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Primary Progressive Aphasia

PPA


Presentation

  • This clinical picture pointed to an atypical presentation of the non-fluent variety.[ncbi.nlm.nih.gov]
Italian
  • Abstract Surface dyslexia is a pattern of reading impairment which has been seldom described in Italian native speakers.[ncbi.nlm.nih.gov]
  • Basso, Semantic amnesia without dementia: documentation of a case, The Italian Journal of Neurological Sciences, 10.1007/BF02048486, 18, 3, (167-171), (1997).[dx.doi.org]
  • Spinnler, An Italian series of patients with organic dementia, The Italian Journal of Neurological Sciences, 10.1007/BF02230415, 7, 1, (27-41), (1986). John Wm. Knesevich, The author replies, Psychiatry Research, 16, 3, (265), (1985). George W.[doi.org]
Vietnamese
  • We describe a case of primary progressive aphasia in a Vietnamese man with corresponding changes on positron emission tomography.[ncbi.nlm.nih.gov]
Drooling
  • 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]
Echolalia
  • Echolalia and personality change were prominent whereas parkinsonian features and signs suggesting parietal lobe dysfunctions were not present. The patient's language deficit was consistent with transcortical motor aphasia.[ncbi.nlm.nih.gov]
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]
Headache
  • Cazzato, Headache in dementia, European Journal of Neurology, 8, 1, (93-94), (2001). Andrew B. Newberg, P. David Mozley, Ahmed H.[dx.doi.org]

Workup

Treatment

  • For all tasks, the change in naming accuracy from baseline to 1 month post-treatment was compared between the UC and each treatment condition.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • 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]
  • “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]
  • 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]

Etiology

  • 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]

Epidemiology

  • 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]
  • Epidemiology and Treatment Because the lvPPA has been only recently described (Gorno-Tempini et al. 2004 ), there is still little known about its epidemiology and treatment.[doi.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]
  • Sudlow, Identifying dementia outcomes in UK Biobank: a validation study of primary care, hospital admissions and mortality data, European Journal of Epidemiology, 10.1007/s10654-019-00499-1, (2019).[dx.doi.org]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]

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