Progressive posterior cortical dysfunction

Abstract

Progressive posterior cortical dysfunction (PPCD) is an insidious syndrome characterized by prominent disorders of higher visual processing. It affects both dorsal (occipito-parietal) and ventral (occipito-temporal) pathways, disturbing visuospatial processing and visual recognition, respectively. We report a case of a 67-year-old woman presenting with progressive impairment of visual functions. Neurologic examination showed agraphia, alexia, hemispatial neglect (left side visual extinction), complete Balint’s syndrome and visual agnosia. Magnetic resonance imaging showed circumscribed atrophy involving the bilateral parieto-occipital regions, slightly more predominant to the right. Our aim was to describe a case of this syndrome, to present a video showing the main abnormalities, and to discuss this unusual presentation of dementia. We believe this article can contribute by improving the recognition of PPCD.

Posterior cortical atrophy - a prototypical case of dementia beginning with visual symptoms: case report

“…In fact, according to some authors (8) , PPCD can sometimes be misattributed to ocular causes, such as cataract or macular disease. PPCD is capable of damaging both the dorsal (related to spatial analysis) and ventral (related to spatial recognition) visual processing streams, where both deficits can be found in the same patient (7) . Both lower and higher level visual impairments contribute to the com plex visual symptoms associated with PPCD, as reported by one study (9) that investigated tasks of visual acuity, line orientation, con tour integration, and rotated object comparison.…”

“…These symptoms may suggest a primarily oph thalmologic disorder and add to the fact that PCA rarely presents with memory complains, this clinical syndrome may not evoke promptly the diagnosis of a dementia disorder, thus postponing its correct treatment. For that reason, it is essential to rule out primary ophthal mologic causes when diagnosing PPCD (6,7) . In fact, according to some authors (8) , PPCD can sometimes be misattributed to ocular causes, such as cataract or macular disease.…”