Dement Neurocogn Disord.  2016 Dec;15(4):93-102. 10.12779/dnd.2016.15.4.93.

Primary Progressive Aphasia and the Left Hemisphere Language Network

• Mesulam MM

• KMID: 2442833
• DOI: http://doi.org/10.12779/dnd.2016.15.4.93

Abstract

Primary progressive aphasia (PPA) is a clinical syndrome diagnosed when three core criteria are met. First, there should be a language impairment (i.e., aphasia) that interferes with the usage or comprehension of words. Second, the neurological work-up should determine that the disease is neurodegenerative, and therefore progressive. Third, the aphasia should arise in relative isolation, without equivalent deficits of comportment or episodic memory. The language impairment can be fluent or non-fluent and may or may not interfere with word comprehension. Memory for recent events is preserved although memory scores obtained in verbally mediated tests may be abnormal. This distinctive clinical pattern is most conspicuous in the initial stages of the disease, and reflects a relatively selective atrophy of the language network, usually located in the left hemisphere. There are different clinical variants of PPA, each with a characteristic pattern of atrophy. Clinicoanatomical correlations in patient with these variants have led to new insights on the organization of the large-scale language network in the human brain. For example, the left anterior temporal lobe, which was not part of the classic language network, has been shown to play a critical role in word comprehension and object naming. Furthermore, patients with PPA have shown that fluency can be dissociated from grammaticality. The underlying neuropathological diseases are heterogeneous and can include Alzheimer's disease as well as frontotemporal lobar degeneration. The clinician's task is to recognize PPA and differentiate it from other neurodegenerative phenotypes, use biomarkers to surmise the nature of the underlying neuropathology, and institute the most fitting multimodal interventions.

Keyword

language; network; dementia; Alzheimer; frontotemporal

MeSH Terms

Alzheimer Disease
Aphasia
Aphasia, Primary Progressive*
Atrophy
Biomarkers
Brain
Comprehension
Dementia
Frontotemporal Lobar Degeneration
Humans
Memory
Memory, Episodic
Neuropathology
Phenotype
Temporal Lobe
Biomarkers

Figure

• 

  Fig. 1 Atrophy maps in PPA subtypes from Mesulam et al.28 PPA-G, PPA-L, PPA-S- agrammatic, logopenic, and semantic variants of primary progressive aphasia. ATL: anterior temporal lobe, IFG: inferior frontal gyrus, STG: superior temporal gyrus, TPJ: temporoparietal junction. Adapted from Mesulam et al. Arch Neurol 2009;66:1545-1551.28

• 

  Fig. 2 A 2-dimensional template for the rapid clinical classification of PPA patients. PPA-G, PPA-L, PPA-M, PPA-S- agrammatic, logopenic, mixed and semantic variants of primary progressive aphasia.

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