Different clinical presentations and imaging findings of 3 subtypes, agrammatic, semantic and logopenic, are presented. Underlying neuropathology, medical and social management aspects of these patients are reviewed. [ncbi.nlm.nih.gov]

• Vietnamese

  We describe a case of primary progressive aphasia in a Vietnamese man with corresponding changes on positron emission tomography. [ncbi.nlm.nih.gov]

• Choking

  RESULTS: Patients with PPA-S had diverse swallowing problems such as drooling of saliva or food, multiple swallows, delayed swallow and choking, all of which correlated with anxiety, apathy and aberrant motor behavior. [ncbi.nlm.nih.gov]

  He was having trouble swallowing and was choking on his food. He had poor coordination skills and it was hard for him to get food to his mouth. He was forgetting to swallow. [consultant360.com]

• Ejection Murmur

  The clinical examination was normal except for a III/VI systolic ejection murmur, an umbilical hernia that was easily reducible, and an abnormal verbal exam. [consultant360.com]

• Suggestibility

  This neuropsychological evidence suggests that GRN-PPA may result from damage involving the temporo-parietal junction and its functional connections in both the dorsal and ventral language networks, with implications for our understanding of language [ncbi.nlm.nih.gov]

• Behavior Problem

  The third case presented with the syndrome of semantic dementia and showed the typical behavioral problems associated with FTD and a pattern of left-greater-than-right temporal atrophy. [ncbi.nlm.nih.gov]

  About 50 percent of people with PPA will develop cognitive or behavioral problems consistent with a more invasive dementia syndrome, such as Alzheimer's disease or frontotemporal dementia, after an average of five years. [theadvocate.com]

• Postural Instability

  At age 64, he developed non-fluent aphasia that progressed slowly over 8 years, eventually associated with behavioral abnormality, postural instability, and dysphagia at 2 years prior to his death. [ncbi.nlm.nih.gov]

• Poor Coordination

  He had poor coordination skills and it was hard for him to get food to his mouth. He was forgetting to swallow. He was not able to perform his instrumental activities of daily living and needed assistance with his activities of daily living. [consultant360.com]

Brain MRI and FDG-PET/CT were performed as initial imaging workup. [ncbi.nlm.nih.gov]

• Ischemic Changes

  A MRI of the brain in 2006 showed small, scattered areas of increased signal intensity involving the white matter bilaterally, nonspecific in appearance—likely representing small vessel ischemic changes. [consultant360.com]

Language treatment shows promise as a means of addressing these difficulties but much remains to be learned with regard to the potential value of treatment across variants and stages of the disorder. [ncbi.nlm.nih.gov]

These varying factors should be taken into account when considering prognosis, disease management, and selection of outcome measures for interventions. © 2016 S. Karger AG, Basel. [ncbi.nlm.nih.gov]

What is the prognosis for people with PPA? This is a difficult question to answer as there has been very little research into it and it can be extremely variable from person to person. [raredementiasupport.org]

“That will help us with prognosis and telling family members what will happen next,” she says. The semantic variant, for example, sometimes leads to agitation and other behavioral changes. There’s no cure for PPA, but research is underway. [theatlantic.com]

Mika Otsuki, Symptoms and prognosis of PNFA / nfvPPA, Higher Brain Function Research, 35, 3, (297), (2015). Pirada Witoonpanich, Sebastian J. Crutch, Jason D. Warren and Martin N. [dx.doi.org]

Progression and prognosis As in Alzheimer disease, researchers of APP refer to a preclinical, prodromal phase that may be called ‘mild aphasic cognitive impairment’. However, the usefulness of this distinction is not clear. [elsevier.es]

Improved reliability of diagnoses will be increasingly important as trials for etiology-specific treatments become available. We also discuss the implications of these syndromes for theories of language function. [ncbi.nlm.nih.gov]

PPA is not due to neoplastic, vascular, or metabolic etiologies nor to infectious disease (Mesulam, 2001; Rogers, 2004). Return to Glossary [asha.org]

This review of the literature aims to characterize the speech and language impairment, cognition, neuroimaging, pathology, genetics, and epidemiology associated with each of these variants. [ncbi.nlm.nih.gov]

There have been no large epidemiological studies on the incidence and prevalence of the PPA variants. [en.wikipedia.org]

Development 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. [elsevier.es]

These results may provide insight into the basic pathophysiologic mechanisms underlying PPA and related forms of dementia and suggest the existence of novel, rapidly reversible, TNF-mediated pathophysiologic mechanisms in both PPA and Alzheimer's disease [ncbi.nlm.nih.gov]

Author information 1 Cognitive Neurology and Alzheimer's Disease Center, Northwestern University (NU) Feinberg School of Medicine, Chicago, IL. 2 The University of Arizona College of Medicine, Tucson, AZ. 3 NU Feinberg School of Medicine, Department of Preventive [ncbi.nlm.nih.gov]

This, experts say, is because there is no way of repairing or preventing the ongoing injury to the brain. [express.co.uk]

Although there is no medical treatment for PPA, often people will take preventative medications for Alzheimer’s as it is commonly the underlying cause of PPA. [constanttherapy.com]

[…] developing better and more effective language interventions in neurodegenerative diseases and understanding how these interventions work at the neuronal level is particularly important since it may inform us on how to develop better interventions to prevent [scienceoflearning.jhu.edu]

Significance : This study may lead to the identification of the chromosome(s) responsible for a genetically determined language disorder, providing a target for interventions to prevent this disorder. [dana.org]