Materiały źródłowe

• #1 Primary Progressive Aphasia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563145/

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. Men and women are equally affected, with an average age of about 60. No demographic, environmental, or socioeconomic risk factors have been identified. Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #2 Incidence of Primary Progressive Apraxia of Speech and Primary Progressive Aphasia in Olmsted County, MN, 2011-2022 – PubMed

  https://pubmed.ncbi.nlm.nih.gov/39079073/

  No epidemiologic studies have formally assessed the incidence of primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS). […] The incidence of PPA + PPAOS was 0.70 persons per 100,000 person-years (95% CI 0.34-1.29 persons per 100,000) during the study period. The incidence of PPAOS was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55 persons per 100,000), whereas for the 8 patients with PPA, the incidence was 0.56 persons per 100,000 person-years (95% CI 0.24-1.10 cases per 100,000). The incidence of nfvPPA was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55), 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for lvPPA, and 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for svPPA. […] As a group, PPA and PPAOS are a relatively rare group of diseases. PPAOS has a slightly lower incidence than PPA as a group but similar incidence to the individual PPA variants.

• #2 Primary Progressive Aphasia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/82030

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. […] A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. […] The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. […] Men and women are equally affected, with an average age of about 60. […] No demographic, environmental, or socioeconomic risk factors have been identified. […] Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. […] A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #3 Primary progressive aphasia | Tidsskrift for Den norske legeforening

  https://tidsskriftet.no/en/2022/11/clinical-review/primary-progressive-aphasia

  Neurodegenerative dementia may, in rare cases, initially manifest as isolated language impairments in the absence of other cognitive symptoms. The prevalence of primary progressive aphasia is estimated to be approximately 34 per 100,000 individuals, i.e., about the same as amyotrophic lateral sclerosis. In a recent registry study, the incidence of primary progressive aphasia (all types) was estimated to be 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for typical Alzheimer’s dementia. […] The purpose of this article is to provide a brief overview of the different variants of primary progressive aphasia. […] Knowledge of the conditions, detailed analysis of diagnostic imaging and the use of targeted cognitive tests to assess speech and language are important for making the correct diagnosis. Patients with primary progressive aphasia are often initially misdiagnosed, for example, their aphasia may be misattributed to a stroke. If the initial workup suggests a language disorder as part of a degenerative dementia, the patient should be referred to the specialist healthcare service for further testing. Speech and language assessment may be necessary to obtain the correct diagnosis, and speech and language therapy can be effective when targeted at the specific difficulties experienced by the patient.

• #4 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Text

  https://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6

  The goal of the present study, based on a French cohort, was to describe the demographics and the evolution of subjects with (PPA) in comparison with Alzheimers disease (AD) on a period of 7 years. […] The incidence rate of PPA was 1.14 per 100,000 person-years, while the incidence rate of AD was 35.7 per 100,000 persons-years. […] This study improves the knowledge of PPA epidemiology and has the potential to help adopting appropriate public health service policies. […] Our secondary objectives were to determine if diagnosis of PPA is more difficult to establish and more delayed than AD and what are the related prescribed treatment over 7 years of follow-up. […] The delay between the first consultation for cognitive troubles and the first diagnosis that is significantly longer than in the AD group.

• #5 Primary Progressive Aphasia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/82030

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. […] A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. […] The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. […] Men and women are equally affected, with an average age of about 60. […] No demographic, environmental, or socioeconomic risk factors have been identified. […] Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. […] A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #6 Primary progressive aphasia | Tidsskrift for Den norske legeforening

  https://tidsskriftet.no/en/2022/11/clinical-review/primary-progressive-aphasia

  Neurodegenerative dementia may, in rare cases, initially manifest as isolated language impairments in the absence of other cognitive symptoms. The prevalence of primary progressive aphasia is estimated to be approximately 34 per 100,000 individuals, i.e., about the same as amyotrophic lateral sclerosis. In a recent registry study, the incidence of primary progressive aphasia (all types) was estimated to be 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for typical Alzheimer’s dementia. […] The purpose of this article is to provide a brief overview of the different variants of primary progressive aphasia. […] Knowledge of the conditions, detailed analysis of diagnostic imaging and the use of targeted cognitive tests to assess speech and language are important for making the correct diagnosis. Patients with primary progressive aphasia are often initially misdiagnosed, for example, their aphasia may be misattributed to a stroke. If the initial workup suggests a language disorder as part of a degenerative dementia, the patient should be referred to the specialist healthcare service for further testing. Speech and language assessment may be necessary to obtain the correct diagnosis, and speech and language therapy can be effective when targeted at the specific difficulties experienced by the patient.

• #7 Incidence of Primary Progressive Apraxia of Speech and Primary Progressive Aphasia in Olmsted County, MN, 2011-2022 – PubMed

  https://pubmed.ncbi.nlm.nih.gov/39079073/

  No epidemiologic studies have formally assessed the incidence of primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS). […] The incidence of PPA + PPAOS was 0.70 persons per 100,000 person-years (95% CI 0.34-1.29 persons per 100,000) during the study period. The incidence of PPAOS was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55 persons per 100,000), whereas for the 8 patients with PPA, the incidence was 0.56 persons per 100,000 person-years (95% CI 0.24-1.10 cases per 100,000). The incidence of nfvPPA was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55), 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for lvPPA, and 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for svPPA. […] As a group, PPA and PPAOS are a relatively rare group of diseases. PPAOS has a slightly lower incidence than PPA as a group but similar incidence to the individual PPA variants.

• #8 Incidence of Primary Progressive Apraxia of Speech and Primary Progressive Aphasia in Olmsted County, MN, 2011-2022 – PubMed

  https://pubmed.ncbi.nlm.nih.gov/39079073/

  No epidemiologic studies have formally assessed the incidence of primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS). […] The incidence of PPA + PPAOS was 0.70 persons per 100,000 person-years (95% CI 0.34-1.29 persons per 100,000) during the study period. The incidence of PPAOS was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55 persons per 100,000), whereas for the 8 patients with PPA, the incidence was 0.56 persons per 100,000 person-years (95% CI 0.24-1.10 cases per 100,000). The incidence of nfvPPA was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55), 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for lvPPA, and 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for svPPA. […] As a group, PPA and PPAOS are a relatively rare group of diseases. PPAOS has a slightly lower incidence than PPA as a group but similar incidence to the individual PPA variants.

• #9 Primary Progressive Aphasia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563145/

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. Men and women are equally affected, with an average age of about 60. No demographic, environmental, or socioeconomic risk factors have been identified. Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #10 Primary Progressive Aphasia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/82030

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. […] A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. […] The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. […] Men and women are equally affected, with an average age of about 60. […] No demographic, environmental, or socioeconomic risk factors have been identified. […] Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. […] A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #11 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898

  Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. […] We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. […] While its frequency in the general population is unknown, it can be extrapolated from available data on patients with frontotemporal dementia (FTD). The prevalence of FTD is calculated as approximately 5 cases per million inhabitants with between 1 and 15 cases per 100000 inhabitants in individuals younger than 65 years. PPA accounts for 20% to 40% of these cases. […] The vast majority of PPA cases are sporadic. […] Some patients belong to families with mutations on chromosome 17, either in the gene coding for microtubule-associated protein tau (MAPT) or the gene coding for progranulin.

• #12 Primary Progressive Aphasia – The Brain Clinic

  https://thebrainclinic.com/articles-3/primary-progressive-aphasia/

  Primary progressive aphasia is a rare disorder, and its prevalence in the general population is largely unknown. The overall prevalence of FTD is five cases per million people, 1-15 cases per 100,000 individuals younger than 65, and 0.2-0.3% of individuals over the age of 65. Primary progressive aphasia accounts for 20-40% of all FTD cases. Some studies have found a slightly higher rate in males than females […] Onset typically occurs between the ages of 50 and 70 years. An individual may be diagnosed with mild cognitive impairment (MCI) in the beginning stages of the illness when the deficits do not substantially interfere with daily life. MCI is a dynamic, transitional stage between normal cognitive status (compared to others of similar age and educational background) and dementia. A person is diagnosed with primary progressive aphasia if their area of deficit is confined to the language domain for at least two years. The median disease duration for primary progressive aphasia is 7-10 years.

• #13 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898?redirectNew=true

  Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. […] We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. […] While its frequency in the general population is unknown, it can be extrapolated from available data on patients with frontotemporal dementia (FTD). The prevalence of FTD is calculated as approximately 5 cases per million inhabitants with between 1 and 15 cases per 100000 inhabitants in individuals younger than 65 years. PPA accounts for 20% to 40% of these cases. […] The vast majority of PPA cases are sporadic. […] Some patients belong to families with mutations on chromosome 17, either in the gene coding for microtubule-associated protein tau (MAPT) or the gene coding for progranulin.

• #14 Primary Progressive Aphasia – The Brain Clinic

  https://thebrainclinic.com/articles-3/primary-progressive-aphasia/

  Primary progressive aphasia is a rare disorder, and its prevalence in the general population is largely unknown. The overall prevalence of FTD is five cases per million people, 1-15 cases per 100,000 individuals younger than 65, and 0.2-0.3% of individuals over the age of 65. Primary progressive aphasia accounts for 20-40% of all FTD cases. Some studies have found a slightly higher rate in males than females […] Onset typically occurs between the ages of 50 and 70 years. An individual may be diagnosed with mild cognitive impairment (MCI) in the beginning stages of the illness when the deficits do not substantially interfere with daily life. MCI is a dynamic, transitional stage between normal cognitive status (compared to others of similar age and educational background) and dementia. A person is diagnosed with primary progressive aphasia if their area of deficit is confined to the language domain for at least two years. The median disease duration for primary progressive aphasia is 7-10 years.

• #15 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  Primary Progressive Aphasias (PPAs) are rare neurodegenerative disorders classified within frontotemporal lobar degeneration (FTLD) and typically manifest between 45 and 70 years of age. […] In Mexico—and many other countries—reliable epidemiological data are lacking; however, estimates suggest that PPA accounts for 0.5–2.5% of neurodegenerative disease cases in Memory Clinics, with an incidence of approximately 1 per 100,000 and an average survival of 8 years. […] PPA is estimated to affect 0.5–2.5% of patients with neurodegenerative diseases in Memory Clinics, with an incidence of roughly 1 per 100,000. Average survival time is around 8 years (ranging from 3 to 17 years), with a generally balanced gender ratio, though some studies indicate a predominance of men. […] A positive family history is observed in 20–40% of cases, with about 10% following an autosomal dominant inheritance pattern.

• #16 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  PPA cases have been reported globally, with no clear geographic clustering. Studies show that PPA affects diverse populations, although prevalence estimates are similar across regions, which are estimated to range between 3 per 100,000 and 7 per 100,000. […] In Memory Clinics, 0.5–2.5% of patients diagnosed with a neurodegenerative disease are estimated to have PPA. The overall impact of PPA in the general population is approximately 1 per 100,000. […] PPA typically begins around the age of 60, with an onset range between 45 and 70 years. The disease duration varies widely, from 4 to 14 years, with an average duration of approximately 8 years. […] Gender prevalence differs depending on the study. Some findings suggest PPA is more frequent in men than in women, with a reported distribution of 66% men and 34% women.

• #17 Frontotemporal Dementia and Frontotemporal Lobar Degeneration: Overview, Etiology, Genetic Distribution and Variation

  https://emedicine.medscape.com/article/1135164-overview

  The exact prevalence of frontotemporal lobe dementia (FTD) is unknown. Among patients presenting with dementia who are younger than 65 years, the prevalence may be similar to or greater than that of Alzheimer disease. Some series based on brain pathology have estimated that FTD is responsible for as many as 10%20% of cases of dementia. In the United States, estimates are generally lower; FTD ranks after Alzheimer disease, vascular dementia, and Lewy body dementia in frequency of dementing illnesses. […] Studies from Lund, Sweden and Manchester, England estimated that FTD accounts for approximately 8% of patients with dementia. Probably the most accurate information comes from the Dutch study by Stevens et al, cited earlier, who reported 74 cases in a population of 15 million (ie, 5 cases per million persons). Among individuals aged 60-70 years, the prevalence was 28 cases per 100,000.

• #18 Primary Progressive Aphasia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563145/

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. Men and women are equally affected, with an average age of about 60. No demographic, environmental, or socioeconomic risk factors have been identified. Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #19 Primary Progressive Aphasia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/82030

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. […] A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. […] The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. […] Men and women are equally affected, with an average age of about 60. […] No demographic, environmental, or socioeconomic risk factors have been identified. […] Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. […] A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #20 Primary Progressive Aphasia – MD Searchlight

  https://mdsearchlight.com/neurology/primary-progressive-aphasia/

  Primary Progressive Aphasia (PPA) is quite rare, affecting roughly 3 to 4 people out of every 100,000. […] A study showed it occurs about 1.14 times per 100,000 person-years, which is significantly lower than the 35.7 times for Alzheimer’s disease. […] PPA isn’t more common in either sex; it affects both men and women equally, typically around the age of 60. […] It’s important to note that there are no known risk factors tied to demographics, the environment, or socioeconomic status.

• #21 Frontotemporal Dementia and Frontotemporal Lobar Degeneration: Overview, Etiology, Genetic Distribution and Variation

  https://emedicine.medscape.com/article/1135164-overview

  FTD can develop at almost any age in either gender. A large case series compiled by Westbury and Bub investigated 112 published cases prior to 1997; males and females accounted for 66% and 34% of the cases, respectively. Most patients with FTD present in their 50s or 60s. In the review by Westbury and Bub, the mean age of onset was 59 years; the median age was 64 years.

• #22 Primary Progressive Aphasia: Symptoms, Types, Treatment

  https://www.verywellhealth.com/primary-progressive-aphasia-98742

  PPA is classified as a rare disease, however, many may be undiagnosed since they might not seek medical help or be misdiagnosed due to the unfamiliarity with PPA. […] Interestingly, men develop PPA about twice as often as women. The average age of onset is between 50 and 70. Those who get PPA are more likely to have a relative with some type of neurological problem. […] The typical life expectancy from the onset of the disease is 3 to 12 years. Often, complications from PPA, such as swallowing difficulties, lead to the eventual decline.

• #23 Primary Progressive Aphasia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563145/

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. Men and women are equally affected, with an average age of about 60. No demographic, environmental, or socioeconomic risk factors have been identified. Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #24 Primary Progressive Aphasia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/82030

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. […] A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. […] The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. […] Men and women are equally affected, with an average age of about 60. […] No demographic, environmental, or socioeconomic risk factors have been identified. […] Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. […] A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #25 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898

  Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. […] We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. […] While its frequency in the general population is unknown, it can be extrapolated from available data on patients with frontotemporal dementia (FTD). The prevalence of FTD is calculated as approximately 5 cases per million inhabitants with between 1 and 15 cases per 100000 inhabitants in individuals younger than 65 years. PPA accounts for 20% to 40% of these cases. […] The vast majority of PPA cases are sporadic. […] Some patients belong to families with mutations on chromosome 17, either in the gene coding for microtubule-associated protein tau (MAPT) or the gene coding for progranulin.

• #26 Primary Progressive Aphasia – The Brain Clinic

  https://thebrainclinic.com/articles-3/primary-progressive-aphasia/

  Primary progressive aphasia is a rare disorder, and its prevalence in the general population is largely unknown. The overall prevalence of FTD is five cases per million people, 1-15 cases per 100,000 individuals younger than 65, and 0.2-0.3% of individuals over the age of 65. Primary progressive aphasia accounts for 20-40% of all FTD cases. Some studies have found a slightly higher rate in males than females […] Onset typically occurs between the ages of 50 and 70 years. An individual may be diagnosed with mild cognitive impairment (MCI) in the beginning stages of the illness when the deficits do not substantially interfere with daily life. MCI is a dynamic, transitional stage between normal cognitive status (compared to others of similar age and educational background) and dementia. A person is diagnosed with primary progressive aphasia if their area of deficit is confined to the language domain for at least two years. The median disease duration for primary progressive aphasia is 7-10 years.

• #27 Primary Progressive Aphasia: Symptoms, Diagnosis, Treatment, and More

  https://www.healthline.com/health/primary-progressive-aphasia

  Its most common to be diagnosed with primary progressive aphasia when youre between the ages of 50 and 70. […] Primary progressive aphasia is a progressive condition without an exact cause. […] There are currently no medications that can treat the symptoms of primary progressive aphasia. […] Primary progressive aphasia is a condition caused by brain atrophy that results in a loss of communication and language skills. The condition is rare and is linked to dementia and Alzheimers disease.

• #28 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  PPA cases have been reported globally, with no clear geographic clustering. Studies show that PPA affects diverse populations, although prevalence estimates are similar across regions, which are estimated to range between 3 per 100,000 and 7 per 100,000. […] In Memory Clinics, 0.5–2.5% of patients diagnosed with a neurodegenerative disease are estimated to have PPA. The overall impact of PPA in the general population is approximately 1 per 100,000. […] PPA typically begins around the age of 60, with an onset range between 45 and 70 years. The disease duration varies widely, from 4 to 14 years, with an average duration of approximately 8 years. […] Gender prevalence differs depending on the study. Some findings suggest PPA is more frequent in men than in women, with a reported distribution of 66% men and 34% women.

• #29 Primary Progressive Aphasia – The Brain Clinic

  https://thebrainclinic.com/articles-3/primary-progressive-aphasia/

  Primary progressive aphasia is a rare disorder, and its prevalence in the general population is largely unknown. The overall prevalence of FTD is five cases per million people, 1-15 cases per 100,000 individuals younger than 65, and 0.2-0.3% of individuals over the age of 65. Primary progressive aphasia accounts for 20-40% of all FTD cases. Some studies have found a slightly higher rate in males than females […] Onset typically occurs between the ages of 50 and 70 years. An individual may be diagnosed with mild cognitive impairment (MCI) in the beginning stages of the illness when the deficits do not substantially interfere with daily life. MCI is a dynamic, transitional stage between normal cognitive status (compared to others of similar age and educational background) and dementia. A person is diagnosed with primary progressive aphasia if their area of deficit is confined to the language domain for at least two years. The median disease duration for primary progressive aphasia is 7-10 years.

• #30 Primary Progressive Aphasia – What is PPA?

  https://tactustherapy.com/what-is-primary-progressive-aphasia-ppa/

  While the exact number of people with PPA is unknown, it is classified as a rare neurological disorder, meaning it affects fewer than 200,000 people in the US. […] A diagnosis of PPA requires at least two years of relatively isolated language impairment with generally intact functioning in other cognitive abilities (e.g. episodic memory, visuospatial skills, reasoning, and comportment). […] PPA is typically a component of a greater underlying neurodegenerative disease such as Alzheimers disease (AD) or Frontotemporal Lobar Degeneration (FTLD). […] There can be a genetic component to PPA, but most cases are not hereditary. […] PPA is a degenerative condition that gets worse over time with no known cure. Treatments serve to slow the progression of the disease and improve quality of life.

• #31 Primary Progressive Aphasia – The Brain Clinic

  https://thebrainclinic.com/articles-3/primary-progressive-aphasia/

  Primary progressive aphasia is a rare disorder, and its prevalence in the general population is largely unknown. The overall prevalence of FTD is five cases per million people, 1-15 cases per 100,000 individuals younger than 65, and 0.2-0.3% of individuals over the age of 65. Primary progressive aphasia accounts for 20-40% of all FTD cases. Some studies have found a slightly higher rate in males than females […] Onset typically occurs between the ages of 50 and 70 years. An individual may be diagnosed with mild cognitive impairment (MCI) in the beginning stages of the illness when the deficits do not substantially interfere with daily life. MCI is a dynamic, transitional stage between normal cognitive status (compared to others of similar age and educational background) and dementia. A person is diagnosed with primary progressive aphasia if their area of deficit is confined to the language domain for at least two years. The median disease duration for primary progressive aphasia is 7-10 years.

• #32 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  Primary Progressive Aphasias (PPAs) are rare neurodegenerative disorders classified within frontotemporal lobar degeneration (FTLD) and typically manifest between 45 and 70 years of age. […] In Mexico—and many other countries—reliable epidemiological data are lacking; however, estimates suggest that PPA accounts for 0.5–2.5% of neurodegenerative disease cases in Memory Clinics, with an incidence of approximately 1 per 100,000 and an average survival of 8 years. […] PPA is estimated to affect 0.5–2.5% of patients with neurodegenerative diseases in Memory Clinics, with an incidence of roughly 1 per 100,000. Average survival time is around 8 years (ranging from 3 to 17 years), with a generally balanced gender ratio, though some studies indicate a predominance of men. […] A positive family history is observed in 20–40% of cases, with about 10% following an autosomal dominant inheritance pattern.

• #33 Life Expectancy with Primary Progressive Aphasia

  https://www.healthline.com/health/how-long-does-someone-live-with-primary-progressive-aphasia

  Limited research suggests that those with primary progressive aphasia (PPA) live on average from 7 to 12 years after symptoms onset. […] PPA survival rates vary depending on the type you’re diagnosed with, and they’re based on extremely limited research. Current estimates vary between 7 to 12 years after symptoms initially appear. […] Keep in mind that the leading causes of death of patients with PPA include cardiac arrest and pneumonia, not the disorder itself. […] According to limited 2021 research of just 34 patients, the leading causes of death for those with PPA were: natural cardiac arrest (26%), pneumonia (24%), cachexia (15%), bedsore infections (12%). […] Though PPA doesn’t directly cause death, research suggests that the survival timeline after onset is anywhere from 7 to 12 years. […] In a small sample, cardiac arrest and pneumonia were the leading causes of death in those with PPA.

• #34 Primary Progressive Aphasia: Symptoms, Diagnosis, Treatment, and More

  https://www.healthline.com/health/primary-progressive-aphasia

  Primary progressive aphasia is a rare type of aphasia that is caused by brain atrophy. Its associated with dementia. […] Primary progressive aphasia is a rare condition that causes communication difficulties. Its linked to frontotemporal dementia and Alzheimers disease. […] More research into the exact reasons some people develop primary progressive aphasia and other conditions related to brain atrophy, such as dementia and seizure disorders, is still needed. […] For most people with primary progressive aphasia, the cause is unknown. However, there are a few known risk factors, including inherited genetic mutations. […] The early stages of primary progressive aphasia generally lasts around 2 years. […] Some research suggests the subtype of primary progressive aphasia can have an effect on life span after diagnosis. However, primary progressive aphasia is rare, and sample sizes are small.

• #35 A Case of Primary Progressive Aphasia Progressing to Severe Dementia and Death | Consultant360

  https://www.consultant360.com/articles/case-primary-progressive-aphasia-progressing-severe-dementia-and-death

  Primary progressive aphasia (PPA) is a rare neurodegenerative disorder characterized by an isolated and gradual dissolution of language function that progresses to dementia. […] The condition begins in middle age in the form of language difficulties. […] PPA may interfere with the ability to memorize word lists or solve reasoning tasks; the patient typically has no difficulty recalling daily events or behaving with sound judgment, indicating that explicit memory, as well as social skills, remain intact at diagnosis. […] PPA should be differentiated from AD. Patients with AD seek medical attention because of forgetfulness, usually accompanied by apathy. […] People with PPA can become mute and may eventually lose the ability to understand written and spoken language. This generally happens within 10 years of diagnosis.

• #36 SSA – POMS: DI 23022.485 – Primary Progressive Aphasia – 09/06/2022

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022485

  Primary Progressive Aphasia (PPA) is a rare type of dementia characterized by slow and gradual loss of language (aphasia). […] Symptoms of PPA begin gradually, usually before the age of 65 years, and worsen over time. Individuals with PPA may become mute and eventually lose the ability to understand spoken or written language within 10 years of diagnosis. […] There is currently no effective treatment that can cure or slow the progression of PPA.

• #37 Primary Progressive Aphasia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563145/

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. Men and women are equally affected, with an average age of about 60. No demographic, environmental, or socioeconomic risk factors have been identified. Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #38 Primary Progressive Aphasia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/82030

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. […] A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. […] The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. […] Men and women are equally affected, with an average age of about 60. […] No demographic, environmental, or socioeconomic risk factors have been identified. […] Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. […] A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #39 Primary Progressive Aphasia – MD Searchlight

  https://mdsearchlight.com/neurology/primary-progressive-aphasia/

  Primary Progressive Aphasia (PPA) is quite rare, affecting roughly 3 to 4 people out of every 100,000. […] A study showed it occurs about 1.14 times per 100,000 person-years, which is significantly lower than the 35.7 times for Alzheimer’s disease. […] PPA isn’t more common in either sex; it affects both men and women equally, typically around the age of 60. […] It’s important to note that there are no known risk factors tied to demographics, the environment, or socioeconomic status.

• #40 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  Primary Progressive Aphasias (PPAs) are rare neurodegenerative disorders classified within frontotemporal lobar degeneration (FTLD) and typically manifest between 45 and 70 years of age. […] In Mexico—and many other countries—reliable epidemiological data are lacking; however, estimates suggest that PPA accounts for 0.5–2.5% of neurodegenerative disease cases in Memory Clinics, with an incidence of approximately 1 per 100,000 and an average survival of 8 years. […] PPA is estimated to affect 0.5–2.5% of patients with neurodegenerative diseases in Memory Clinics, with an incidence of roughly 1 per 100,000. Average survival time is around 8 years (ranging from 3 to 17 years), with a generally balanced gender ratio, though some studies indicate a predominance of men. […] A positive family history is observed in 20–40% of cases, with about 10% following an autosomal dominant inheritance pattern.

• #41 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  A positive family history of neurodegenerative diseases is reported in 20–40% of PPA patients, suggesting a role for genetic factors. […] The limited representation of non-Caucasian populations in PPA research highlights the need for broader, more inclusive epidemiological studies to clarify whether any demographic or racial disparities exist.

• #42 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898?redirectNew=true

  Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. […] We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. […] While its frequency in the general population is unknown, it can be extrapolated from available data on patients with frontotemporal dementia (FTD). The prevalence of FTD is calculated as approximately 5 cases per million inhabitants with between 1 and 15 cases per 100000 inhabitants in individuals younger than 65 years. PPA accounts for 20% to 40% of these cases. […] The vast majority of PPA cases are sporadic. […] Some patients belong to families with mutations on chromosome 17, either in the gene coding for microtubule-associated protein tau (MAPT) or the gene coding for progranulin.

• #43 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  Primary Progressive Aphasias (PPAs) are rare neurodegenerative disorders classified within frontotemporal lobar degeneration (FTLD) and typically manifest between 45 and 70 years of age. […] In Mexico—and many other countries—reliable epidemiological data are lacking; however, estimates suggest that PPA accounts for 0.5–2.5% of neurodegenerative disease cases in Memory Clinics, with an incidence of approximately 1 per 100,000 and an average survival of 8 years. […] PPA is estimated to affect 0.5–2.5% of patients with neurodegenerative diseases in Memory Clinics, with an incidence of roughly 1 per 100,000. Average survival time is around 8 years (ranging from 3 to 17 years), with a generally balanced gender ratio, though some studies indicate a predominance of men. […] A positive family history is observed in 20–40% of cases, with about 10% following an autosomal dominant inheritance pattern.

• #44 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898

  Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. […] We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. […] While its frequency in the general population is unknown, it can be extrapolated from available data on patients with frontotemporal dementia (FTD). The prevalence of FTD is calculated as approximately 5 cases per million inhabitants with between 1 and 15 cases per 100000 inhabitants in individuals younger than 65 years. PPA accounts for 20% to 40% of these cases. […] The vast majority of PPA cases are sporadic. […] Some patients belong to families with mutations on chromosome 17, either in the gene coding for microtubule-associated protein tau (MAPT) or the gene coding for progranulin.

• #45 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898?redirectNew=true

  Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. […] We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. […] While its frequency in the general population is unknown, it can be extrapolated from available data on patients with frontotemporal dementia (FTD). The prevalence of FTD is calculated as approximately 5 cases per million inhabitants with between 1 and 15 cases per 100000 inhabitants in individuals younger than 65 years. PPA accounts for 20% to 40% of these cases. […] The vast majority of PPA cases are sporadic. […] Some patients belong to families with mutations on chromosome 17, either in the gene coding for microtubule-associated protein tau (MAPT) or the gene coding for progranulin.

• #46 Primary progressive aphasia – Wikipedia

  https://en.wikipedia.org/wiki/Primary_progressive_aphasia

  There have been no large epidemiological studies on the incidence and prevalence of the PPA variants. […] Though it most likely has been underestimated, onset of PPA has been found to occur in the sixth or seventh decade. […] PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration (FTLD), including PPA, are at slightly greater risk of developing PPA or another form of the condition. […] In a quarter of patients diagnosed with PPA, there is a family history of PPA or one of the other disorders in the FTLD spectrum of disorders. […] It has been found that genetic predisposition varies among the different PPA variants, with progressive nonfluent aphasia (PNFA) being more commonly familial in nature than semantic dementia (SD).

• #47 Primary progressive aphasia – Wikipedia

  https://en.wikipedia.org/wiki/Primary_progressive_aphasia

  There have been no large epidemiological studies on the incidence and prevalence of the PPA variants. […] Though it most likely has been underestimated, onset of PPA has been found to occur in the sixth or seventh decade. […] PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration (FTLD), including PPA, are at slightly greater risk of developing PPA or another form of the condition. […] In a quarter of patients diagnosed with PPA, there is a family history of PPA or one of the other disorders in the FTLD spectrum of disorders. […] It has been found that genetic predisposition varies among the different PPA variants, with progressive nonfluent aphasia (PNFA) being more commonly familial in nature than semantic dementia (SD).

• #48 Primary progressive aphasia – Wikipedia

  https://en.wikipedia.org/wiki/Primary_progressive_aphasia

  There have been no large epidemiological studies on the incidence and prevalence of the PPA variants. […] Though it most likely has been underestimated, onset of PPA has been found to occur in the sixth or seventh decade. […] PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration (FTLD), including PPA, are at slightly greater risk of developing PPA or another form of the condition. […] In a quarter of patients diagnosed with PPA, there is a family history of PPA or one of the other disorders in the FTLD spectrum of disorders. […] It has been found that genetic predisposition varies among the different PPA variants, with progressive nonfluent aphasia (PNFA) being more commonly familial in nature than semantic dementia (SD).

• #49 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898

  A higher rate of vasectomies has also been measured in this population, which might suggest an autoimmune basis. […] However, the above associations have not been found by other studies. […] PPA is an emerging syndrome which may be more prevalent than we might expect. […] Clinical diagnosis, complemented by a biomarker evaluation, may predict the underlying pathology, which in turn will improve treatment possibilities.

• #50 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898?redirectNew=true

  A higher rate of vasectomies has also been measured in this population, which might suggest an autoimmune basis. […] However, the above associations have not been found by other studies. […] Although the clinical variants are defined according to aphasia semiology, there are other associated symptoms that can help provide a diagnosis. […] PPA is an emerging syndrome which may be more prevalent than we might expect. It was previously listed as part of the frontotemporal dementia spectrum, and it is also related to Alzheimer disease. […] Clinical diagnosis, complemented by a biomarker evaluation, may predict the underlying pathology, which in turn will improve treatment possibilities.

• #51 A Case of Primary Progressive Aphasia Progressing to Severe Dementia and Death | Consultant360

  https://www.consultant360.com/articles/case-primary-progressive-aphasia-progressing-severe-dementia-and-death

  Learning disabilities and certain gene mutations are 2 risk factors for PPA. […] The diagnosis of PPA is made primarily by clinical assessment. […] The defining feature of all PPA variants is a progressive language disorder that emerges as the principal feature of the initial clinical picture. […] Studies are being done with cerebral spinal fluid (CSF) biomarkers to help classify PPA. […] There are no medications that specifically treat PPA. […] PPA is a focal dementia characterized by an isolated and gradual dissolution of language function in many ways. […] Differentiating the diagnosis of AD and PPA is essential through careful clinical assessment. […] There is no treatment at this time for PPA; studies are being done with CSF and biomarkers to help classify PPA. […] An interdisciplinary team approach to care is essential in supporting the patient and family members.

• #52 Primary progressive aphasia | Tidsskrift for Den norske legeforening

  https://tidsskriftet.no/en/2022/11/clinical-review/primary-progressive-aphasia

  Neurodegenerative dementia may, in rare cases, initially manifest as isolated language impairments in the absence of other cognitive symptoms. The prevalence of primary progressive aphasia is estimated to be approximately 34 per 100,000 individuals, i.e., about the same as amyotrophic lateral sclerosis. In a recent registry study, the incidence of primary progressive aphasia (all types) was estimated to be 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for typical Alzheimer’s dementia. […] The purpose of this article is to provide a brief overview of the different variants of primary progressive aphasia. […] Knowledge of the conditions, detailed analysis of diagnostic imaging and the use of targeted cognitive tests to assess speech and language are important for making the correct diagnosis. Patients with primary progressive aphasia are often initially misdiagnosed, for example, their aphasia may be misattributed to a stroke. If the initial workup suggests a language disorder as part of a degenerative dementia, the patient should be referred to the specialist healthcare service for further testing. Speech and language assessment may be necessary to obtain the correct diagnosis, and speech and language therapy can be effective when targeted at the specific difficulties experienced by the patient.

• #53 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Text

  https://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6

  The goal of the present study, based on a French cohort, was to describe the demographics and the evolution of subjects with (PPA) in comparison with Alzheimers disease (AD) on a period of 7 years. […] The incidence rate of PPA was 1.14 per 100,000 person-years, while the incidence rate of AD was 35.7 per 100,000 persons-years. […] This study improves the knowledge of PPA epidemiology and has the potential to help adopting appropriate public health service policies. […] Our secondary objectives were to determine if diagnosis of PPA is more difficult to establish and more delayed than AD and what are the related prescribed treatment over 7 years of follow-up. […] The delay between the first consultation for cognitive troubles and the first diagnosis that is significantly longer than in the AD group.

• #54 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Text

  https://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6

  The number of different diagnoses before the diagnosis of interest that is significantly higher in the PPA group than in the AD group. […] To summarize, despite the evolution of the diagnostic criteria of primary progressive aphasia over time, their diagnostic still does not seem optimal. […] Our study shows also that PPA diagnosis is particularly important because it modifies pharmacological and nonpharmacological interventions. […] Indeed, the number of patients under pharmacological treatment increases of more than 50% after the diagnosis of PPA. […] This study highlights importance to improve early diagnosis of PPA to better understand links with AD.

• #55 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Text

  https://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6

  The number of different diagnoses before the diagnosis of interest that is significantly higher in the PPA group than in the AD group. […] To summarize, despite the evolution of the diagnostic criteria of primary progressive aphasia over time, their diagnostic still does not seem optimal. […] Our study shows also that PPA diagnosis is particularly important because it modifies pharmacological and nonpharmacological interventions. […] Indeed, the number of patients under pharmacological treatment increases of more than 50% after the diagnosis of PPA. […] This study highlights importance to improve early diagnosis of PPA to better understand links with AD.

• #56

  https://link.springer.com/article/10.1007/s00415-018-8762-6

  The primary progressive aphasias (PPA) are a diverse group of disorders that collectively present with relatively focal degeneration of the brain systems that govern language. […] PPA is uncommon (estimated prevalence is conservatively around three cases per 100,000), the underlying pathology is heterogeneous and generally inaccessible and the functions principally targeted are uniquely complex. […] Accurate and early identification of PPA syndromes is essential for clinical counselling and planning appropriate management. […] Most patients presenting with a language complaint will not have PPA and further, a number of patients with PPA (as many as 40% in some series) do not conform closely to one of the canonical syndromic diagnoses. […] Structural brain imaging (ideally, MRI) is essential in all cases of suspected PPA, both to rule out other causes of progressive language failure and more positively, to identify features of particular radiological phenotypes. […] In most cases, lvPPA is a variant presentation of Alzheimers disease. […] The pathological associations of nfvPPA are (in keeping with the clinical spectrum) more heterogeneous than other PPA syndromes.

• #57

  https://criticalstudycommunicationdisability.org/index.php/jcscd/article/view/24

  Primary progressive aphasia (PPA) is a neurodegenerative disease involving insidious onset language loss in the context of relatively spared cognitive function, where language is the initial and dominant factor impacting activities of daily living. […] Detection and identification of PPA requires a level of interdisciplinary expertise and resources that are not uniformly available across healthcare settings at the local and global levels. […] This potential inequity raises concerns for increased risk of underdiagnosis, misdiagnosis, and/or delayed diagnosis of PPA, in turn impacting access to the required specialty care. […] The current study sought to characterize the PPA population at a Southeastern United States academic medical center, and to examine potential delays in PPA diagnosis and access to speech-language pathology services related to social determinants of health.

• #58

  https://criticalstudycommunicationdisability.org/index.php/jcscd/article/view/24

  Primary progressive aphasia (PPA) is a neurodegenerative disease involving insidious onset language loss in the context of relatively spared cognitive function, where language is the initial and dominant factor impacting activities of daily living. […] Detection and identification of PPA requires a level of interdisciplinary expertise and resources that are not uniformly available across healthcare settings at the local and global levels. […] This potential inequity raises concerns for increased risk of underdiagnosis, misdiagnosis, and/or delayed diagnosis of PPA, in turn impacting access to the required specialty care. […] The current study sought to characterize the PPA population at a Southeastern United States academic medical center, and to examine potential delays in PPA diagnosis and access to speech-language pathology services related to social determinants of health.

• #59

  https://criticalstudycommunicationdisability.org/index.php/jcscd/article/view/24

  We hypothesized that individuals from minoritized and otherwise disenfranchised groups would have longer duration of time between first reported symptom, and PPA diagnosis. […] Further, we hypothesized that these groups would be less likely to be referred to speech-language pathology services. […] A discussion of PPA and related disorders as they relate to social determinants of health follows.

• #60 Primary progressive aphasia research updates – UChicago Medicine

  https://www.uchicagomedicine.org/forefront/neurosciences-articles/2024/november/primary-progressive-aphasia-papers-november-2024

  PPA is a relatively rare dementia, said Emily Rogalski, PhD, the Rosalind Franklin PhD Professor of Neurology at UChicago and a leading researcher in the field. It’s often overlooked in the literature because it can be difficult to assemble large groups of people to survey lived experiences. […] She said its frequently overlooked by medical professionals as well, going undiagnosed particularly among those with lower socioeconomic status. […] Lack of diagnosis can be such a barrier to care for anyone not living next to a specialized medical center, she said. […] Even while working to establish objective measurements of PPAs impact, Rogalski and her colleagues were simultaneously enrolling patients in a clinical trial exploring the feasibility of delivering speech-language therapy for PPA via telemedicine. […] This success also paves the way for future research and interventions and not just for PPA. […] Despite these research advancements, challenges remain in diagnosing and treating PPA, and no drug or therapy that can cure the disease has been developed yet.

• #61 Primary progressive aphasia research updates – UChicago Medicine

  https://www.uchicagomedicine.org/forefront/neurosciences-articles/2024/november/primary-progressive-aphasia-papers-november-2024

  PPA is a relatively rare dementia, said Emily Rogalski, PhD, the Rosalind Franklin PhD Professor of Neurology at UChicago and a leading researcher in the field. It’s often overlooked in the literature because it can be difficult to assemble large groups of people to survey lived experiences. […] She said its frequently overlooked by medical professionals as well, going undiagnosed particularly among those with lower socioeconomic status. […] Lack of diagnosis can be such a barrier to care for anyone not living next to a specialized medical center, she said. […] Even while working to establish objective measurements of PPAs impact, Rogalski and her colleagues were simultaneously enrolling patients in a clinical trial exploring the feasibility of delivering speech-language therapy for PPA via telemedicine. […] This success also paves the way for future research and interventions and not just for PPA. […] Despite these research advancements, challenges remain in diagnosing and treating PPA, and no drug or therapy that can cure the disease has been developed yet.

• #62 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Text

  https://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6

  The number of different diagnoses before the diagnosis of interest that is significantly higher in the PPA group than in the AD group. […] To summarize, despite the evolution of the diagnostic criteria of primary progressive aphasia over time, their diagnostic still does not seem optimal. […] Our study shows also that PPA diagnosis is particularly important because it modifies pharmacological and nonpharmacological interventions. […] Indeed, the number of patients under pharmacological treatment increases of more than 50% after the diagnosis of PPA. […] This study highlights importance to improve early diagnosis of PPA to better understand links with AD.

• #63 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Text

  https://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6

  The number of different diagnoses before the diagnosis of interest that is significantly higher in the PPA group than in the AD group. […] To summarize, despite the evolution of the diagnostic criteria of primary progressive aphasia over time, their diagnostic still does not seem optimal. […] Our study shows also that PPA diagnosis is particularly important because it modifies pharmacological and nonpharmacological interventions. […] Indeed, the number of patients under pharmacological treatment increases of more than 50% after the diagnosis of PPA. […] This study highlights importance to improve early diagnosis of PPA to better understand links with AD.

• #64 Global perspectives on the management of primary progressive aphasia | Scientific Reports

  https://www.nature.com/articles/s41598-024-70156-5

  Evidence demonstrates that PwPPA must begin speech and language intervention as early as possible to avoid the risk of not benefitting from impairment-based or functional interventions. […] Furthermore, as highlighted by Swaffer (2019), people living with early-onset dementias, such as PPA, and their families seek support to optimize participation and quality of life outcomes, which is bolstered by appropriate and timely access to SLT/P intervention. […] In response to these concerns, a group of international expert clinician-academic SLT/Ps worked collaboratively to establish best-practice principles to guide SLT/P services for PwPPA and their families. […] The principles provide a framework for the essential components of SLT/P intervention and can be used to guide clinical decision-making, care coordination and service development in the SLT/P field.

• #65 Global perspectives on the management of primary progressive aphasia | Scientific Reports

  https://www.nature.com/articles/s41598-024-70156-5

  Evidence demonstrates that PwPPA must begin speech and language intervention as early as possible to avoid the risk of not benefitting from impairment-based or functional interventions. […] Furthermore, as highlighted by Swaffer (2019), people living with early-onset dementias, such as PPA, and their families seek support to optimize participation and quality of life outcomes, which is bolstered by appropriate and timely access to SLT/P intervention. […] In response to these concerns, a group of international expert clinician-academic SLT/Ps worked collaboratively to establish best-practice principles to guide SLT/P services for PwPPA and their families. […] The principles provide a framework for the essential components of SLT/P intervention and can be used to guide clinical decision-making, care coordination and service development in the SLT/P field.

• #66 Global perspectives on the management of primary progressive aphasia | Scientific Reports

  https://www.nature.com/articles/s41598-024-70156-5

  Speech-language therapists/pathologists (SLT/Ps) are key professionals in the management and treatment of primary progressive aphasia (PPA), however, there are gaps in education and training within the discipline, with implications for skills, confidence, and clinical decision-making. […] This is the first international survey to comprehensively explore the perspectives of SLT/Ps working with PwPPA. […] Based on survey outcomes, there is a pressing need to enhance current educational and ongoing training opportunities to better promote the well-being of PwPPA and their families, and to ensure appropriate preparation of the current and future SLT/P workforce. […] Despite this need, there remains a consequential gap in the number of people living with dementia globally who qualify for speech and language therapy and those that receive this service.

• #67 Global perspectives on the management of primary progressive aphasia | Scientific Reports

  https://www.nature.com/articles/s41598-024-70156-5

  Evidence demonstrates that PwPPA must begin speech and language intervention as early as possible to avoid the risk of not benefitting from impairment-based or functional interventions. […] Furthermore, as highlighted by Swaffer (2019), people living with early-onset dementias, such as PPA, and their families seek support to optimize participation and quality of life outcomes, which is bolstered by appropriate and timely access to SLT/P intervention. […] In response to these concerns, a group of international expert clinician-academic SLT/Ps worked collaboratively to establish best-practice principles to guide SLT/P services for PwPPA and their families. […] The principles provide a framework for the essential components of SLT/P intervention and can be used to guide clinical decision-making, care coordination and service development in the SLT/P field.

• #68 A Case of Primary Progressive Aphasia Progressing to Severe Dementia and Death | Consultant360

  https://www.consultant360.com/articles/case-primary-progressive-aphasia-progressing-severe-dementia-and-death

  Learning disabilities and certain gene mutations are 2 risk factors for PPA. […] The diagnosis of PPA is made primarily by clinical assessment. […] The defining feature of all PPA variants is a progressive language disorder that emerges as the principal feature of the initial clinical picture. […] Studies are being done with cerebral spinal fluid (CSF) biomarkers to help classify PPA. […] There are no medications that specifically treat PPA. […] PPA is a focal dementia characterized by an isolated and gradual dissolution of language function in many ways. […] Differentiating the diagnosis of AD and PPA is essential through careful clinical assessment. […] There is no treatment at this time for PPA; studies are being done with CSF and biomarkers to help classify PPA. […] An interdisciplinary team approach to care is essential in supporting the patient and family members.

• #69 Primary progressive aphasia research updates – UChicago Medicine

  https://www.uchicagomedicine.org/forefront/neurosciences-articles/2024/november/primary-progressive-aphasia-papers-november-2024

  PPA is a relatively rare dementia, said Emily Rogalski, PhD, the Rosalind Franklin PhD Professor of Neurology at UChicago and a leading researcher in the field. It’s often overlooked in the literature because it can be difficult to assemble large groups of people to survey lived experiences. […] She said its frequently overlooked by medical professionals as well, going undiagnosed particularly among those with lower socioeconomic status. […] Lack of diagnosis can be such a barrier to care for anyone not living next to a specialized medical center, she said. […] Even while working to establish objective measurements of PPAs impact, Rogalski and her colleagues were simultaneously enrolling patients in a clinical trial exploring the feasibility of delivering speech-language therapy for PPA via telemedicine. […] This success also paves the way for future research and interventions and not just for PPA. […] Despite these research advancements, challenges remain in diagnosing and treating PPA, and no drug or therapy that can cure the disease has been developed yet.

• #70 Primary progressive aphasia – Wikipedia

  https://en.wikipedia.org/wiki/Primary_progressive_aphasia

  There have been no large epidemiological studies on the incidence and prevalence of the PPA variants. […] Though it most likely has been underestimated, onset of PPA has been found to occur in the sixth or seventh decade. […] PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration (FTLD), including PPA, are at slightly greater risk of developing PPA or another form of the condition. […] In a quarter of patients diagnosed with PPA, there is a family history of PPA or one of the other disorders in the FTLD spectrum of disorders. […] It has been found that genetic predisposition varies among the different PPA variants, with progressive nonfluent aphasia (PNFA) being more commonly familial in nature than semantic dementia (SD).

• #71 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  A positive family history of neurodegenerative diseases is reported in 20–40% of PPA patients, suggesting a role for genetic factors. […] The limited representation of non-Caucasian populations in PPA research highlights the need for broader, more inclusive epidemiological studies to clarify whether any demographic or racial disparities exist.

• #72 Brain Sciences | Special Issue : Advances in Primary Progressive Aphasia

  https://www.mdpi.com/journal/brainsci/special_issues/Primary_Progressive_Aphasia

  Primary progressive aphasia is a clinical syndrome that includes a group of neurodegenerative disorders characterized by progressive language impairment. […] further data about epidemiology and natural history of the diseases are needed; and, as in other neurodegenerative disorders, successful therapies are lacking. […] We invite scholars to submit original research studies and reviews regarding all possible aspects of primary progressive aphasia, from basic science, epidemiology, clinical findings, longitudinal course, language and speech features, validation of neuropsychological tools for diagnosis, neuroimaging, blood and cerebrospinal fluid biomarkers, pathology, and treatments.

• #73 Brain Sciences | Special Issue : Advances in Primary Progressive Aphasia

  https://www.mdpi.com/journal/brainsci/special_issues/Primary_Progressive_Aphasia

  Primary progressive aphasia is a clinical syndrome that includes a group of neurodegenerative disorders characterized by progressive language impairment. […] further data about epidemiology and natural history of the diseases are needed; and, as in other neurodegenerative disorders, successful therapies are lacking. […] We invite scholars to submit original research studies and reviews regarding all possible aspects of primary progressive aphasia, from basic science, epidemiology, clinical findings, longitudinal course, language and speech features, validation of neuropsychological tools for diagnosis, neuroimaging, blood and cerebrospinal fluid biomarkers, pathology, and treatments.

• #74 Primary progressive aphasia research updates – UChicago Medicine

  https://www.uchicagomedicine.org/forefront/neurosciences-articles/2024/november/primary-progressive-aphasia-papers-november-2024

  PPA is a relatively rare dementia, said Emily Rogalski, PhD, the Rosalind Franklin PhD Professor of Neurology at UChicago and a leading researcher in the field. It’s often overlooked in the literature because it can be difficult to assemble large groups of people to survey lived experiences. […] She said its frequently overlooked by medical professionals as well, going undiagnosed particularly among those with lower socioeconomic status. […] Lack of diagnosis can be such a barrier to care for anyone not living next to a specialized medical center, she said. […] Even while working to establish objective measurements of PPAs impact, Rogalski and her colleagues were simultaneously enrolling patients in a clinical trial exploring the feasibility of delivering speech-language therapy for PPA via telemedicine. […] This success also paves the way for future research and interventions and not just for PPA. […] Despite these research advancements, challenges remain in diagnosing and treating PPA, and no drug or therapy that can cure the disease has been developed yet.

• #75 Primary Progressive Aphasia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563145/

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. Men and women are equally affected, with an average age of about 60. No demographic, environmental, or socioeconomic risk factors have been identified. Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #76 Incidence of Primary Progressive Apraxia of Speech and Primary Progressive Aphasia in Olmsted County, MN, 2011-2022 – PubMed

  https://pubmed.ncbi.nlm.nih.gov/39079073/

  No epidemiologic studies have formally assessed the incidence of primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS). […] The incidence of PPA + PPAOS was 0.70 persons per 100,000 person-years (95% CI 0.34-1.29 persons per 100,000) during the study period. The incidence of PPAOS was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55 persons per 100,000), whereas for the 8 patients with PPA, the incidence was 0.56 persons per 100,000 person-years (95% CI 0.24-1.10 cases per 100,000). The incidence of nfvPPA was 0.14 persons per 100,000 person-years (95% CI 0.02-0.55), 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for lvPPA, and 0.21 persons per 100,000 person-years (95% CI 0.04-0.61) for svPPA. […] As a group, PPA and PPAOS are a relatively rare group of diseases. PPAOS has a slightly lower incidence than PPA as a group but similar incidence to the individual PPA variants.

• #77 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Text

  https://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6

  The goal of the present study, based on a French cohort, was to describe the demographics and the evolution of subjects with (PPA) in comparison with Alzheimers disease (AD) on a period of 7 years. […] The incidence rate of PPA was 1.14 per 100,000 person-years, while the incidence rate of AD was 35.7 per 100,000 persons-years. […] This study improves the knowledge of PPA epidemiology and has the potential to help adopting appropriate public health service policies. […] Our secondary objectives were to determine if diagnosis of PPA is more difficult to establish and more delayed than AD and what are the related prescribed treatment over 7 years of follow-up. […] The delay between the first consultation for cognitive troubles and the first diagnosis that is significantly longer than in the AD group.

• #78 Primary Progressive Aphasia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/82030

  PPA’s prevalence is estimated to be 3 to 4 per 100,000 population. […] A recent registry study revealed that this condition’s incidence was around 1.14 per 100,000 person-years, compared with 35.7 per 100,000 person-years for Alzheimer disease. […] The nfvPPA’s prevalence is approximately 0.5 to 3.9 per 100,000 people. […] Men and women are equally affected, with an average age of about 60. […] No demographic, environmental, or socioeconomic risk factors have been identified. […] Approximately 56% to 86% of individuals with PPA have coexisting speech apraxia. […] A recent study showed that bvFTD was more common in men, whereas PPA was more common in women.

• #79 Primary progressive aphasia: From syndrome to disease | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-primary-progressive-aphasia-from-syndrome-S2173580813000898

  Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. […] We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. […] While its frequency in the general population is unknown, it can be extrapolated from available data on patients with frontotemporal dementia (FTD). The prevalence of FTD is calculated as approximately 5 cases per million inhabitants with between 1 and 15 cases per 100000 inhabitants in individuals younger than 65 years. PPA accounts for 20% to 40% of these cases. […] The vast majority of PPA cases are sporadic. […] Some patients belong to families with mutations on chromosome 17, either in the gene coding for microtubule-associated protein tau (MAPT) or the gene coding for progranulin.

• #80 Primary Progressive Aphasias: Diagnosis and Treatment

  https://www.mdpi.com/2076-3425/15/3/245

  Primary Progressive Aphasias (PPAs) are rare neurodegenerative disorders classified within frontotemporal lobar degeneration (FTLD) and typically manifest between 45 and 70 years of age. […] In Mexico—and many other countries—reliable epidemiological data are lacking; however, estimates suggest that PPA accounts for 0.5–2.5% of neurodegenerative disease cases in Memory Clinics, with an incidence of approximately 1 per 100,000 and an average survival of 8 years. […] PPA is estimated to affect 0.5–2.5% of patients with neurodegenerative diseases in Memory Clinics, with an incidence of roughly 1 per 100,000. Average survival time is around 8 years (ranging from 3 to 17 years), with a generally balanced gender ratio, though some studies indicate a predominance of men. […] A positive family history is observed in 20–40% of cases, with about 10% following an autosomal dominant inheritance pattern.

• #81 Top Published Expert Doctors for Primary Progressive Aphasia

  https://findexpertmd.com/d/Primary_Progressive_Aphasia

  330 top medical experts on Primary Progressive Aphasia across 28 countries and 23 U.S. states, including 211 MDs (Physicians). This is based on an objective analysis of their Scientific Publications, Clinical Trials, Medicare, and NIH Grants. […] Clinical Trials ClinicalTrials.gov : at least 192 including 9 Active, 75 Completed, 62 Recruiting.

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