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Progressive Nonfluent Aphasia
Progressive nonfluent aphasia (PNFA) is one of three clinical syndromes associated with frontotemporal lobar degeneration. PNFA has an insidious onset of language deficits over time as opposed to other stroke-based aphasias, which occur acutely following trauma to the brain. The specific degeneration of the frontal and temporal lobes in PNFA creates hallmark language deficits differentiating this disorder from other Alzheimer-type disorders by the initial absence of other cognitive and memory deficits. This disorder commonly has a primary effect on the left hemisphere, causing the symptomatic display of expressive language deficits (production difficulties) and sometimes may disrupt receptive abilities in comprehending grammatically complex language. Presentation The main clinical features are signature language progressive difficulties with speech production. There can be problems in different parts of the speech production system, hence patients can present with articulatory bre ...
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Frontotemporal Lobar Degeneration
Frontotemporal lobar degeneration (FTLD) is a pathological process that occurs in frontotemporal dementia. It is characterized by atrophy in the frontal lobe and temporal lobe of the brain, with sparing of the parietal and occipital lobes. Common proteinopathies that are found in FTLD include the accumulation of tau proteins and TAR DNA-binding protein 43 (TDP-43). Mutations in the '' C9orf72'' gene have been established as a major genetic contribution of FTLD, although defects in the granulin (GRN) and microtubule-associated proteins (MAPs) are also associated with it. Classification There are 3 main histological subtypes found at post-mortem: * FTLD-tau is characterised by tau positive inclusion bodies often referred to as Pick-bodies. Examples of FTLD-tau include; Pick's disease, corticobasal degeneration, progressive supranuclear palsy. * FTLD-TDP (or FTLD-U ) is characterised by ubiquitin and TDP-43 positive, tau negative, FUS negative inclusion bodies. The pat ...
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Frontotemporal Dementia
Frontotemporal dementia (FTD), also called frontotemporal degeneration disease or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of the brain's frontal lobe, frontal and temporal lobes. Men and women appear to be equally affected. FTD generally presents as a behavioral or language disorder with gradual onset. Signs and symptoms tend to appear in late adulthood, typically between the ages of 45 and 65, although it can affect people younger or older than this. There is currently no cure or approved symptomatic treatment for FTD, although some Off-label use, off-label drugs and behavioral methods are prescribed. Features of FTD were first described by Arnold Pick between 1892 and 1906. The name ''Pick's disease'' was coined in 1922. This term is now reserved only for the behavioral variant of FTD, in which characteristic Pick bodies and Pick cells are present. These were first described by Alois Alzheimer in 1911. ...
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Aphasias
Aphasia, also known as dysphasia, is an impairment in a person's ability to comprehend or formulate language because of dysfunction in specific brain regions. The major causes are stroke and head trauma; prevalence is hard to determine, but aphasia due to stroke is estimated to be 0.1–0.4% in Developed country, developed countries. Aphasia can also be the result of brain tumors, epilepsy, autoimmune neurological diseases, brain infections, or neurodegenerative diseases (such as dementias). To be diagnosed with aphasia, a person's language must be significantly impaired in one or more of the four aspects of communication. In the case of progressive aphasia, a noticeable decline in language abilities over a short period of time is required. The four aspects of communication include spoken language production, spoken language comprehension, written language production, and written language comprehension. Impairments in any of these aspects can impact functional communication. Th ...
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Pick's Disease
Frontotemporal dementia (FTD), also called frontotemporal degeneration disease or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of the brain's frontal and temporal lobes. Men and women appear to be equally affected. FTD generally presents as a behavioral or language disorder with gradual onset. Signs and symptoms tend to appear in late adulthood, typically between the ages of 45 and 65, although it can affect people younger or older than this. There is currently no cure or approved symptomatic treatment for FTD, although some off-label drugs and behavioral methods are prescribed. Features of FTD were first described by Arnold Pick between 1892 and 1906. The name ''Pick's disease'' was coined in 1922. This term is now reserved only for the behavioral variant of FTD, in which characteristic Pick bodies and Pick cells are present. These were first described by Alois Alzheimer in 1911. Common signs and symptoms ...
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Corticobasal Degeneration
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. It is characterized by marked disorders in movement and cognition, and is classified as one of the Parkinson plus syndromes. Diagnosis is difficult, as symptoms are often similar to those of other disorders, such as Parkinson's disease, progressive supranuclear palsy, and dementia with Lewy bodies, and a definitive diagnosis of CBD can only be made upon neuropathologic examination. Signs and symptoms Because CBD is progressive, a standard set of diagnostic criteria can be used, which is centred on the disease's evolution. Included in these fundamental features are problems with cortical processing, dysfunction of the basal ganglia, and a sudden and detrimental onset. Psychiatric and cognitive dysfunctions, although present in CBD, are much l ...
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Alzheimer's Disease
Alzheimer's disease (AD) is a neurodegenerative disease and the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events. As the disease advances, symptoms can include problems with language, disorientation (including easily getting lost), mood swings, loss of motivation, self-neglect, and behavioral issues. As a person's condition declines, they often withdraw from family and society. Gradually, bodily functions are lost, ultimately leading to death. Although the speed of progression can vary, the average life expectancy following diagnosis is three to twelve years. The causes of Alzheimer's disease remain poorly understood. There are many environmental and genetic risk factors associated with its development. The strongest genetic risk factor is from an allele of apolipoprotein E. Other risk factors include a history of head injury, clinical depression, and high blood pressure. The progression of the di ...
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Logopenic Progressive Aphasia
Logopenic progressive aphasia (LPA) is a variant of primary progressive aphasia. It is defined clinically by impairments in naming and sentence repetition. It is similar to conduction aphasia and is associated with atrophy to the left posterior temporal cortex and inferior parietal lobule. It is suspected that an atypical form of Alzheimer's disease is the most common cause of logopenic progressive aphasia. Although patients with the logopenic variant of PPA are still able to produce speech, their speech rate may be significantly slowed due to word retrieval difficulty. Over time, they may experience the inability to retain lengthy information, causing problems with understanding complex verbal information.UCSF Memory and Aging Center. (2011, February 22). SF: The Regents of the University of California. Retrieved December 5, 2011 from http://memory.ucsf.edu/education/diseases/ppa Some additional behavioral features include irritability, anxiety and agitation. Compared to oth ...
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Semantic Dementia
In neurology, semantic dementia (SD), also known as semantic variant primary progressive aphasia (svPPA), is a progressive neurodegenerative disorder characterized by loss of semantic memory in both the verbal and non-verbal domains. However, the most common presenting symptoms are in the verbal domain (with loss of word meaning). Semantic dementia is a disorder of semantic memory that causes patients to lose the ability to match words or images to their meanings. However, it is fairly rare for patients with semantic dementia to develop category specific impairments, though there have been documented cases of it occurring. Typically, a more generalized semantic impairment results from dimmed semantic representations in the brain. SD is one of the three canonical clinical syndromes associated with frontotemporal lobar degeneration (FTLD), with the other two being frontotemporal dementia and progressive nonfluent aphasia. SD is a clinically defined syndrome but is associated wi ...
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Primary Progressive Aphasia
In neurology, primary progressive aphasia (PPA) is a type of neurological syndrome in which language capabilities slowly and progressively become impaired. As with other types of aphasia, the symptoms that accompany PPA depend on what parts of the brain's left hemisphere are significantly damaged. However, unlike most other aphasias, PPA results from continuous deterioration in brain tissue, which leads to early symptoms being far less detrimental than later symptoms. Those with PPA slowly lose the ability to speak, write, read, and generally comprehend language. Eventually, almost every patient becomes mute and completely loses the ability to understand both written and spoken language. Although it was first described as solely impairment of language capabilities while other mental functions remain intact, it is now recognized that many, if not most of those with PPA experience impairment of memory, short-term memory formation and loss of executive functions. It was fi ...
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Language Center
In neuroscience and psychology, the term language center refers collectively to the areas of the brain which serve a particular function for speech processing and production. Language is a core system that gives humans the capacity to solve difficult problems and provides them with a unique type of social interaction. Language allows individuals to attribute symbols (e.g. words or signs) to specific concepts, and utilize them through sentences and phrases that follow proper grammatical rules. Finally, speech is the mechanism by which language is orally expressed. Information is exchanged in a larger system, including language-related regions. These regions are connected by white matter fiber tracts that make possible the transmission of information between regions. The white matter fiber bunches were recognized to be important for language production after suggesting that it is possible to make a connection between multiple language centers. The three classical language areas t ...
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Muteness
In human development, muteness or mutism is defined as an absence of speech, with or without an ability to hear the speech of others. Mutism is typically understood as a person's inability to speak, and commonly observed by their family members, caregivers, teachers, doctors or speech and language pathologists. It may not be a permanent condition, as muteness can be Cause (medicine), caused or manifest due to several different phenomena, such as physiological injury, illness, medical side effects, psychological trauma, Developmental disorder, developmental disorders, or Neurological disorder, neurological disorders. A specific physical disability or communication disorder can be more easily diagnosed. Loss of previously normal speech (aphasia) can be due to accidents, disease, or surgical complication; it is rarely for Psychology, psychological reasons. Treatment or management also varies by cause and this can often be determined after a speech assessment. Treatment can sometimes r ...
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Frontal Lobe
The frontal lobe is the largest of the four major lobes of the brain in mammals, and is located at the front of each cerebral hemisphere (in front of the parietal lobe and the temporal lobe). It is parted from the parietal lobe by a Sulcus (neuroanatomy), groove between tissues called the central sulcus and from the temporal lobe by a deeper groove called the lateral sulcus (Sylvian fissure). The most anterior rounded part of the frontal lobe (though not well-defined) is known as the frontal pole, one of the three Cerebral hemisphere#Poles, poles of the cerebrum. The frontal lobe is covered by the frontal cortex. The frontal cortex includes the premotor cortex and the primary motor cortex – parts of the motor cortex. The front part of the frontal cortex is covered by the prefrontal cortex. The nonprimary motor cortex is a functionally defined portion of the frontal lobe. There are four principal Gyrus, gyri in the frontal lobe. The precentral gyrus is directly anterior to the ...
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