A review of the impairments, preserved visual functions, and neuropathology in 21 patients with visual form agnosia – A unique defect with line drawings

We present a comprehensive review of the rare syndrome visual form agnosia (VFA). We begin by documenting its history, including the origins of the term, and the first case study labelled as VFA. The defining characteristics of the syndrome, as others have previously defined it, are then described. The impairments, preserved aspects of visual perception, and areas of brain damage in 21 patients who meet these defining characteristics are described in detail, including which tests were used to verify the presence or absence of key symptoms. From this, we note important similarities along with notable areas of divergence between patients. Damage to the occipital lobe (20/21), an inability to recognise line drawings (19/21), preserved colour vision (14/21), and visual field defects (16/21) were areas of consistency across most cases. We found it useful to distinguish between shape and form as distinct constructs when examining perceptual abilities in VFA patients. Our observations suggest that these patients often exhibit difficulties in processing simplified versions of form. Deficits in processing orientation and size were uncommon. Motion perception and visual imagery were not widely tested for despite being typically cited as defining features of the syndrome – although in the sample described, motion perception was never found to be a deficit. Moreover, problems with vision (e.g., poor visual acuity and the presence of hemianopias/scotomas in the visual fields) are more common than we would have thought and may also contribute to perceptual impairments in patients with VFA. We conclude that VFA is a perceptual disorder where the visual system has a reduced ability to synthesise lines together for the purposes of making sense of what images represent holistically.