Spontaneous perceptual facial distortions correlate with ventral occipitotemporal activity

• Prosopometamorphopsia is characterized by distorted perception of faces.
• Patient AS perceives facial distortions persistently and with no known cause.
• AS rated the magnitude of her distortions as she viewed faces during an fMRI scan.
• AS’ ratings were correlated with signal changes in various face-selective areas.
• AS’ prosopometamorphopsia reflects anomalous neural activity in these face areas.

Prosopometamorphopsia is a disorder of face perception in which faces appear distorted to the perceiver. The neural basis of prosopometamorphopsia is unclear, but may involve abnormal activity in face-selective areas in the ventral occipito-temporal pathway. Here we present the case of AS, a 44-year-old woman who reports persistent perceptual distortions of faces with no known cause. AS was presented with facial images and rated the magnitude of her distortions while activity in her core face areas and other areas in the ventral visual pathway was measured using functional magnetic resonance imaging. The magnitude of her distortions was positively correlated with signal changes in the right occipital face area (OFA) and right fusiform face area (FFA), as well as right V1–V3, and right lateral occipital cortex (LOC). There was also a trend for a significant correlation with signal in the left OFA and right inferior frontal gyrus (IFG), but not in the right or left superior temporal sulcus (STS). These results suggest that AS’ prosopometamorphopsia reflects anomalous activity in face-processing network, particularly in the ventral occipitotemporal cortex.