Progressive Non -Fluid aphasia (2024)

Activities of permanent education

Aphasia is inability to vocalize or understand spoken or written language.Shortages by expressing or understanding languages, such as with a stroke, where primary progressive aphasia concerns the gradual limitation of these functions..

Goal:

• Identify the etiology and epidemiology of progressive non -fluid aphasia.

• View the relevant story, physical and evaluation to diagnose progressive non -fluid aphasia.

• Sketch the treatment and management options that are available for progressive non -liquidhasia.

• Summit the interprofessional team strategies to improve care coordination and communication to promote the improvement and rehabilitation of progressive non -fluid aphasia and improve the results.

Access to free questions about multiple choices on this subject.

Introduction

Afasi Isa -language disorder caused by injuries in a specific brain area that controls language expressions and understanding..[1]Although aphasia suddenly has access to deficits, as with a stroke, PPA gradually includes the deterioration of these functions.

With regard to clinical progress, recent studies have made better characterization and differentiation possible on the basis of non -linguistic profiles of PPA patients.A deeper understanding of a deeper insight into the effects of his PPA on changes in structural and functional connection is still far from crucial clinic -copathological consensus on pathology.

Fluent Aphasi refers to deficits related to understanding and is usually overwritten with Wernice's brain area -pathologies.Although the general concept of a primary progressive language for reduced disease without cognitive effects was first described in the 1890s by Pick and Serieux, it was only in the 1980s that PPA was invented and further examined by Mesulam.[2][3]It was originally slowly called progressive aphasia, but was later replaced by the primary progressive Aphasia (PPA) name.[4]

Due to the close relationship with Alzheimer's disease and choosing diseases, the classification and diagnosis of PPA can sometimes be controversial.[5]PPA is mainly divided into three main variants: non -flowing/agrammatical, semantic and logopenic.[6]There is also the disruption of primary progressive apraxi, a similar gradual, degenerative disorder that influences the planning, programming and sensorimotor commands needed for the actual production of speech.[7]

The non -fluid/agrammatic and semantic variants are often grouped under the frontotemporal dementia syndromes, and the logopenic variant is often classified as an atypical variant of Alzheimer's disease.

Etiology

Non -flowing/Agrammatic variant of PPA (NVFPPA)

NFVPPA is usually associated with a form of FTD-4R Tau.[8][9][10]TDP-43-A patology indicates other reports in NFVPPA.[8][9][11]And in some cases associated with progranulin (pgrn) or chromosome 9 open reading round 72 (C9ORF72) Gen mutations.[12]Less often ad -pathology is also reported in NFVPPA.[8][9]The three most important genes associated with frontotemporal lobar degeneration are microtubule-associated protein-tau (MAPT), Granulin (GRN) and C90RF72.[7]Frontotemporal Lobar -Ueteneration is often associated with NFVPPA;Interesting enough, potential recruited cohorts from PPA that mutations are rarely the cause of NFVPA.[13]

NFVPPA is the most variable of the PPAs and lacks clinical -pathological associations.[7]GRN (granulin) Gen mutations on chromosome 17Q21.31, which codes for a protein called progranulin, is identified in two families with PPA.[14]This mutation garden is associated with behavioral variants of frontotemporal Lob Dementia.

Semantic variant of PPA (SVPPA)

SVPPA is almost always associated with underlying TDP-43-C pathological aggregates (75-100% in clinical-pathological correlation series) and for the rest of the patients, usually with FTD Tau.[8][10]

Lopogene variant van PPA (LVPPA)

LVPPA is usually caused by AD pathology in no less than 95% of the cases.[8][10][15]It is considered one of the possible focal and early starting presentations of AD, although other pathological profiles are rarely described, including Lewy Body Dementia, TDP-43 and Tau.[16][10]

Epidemiology

The estimated performance of NFVPPA is approximately.0.5 to 3.9 Per100,000 people.[13]Men and women are struck equally with an average age of approximately.60.No demographic, environmental or social -economic risk factors have been identified.Cirka 56% to 86% of the cases have an existing speech side by side.[7]

Pudding

The pathophysiology of NFVPPA is not clear and is still being investigated.[17]This coincides with the brain areas involved in language production, sentence treatment and programming of motor speech.

The high variation of NFVPPA compared to other PPAs is maintained histologically and remains a continued subject of study.found with a minority of patients.[8]

Story and physical

Patients with NFVPPA will often have a gradual deterioration of speech disorders over the years.Poor syntax, omission of verbs and reversal of binary terms such as "yes" and "no" with a few attempts to repair themselves.[3]Speech sound errors can be visible and there is a prisoner, hesitant speech with frequent breaks or stopped.[17]

Patients can complain about dropping words or family members can worry about the patient by means of words out of effect.Writing and abuse of functional morphemes (the majority, verbs that -ing, -ed and conjunctions).[7]Insight into shortage can often be seen with synthetic and grammatically complex sentences.Diagnosis of NFVPA and primary progressive apraxia.[7]However, the more prominent symptom of the presentation is often used to distinguish the diagnosis between the two pathologies.

Patients will often express frustration and sometimes depression because of their prisoner insight into their condition.[17]Patients can have a behavioral flow in the course of the disease and can even meet behavioral variant Frontotemporal dementia criteria. Patients with NFVPA can also develop a mild cognitive decline a few years after the start of Afasic symptoms.[7]

Physical examination, including the neurological examination, is usually normal.[7]Patients can also show mild frontal LOB disfunction with impulsivity, endurance and attentive deficits.

Non -flowing/Agrammatic variant of PPA (NVFPPA)

The characteristic clinical characteristics of NFVPPA are company and agrammatism.Apraxia of speech (AOS).[18]Speech sound errors consisting of distortions, wiping, substitutions, inserts or speech sounds are present.Down in motor speech or a shortage of Phonema Committee.[19][20]However, there are some important challenges in clinically distinguishing these two types of errors, and both studies show higher frequencies of phonetic orphonemic errors.[21][20]

NFVPPA has reported on patients. With the development of the disease, other cognitive deficits can arise, including a decrease in attentive sources and verbal working memory and performing functions, episodic memory, practice and behavioral symptoms.[22]

The current clinical criteria for NFVPPA include at least one of the following core functions: (1) agramatism in language production;Other functions: (1) reduced concept of syntactic complex sentences;(2) saved with a single word concept;[3]

Semantic Variant PPA

Patients with SVPPA gradually lose the meaning of words and are usually present with severe, progressive anomy and reduced understanding of individual words.[3]In earlier phases, names and understanding of one word are more prominent for low -frequency/confidentiality (eg "Rhinoceros" versus "dog").[23]Often patients replace less frequent words with more known words, usually with the help of the superordinate category (eg "animals" for "cat").[24]

In the early stages, the inability to understand words of low family law can understand the only symptom accompanying anomia, and patients often ask for the meaning of words.Touch, olfactie and wind flight.Concepts in relation to abstract concepts.[25]

Episodic memory is relatively stored in SVPPA, especially when using tasks with minimal conceptual load.[26]Behavioral abnormalities are typically present in medium -sized phases, including disinhibition, irritability and changes in food tastings (for example, preference for sweet foods).[27]

The current diagnostic guidelines[3]Identify Anomi and Single-Word conceptual shortages as core functions, both essential for diagnosis.2) surface -dyslexia or dysgrafia;(3) spatted repetition;

Lopogen variant PPA

Patients with LVPPA usually show problems with finding words together with repetition deficits and as the disease progresses is a reduced sentence.[28]In accordance with this interpretation, repetition and understanding of individual words are largely spared.[29]But also visuospatial skills.

Criteria for LVPPA require that both core functions are present: reduced single-word collection in spontaneous speech and naming together with reduced repetition of sentences and sentences.Spontaneous speech and naming;(2) saved with a single word understanding and object knowledge;(3) Save motor speech;[3]

Evaluation

The evaluation of a patient with progressive aphasia symptoms requires achieving a detailed story and a specific test of the speech and language of the patient.[3]The first step requires that the patient meets the PPA requirements designed by Mesulam, with a series of inclusion criteria, all of which must be answered positively and a different series of exclusion criteria, all of which must be answered negatively.[30]This is followed by a group of diagnostic characteristics developed by Gorno-Tempini, which helps to distinguish the different types of PPA.[3]

In addition to the history of the patient, the Northwestern Anagram test (night) is a commonly used grammar test and patients with NFVPA will also have reduced performance.Out of symptoms of dementia.However, MOCA must be used with caution, because patients with NFVPA can only score during the examination as a result of reduced spontaneous verbal output and the apraxia of speech can be developed. After the diagnosis, the progressive aphasia difficulty (pass) clinicians set the cliniciis to follow progress and characterize symptoms, especially with PPA patients.[31]

Imaging can also be useful, because fludeoxyglucose -Possitron -emissite tomography is often used in the context of the brain's magnetic resonance image to evaluate primary progressive aphasis.[7]

Neuroimaging -fund

Non-flowing/agrammatic variant of PPA: The left-left frontal gyrus (PARS Opercularis) is considered the Syndrome-specific episodes of NFVPPA.[32][33]It is also associated with gray matter (GM) atrophy in Insula, Premotor Regions, SMA and Striatum.[8][34]Diffusion Magnetic Resonance Imaging (MRI) techniques have shown that the dorsal language path for long -range white substance (WM) fibers that connect frontal, subcortical and parietal areas is mainly involved in neurodegeneration in NFVPPA.[34]

The semantic variant of PPA: the front temporal lobes show bilateral atrophy and hypoperfusion in SVPPA and is considered the syndrome -specific episent.[8][35]This focal anatomical injury makes neuroimaging a complementary tool in the diagnostic process of this PPA variant.[36]The damage is usually greater on the left hemisphere in the first stages of the disease.[37]

Lopogenic variant PPA: Anatomical damage to LVPPA is usually in the rear total temporal and middle temporal gyri and the subordinate parietal lobule.[38]This pattern of atrophy is consistent with the classic anatomical model of the phonological loop.[28]This neurodegenerative pattern is very similar to what was observed at the beginning of the beginning.[39]

Treatment / control

There are no medicines that have proven to be beneficial for primary progressive aphasis.The relevant treatment regime and strategy. Supply and language therapy are the most effective treatment of primary progressive aphasia, especially for symptoms of speech praxia.[7]

Transcranial DC stimulation (TDCs) have been identified as a promising intervention to increase the benefits of behavioral treatment in language therapy programs.[40]

Differentiele diagnose

Dementia: A patient can have aphasia components as part of a diagnosis of dementia, but NFVPPA will always have aphasia as the first and primary symptom.

• Ischemic aphasia: an ischemic process will have symptoms of aphasia that occur more acute.

• Brain -Tumor: A brain tumor that is in a place that influences speech and language can cause aphasia in a progressive course as lesion gradually grows.

• Alzheimer's disease: Dementia is an initial and prominent sign of Alzheimer's disease;NFVPPA will have no dementia at the start of the disease process, because aphasia should be the primary and first symptom.

• Frontotemporal dementia: Dementia can develop a few years after diagnosing NFVPPA;

prophesy

The disease is progressive and because there is currently no remedy, patients ultimately lose the ability to speak and understand in written and spoken language.After the first performance of symptom.[41]Due to the heterogeneous variety of the NFVPPA disease process, individual prognosticization is somewhat unreliable.

Most patients with PPA live 3 to 12 years after diagnosis.[13]Some rare cases are described in which patients with NFVPPA ultimately developed corticobasal syndrome or progressive supranuclear palsy;However, these are often noticed with simultaneous apraxi.[7]

Complications

• Loss of the ability to speak and write

• Loss of ability to understand in writing and spoken language

• Depression

  See Also

  Aphasia: What You Need to Know

• Bad judgment

• Inappropriate social behavior

• Parkinsonism, raised reflexes or corticobasal syndrome can rarely occur

Consultation

• Neurologist

• Speaking and language pathologist

• Speech education

• Social worker

• Psychologist

Deterrence and patient education

Because PPA can have an early start (NVFPA variant), with a progressive clinical course, patients and families are the risk of developing depression symptoms.And family members to offer expectations, to develop coping mechanisms and offer emotional support.

Improving the results of the health care team

Patients with NFVPPA are best checked with an interprofessional team approach.With more liquid limits between certain diagnoses.New findings that may have to be treated a few years after the first symptom presentation.In later stages, PPA may require psychological and psychiatric intervention for cases of significant depression.

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