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Alzheimer Disease l: Introduction01:29

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Alzheimer disease is a chronic, progressive, and irreversible neurodegenerative disorder and the most common cause of dementia in older adults. It leads to gradual neuronal loss, causing cognitive decline, behavioral changes, and loss of functional independence.Risk Factors and EtiologyThe disease is multifactorial. Age is the strongest risk factor, with prevalence doubling every 5 years after age 65. Genetic factors include mutations in genes such as APP, PSEN1, and PSEN2, which are associated...
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Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and...
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Dementia is an acquired, progressive syndrome characterized by a decline in multiple cognitive domains severe enough to impair daily functioning and reduce independence. Although memory loss is a central feature, the diagnosis requires additional deficits involving language, executive function, visuospatial skills, judgment, calculation, or abstract reasoning. These cognitive impairments reflect underlying neurodegenerative or vascular processes that gradually disrupt neuronal networks...
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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show...
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Related Experiment Video

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Primary progressive aphasia.

Andrew Kertesz1, Michał Harciarek

  • 1Department of Clinical Neurological Sciences, Western University, London, Ontario, Canada.

Scandinavian Journal of Psychology
|April 11, 2014
PubMed
Summary

Primary progressive aphasia (PPA) involves language decline, classified into nonfluent, semantic, or logopenic variants. Each PPA type presents distinct speech, cognitive, and neuroimaging characteristics.

Area of Science:

  • Neurology
  • Linguistics
  • Neuroscience

Background:

  • Primary progressive aphasia (PPA) is a neurodegenerative disorder causing progressive language impairment.
  • PPA is categorized into three main variants: nonfluent (nfvPPA), semantic (svPPA), and logopenic (lvPPA).
  • Each variant exhibits unique clinical, radiological, and pathological features.

Purpose of the Study:

  • To outline the distinct characteristics of the three primary progressive aphasia variants.
  • To correlate clinical presentations with neuroimaging and neuropathological findings.
  • To provide a comprehensive overview for differential diagnosis and research.

Main Methods:

  • Review of clinical features, including speech production, anomia, and repetition deficits.
Keywords:
Primary progressive aphasiadegenerative diseasefrontotemporal dementia

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  • Analysis of neuroimaging findings, focusing on patterns of brain atrophy.
  • Examination of neuropathological correlates, such as tauopathy and TDP-43.

    • Main Results:

    • Nonfluent variant PPA (nfvPPA) shows effortful speech, agrammatism, apraxia of speech, and left perisylvian/insular atrophy, often linked to tauopathy.
    • Semantic variant PPA (svPPA) presents with fluent but empty speech, anomia, semantic knowledge loss, and anterior temporal lobe atrophy, typically associated with TDP-43 pathology.
    • Logopenic variant PPA (lvPPA) is characterized by word-finding pauses, repetition difficulties, and left temporoparietal atrophy, often associated with Alzheimer's disease pathology.

    Conclusions:

    • The three variants of PPA (nfvPPA, svPPA, lvPPA) are distinguishable by their unique clinical, imaging, and pathological profiles.
    • Understanding these distinctions is crucial for accurate diagnosis and guiding future research.
    • PPA variants represent distinct neurodegenerative pathways affecting language networks differently.