Asymmetric cortical degenerative syndromes: clinical and radiologic correlations

Eight patients presented with slowly progressive focal neurologic syndromes that conformed to one of three clinically defined categories: progressive nonfluent aphasia (three patients), progressive perceptual-motor impairment (four patients), and progressive frontal lobe syndrome (one patient). Planar MRI and MRI-based surface or volume renderings demonstrated focal areas of atrophy that correlated well with clinical deficits. Single-photon emission computed tomography (SPECT) showed areas of cortical hypoperfusion that corresponded to focally atrophic regions revealed by MRI, but abnormal areas with SPECT were larger than those suggested by MRI. MRI and SPECT are useful in defining the regional structural and functional cerebral abnormalities that underlie slowly progressive focal neurologic syndromes caused by asymmetric cortical degeneration.