedia of Human Communication Sciences and Disorders2019
- The SAGE Encyclopedia of Human Communication Sciences and Disorders2019
- Identifying Children with Special Needs: Checklists and Action Plans for Teachers2006
- The SAGE Encyclopedia of Human Communication Sciences and Disorders2019
- Language and Communication in Primary Schools2016
- Lea A. Theodore and more…Encyclopedia of School Psychology
- The SAGE Encyclopedia of Human Communication Sciences and Disorders2019
- The SAGE Handbook of Developmental Disorders2011
Dementia & Alzheimer’s Disease
It is easy to confuse some of the symptoms of aphasia and dementia, which often leads to questions on whether the two medical conditions are related.
Before we go deeper into answering the query, it is important to first define these conditions.
Aphasia is a language or speech disorder that is a result of complications in the brain.
In Greek, the word Aphasia translates to speechlessness.
Dementia, on the other hand, is a general term that describes a range of medical conditions that are caused by abnormal brain changes.
In Latin, the term dementia stands for madness which implies severe memory loss in a person who was previously normal beyond what is expected as a person grows older.
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What Causes Primary Progressive Aphasia
Primary progressive aphasia develops when the parts of your brain that control language start breaking down. Your brain tissue shrinks , affecting your ability to communicate. Sometimes, this breakdown starts because of a gene mutation that youre born with. Other times, it may happen for no known reason.
In most people, healthcare providers often cant identify a clear predisposing risk factor. A combination of environmental and genetic factors could cause PPA. Even if you dont have a gene mutation, PPA may still run in your family.
Whats The Difference Between Dysphasia And Aphasia
Dysphasia and aphasia have the same causes and symptoms. Some sources suggest aphasia is more severe, and involves a complete loss of speech and comprehension abilities. Dysphasia, on the other hand, only involves moderate language impairments.
However, many health professionals and researchers use these terms interchangeably to refer to full and partial disruptions of language abilities. Aphasia is the preferred term in North America, while dysphasia may be more common in other parts of the world.
There are several different types and subtypes of dysphasia. Each type is associated with damage to a particular area of the brain. However, among those affected by dysphasia, distinctions are often less clear. Brain damage is rarely clear-cut.
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What Are The Types Of Primary Progressive Aphasia
Experts divide PPA into three sub-types:
- Lopogenic progressive aphasia may cause difficulty finding the right words or understanding others.
- Progressive non-fluent aphasia may cause poor grammar or difficulty talking fluently.
- Semantic dementia may cause difficulty naming objects or understanding the meaning of stand-alone words.
What’s The Difference Between Aphasia And Dementia
Aphasia and dementia are two conditions that are caused by damage to the brain, but they are not the same. Aphasia is usually caused by damage to areas of the brain that are associated with language. The National Institutes of Health explains that the disorder diminishes the ability to express language, including speaking, reading, and writing.
Aphasia generally occurs quickly after a head injury or stroke, but in some cases, it can evolve slowly over time due to some progressive neurological conditions. Aphasia can be categorized as fluent and nonfluent, and there are several types of each. Communication is hindered in most cases, and in some instances, people may have difficulty understanding words and identifying objects.
Treatment for aphasia includes speech-language therapy, which helps patients recover the ability to communicate. The NIH notes that many communication skills can improve, depending on where the brain was injured.
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About Primary Progressive Aphasia
Parts of the brain affected in PPA. The different colours represent the areas most affected in each PPA subtype .
Over 30,000 Australians live with younger-onset dementia and about a quarter are diagnosed with PPA.
The three subtypes of PPA are logopenic , non-fluent and semantic .
Over time, other cognitive and functional abilities deteriorate, resulting in death approximately 7-12 years from diagnosis. PPA generally presents before the age of 65 years, significantly impacting family, vocational, social and community roles.
It is caused by an abnormal build-up of proteins in the brain , resulting in a slow, progressive loss of brain cells.
There is currently no cure for PPA but University of Sydney researchers are investigating the underlying pathological causes of these diseases – which could lead to novel pharmacological interventions.
In the meantime, treatments such as speech therapy have been found to alleviate symptoms and improve quality of life in the early disease stages. Recently, FRONTIER and the Universitys Communication Disorders Treatment and Research Clinic have formed a partnership to provide student-led speech pathology clinical services to PPA patients and their families.
How Is Primary Progressive Aphasia Diagnosed
Your healthcare provider may suspect PPA based on your symptoms. Theyll evaluate your medical history and family history to see if you have an increased risk of primary progressive aphasia.
You might have specialized cognitive tests and brain scans, such as an MRI or CT scan, to confirm a diagnosis.
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Bilateral Temporal Lobe Atrophy
The VBM analysis confirmed bilateral temporal lobe atrophy in the patient group as a whole. On visual inspection of MRI scans, however, five of the seven patients had either more prominent atrophy in the left than right ATL or relatively symmetrical ATL damage, and only two cases had an apparent pattern of right > left atrophy. It is perhaps not surprising, therefore, that the regression analysis did not identify a significant relationship between right temporal lobe atrophy and cognitive performance for the group as a whole. It is however important to note that, apart from the fact that cases 1 and 2 were somewhat more mildly impaired than the remaining cases on both verbal and non-verbal assessments, these two cases were characterized by largely the same profile of performance as the others. This suggests that significant bilateral damage to the temporal lobes, independent of its degree or side of asymmetry, typically disrupts both verbal and non-verbal semantic memory.
What Happens To Someone With Primary Progressive Aphasia
Because PPA is progressive, language ability continues to decline. Additionally, changes in non-language abilities may occur. Movement or swallowing may also become challenging for some individuals. The rate of decline is variable from person to person and unfolds over years. Scientists are working to understand why some people progress more rapidly than others.
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A Case Of Primary Progressive Aphasia Progressing To Severe Dementia And Death
Jonathan Obert, MD
ABSTRACT: Primary progressive aphasia is a rare neurodegenerative disorder characterized by an isolated and gradual dissolution of language function that progresses to dementia. This is a case study of a 70-year-old male who was diagnosed with progressive nonfluent aphasia in 2003. Eight years later, he had a marked decline in his condition, ultimately progressing to dementia and death. This article reviews the clinical features of PPA, risk factors, diagnosis, treatment options, and autopsy findings.
Primary progressive aphasia is a rare neurodegenerative disorder characterized by an isolated and gradual dissolution of language function that progresses to dementia.1 People with PPA may have trouble with naming objects, word endings, verb tenses, conjunctions, and pronouns. The disease starts with word-finding disturbances and progresses to impair the grammatical structure and comprehension of language.1
The condition begins in middle age in the form of language difficulties. Memory, visual processing, and personality remain relatively well-preserved until the advanced stages of the disease, which helps distinguish PPA from frontal lobe dementia and the typical forms of Alzheimers disease .1,2
There are 3 classifications of PPA3:
Progressive nonfluent aphasia is characterized by progressive difficulties with the production of speech.
Semantic dementia is characterized as a disorder of word and object meaning.
A Case Study
Dementia Involves Greater Cognitive Decline
Unlike aphasia, dementia is not a single disease but rather a term used to describe many forms of cognitive decline that may include difficulty communicating. People suffering from dementia will also experience other symptoms, such as memory loss, slower thinking, trouble with social skills, confusion, and difficulty reasoning, per the Mayo Clinic. Alzheimer’s disease is just one form of dementia, and it is the most common form of the disorder. Nerve damage in dementia is caused by plaque in the brain, while others forms are caused by damaged blood vessels or a buildup of certain proteins.
Some medications can help treat certain types of dementia, while others can help slow cognitive decline, but in many cases, the results are small and cannot stop the progression of the disease. Many drugs work to improve mood and behavior to improve the patient’s quality of life, per Stanford Health Care.
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