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Román Alberca, MD; Enrique Montes, MD; Eric Russell, MD; Eulogio Gil-Néciga, MD; Marsel Mesulam, MD

Arch Neurol. 2004;61:265-268.

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.

From the Department of Neurology, Virgen del Rocío, Sevilla, Spain (Drs Alberca, Montes, and Gil-Néciga); and the Departments of Radiology (Dr Russell) and Cognitive Neurology and Alzheimer's Disease Center (Dr Mesulam), Northwestern University Feinberg School of Medicine, Chicago, Ill.

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