Neuroradiological findings in primary progressive aphasia: CT, MRI and cerebral perfusion SPECT

Abstract

Primary progressive aphasia (PPA) is defined as progressive decline in language for 2 or more years with preservation of activities of daily living and general cognitive functions. Whereas the clinical features of this syndrome have been well documented, the neuroradiological findings have not been studied systematically. We studied 13 patients with PPA retrospectively: 10 underwent CT, 12 MRI and 12 cerebral perfusion studies using^99mTc-HMPAO SPECT. CT and MR images were scored for focal atrophy by two independent assessors. Initial qualitative assessment of SPECT images was confirmed by quantitative analysis. CT was normal in 5 patients. Focal atrophy, affecting predominantly the left temporal lobe, was seen in 4 of 10 patients on CT, and 10 of 12 on MRI. Atrophy was localised primarily to the superior and middle temporal gyri on MRI. All 12 patients who underwent SPECT had unilateral temporal lobe perfusion defects, in 2 patients of whom MRI was normal. CT is relatively insensitive to focal abnormalities in PPA; MRI and SPECT are the imaging modalities of choice. MRI allows accurate, specific localisation of atrophy within the temporal neocortex. SPECT may reveal a functional decrease in cerebral perfusion prior to establishment of structural change.