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Primary progressive aphasia.

¹Cognitive Neurology and Alzheimer's Disease Center, Department of Neurology, Northwestern University, Chicago, IL 60611, USA. mmesulam@nwu.edu

Annals of Neurology

|April 20, 2001

Summary

Primary progressive aphasia (PPA) is a dementia affecting language. Neuroimaging shows left hemisphere involvement, and neuropathology often reveals Pick's disease or lobar atrophy.

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Area of Science:

Neurology
Neuroscience
Linguistics

Background:

Primary progressive aphasia (PPA) is a focal dementia characterized by gradual language dissolution.
It presents with anomia, progressing to affect syntax and semantics, with either fluent or nonfluent speech.
PPA is distinguished from other dementias by preserved memory, visual processing, and personality in early stages.

Purpose of the Study:

Clarify the diagnostic ambiguity of semantic dementia in relation to PPA subtypes.
Highlight the importance of specifying the diagnostic criteria when using the term 'semantic dementia'.
Underscore the need for a precise understanding of PPA for effective clinical management.

Main Methods:

Review of clinical presentations and diagnostic criteria for PPA and semantic dementia.
Analysis of neuroimaging findings (structural and physiological) in PPA patients.
Examination of neuropathological findings from autopsy studies in PPA.

Main Results:

PPA primarily affects left hemisphere language cortices, confirmed by neuroimaging.
Neuropathology in PPA commonly includes Pick's disease or lobar atrophy, distinct from typical Alzheimer's disease distribution.
Semantic dementia diagnosis has dual meanings, necessitating clear specification in clinical practice.

Conclusions:

PPA is a distinct dementia syndrome with specific neuroanatomical and neuropathological correlates.
Distinguishing PPA subtypes and clarifying semantic dementia definitions are crucial for accurate diagnosis.
Further research into chromosome 17-linked dementias may illuminate sporadic PPA, improving patient care.

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