Background: Primary progressive aphasia (PPA) leads to a gradual and relatively isolated dissolution of language function. The factors that determine the selectivity of the disease process remain unknown. We had speculated that PPA may occasionally arise as a tardive manifestation of genetic or acquired vulnerabilities involving the language network of the brain. Objective: To explore predisposing factors for PPA. Results: In 2 patients, PPA developed with a background of mild left hemicranial hypoplasia. Conclusion: In keeping with other observations of PPA in patients with dyslexia and childhood injury to the left temporal lobe, these 2 patients support the contention that some cases of PPA may arise in settings where the language network has become a locus of least resistance.
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[Anonymous], 1992, UNUSUAL DEMENTIAS, DOI DOI 10.1093/BRAIN/AWP136