Prosopagnosia: A clinicopathological study

Abstract

T w o cases of prosopagnosia (failure to recognize faces) are presented, together with the autopsy findings. Only 7 other cases of prosopagnosia caused by pathologically verified occlusive vascular lesions have been described. All t h e patients had significant bilateral lesions of the cerebral hemispheres, at least one being in the medial-ventral occipital region. In our patients the bilateral lesions were grossly symmetrical in the distribution of the posterior cerebral arteries but with somewhat more extensive involvement on one side. In all reported cases one or both fusiform gyri were destroyed, and there were variably sized lesions in the pericalcarine structures. These findings suggest that the fusiform gyrus functions as a visual association area for the recognition of specific faces.Cohn R, Neumann MA, Wood DH: Prosopagnosia: a clinicopathological study. Ann Neurol 1: [177][178][179][180][181][182] 1977 Failure to recognize previously well-known human faces, which occurs in certain patients with cerebral lesions, was explicitly noted by Jackson [ 11, Charcot [2], and Wilbrand [3-51. Nevertheless, many present-day investigators begin their discussions of the phenomenon with the report of Hoff and Potzl[6] because these authors specifically considered it as a distinct entity. Bodamer [7] named the condition prosopagnosia. Although m o r e than 100 papers have been published o n this subject, few have included the pathological findings in the brain. However, it should be recognized that only selected localized vascular lesions allow precise correlation between behavior and pathological findings. O t h e r types of central nervous system abnormalities such as those presented by Meadows [8], which included trauma and neoplasia, may generate useful information, but their general and progressive effects preclude anatomical accuracy. Similarly, neurosurgical observations, because

Structure and function in acquired prosopagnosia: Lessons from a series of 10 patients with brain damage

“…As a symptom, it can be one of many deficits in patients with widespread cognitive dysfunction, as in Alzheimer's disease (Cronin‐Coulomb et al , 2000; Mendez, Martin, Smyth, & Whitehouse, 1992; Roudier et al , 1998), Huntington's disease (Janati, 1985), Parkinson's disease (Dewick, Hanley, Davies, Playfer, & Turnbull, 1991), autism (Sasson, 2006) and schizophrenia (Feinberg, Rifkin, Schaffer, & Walker, 1986; Onitsuka et al , 2003). As a syndrome, while it has sometimes been attributed to a similar combination of generalized cognitive and visual disturbances (Bay, 1953; Cohn, Neumann, & Wood, 1977), it is now recognized as a selective functional entity generated by discrete neurologic lesions to specific anatomic structures involved in face processing, as had been proposed decades ago (Bodamer, 1947; Hoff & Potzl, 1937).…”

The Capgras syndrome: Neurological/neuropsychological perspectives.

“…Unfortunately, at least for clarity and understanding’s sake, numerous other, and seemingly similar, clinical disorders were “discovered” after Capgras’ disorder, and have been classified under the heading of misidentification syndromes . These disorders include the illusion of Fregoli, the illusion of intermetamorphosis, the syndrome of subjective doubles, autoscopy, prosopagnosia, and reduplicative paramnesia (Alexander, Stuss, & Benson, 1979; Berson, 1983; Cohn, Neumann, & Wood, 1977; Courbon & Fail, 1927; Courbon & Tusques, 1932; Hacaen & Angelergues, 1962; Lukianowicz, 1958; Malliaras, Kossovitsa, & Christodoulou, 1978; Meadows, 1974; Morrison, 1980; Pick, 1903; Weinstein & Kahn, 1955). The misidentification syndromes show differences from the Capgras phenomenon, but also show dysfunctions common to the right temporoparietal cortex, i.e., disturbed visuospatial analysis, impaired facial recognition and memory, and abnormal sensations of general familiarity or unfamiliarity.…”

Prosopagnosia: a clinical, psychological, and anatomical study of three patients.

“…Postmortem studies, which are infrequent, give the most accurate information, and the published cases show a common lesion in the right inferior occipitotemporal region in the lingual and fusiform gyri. These cases, however, also have a left hemisphere lesion which, in all but two cases, is symmetrically placed in the left occipito-temporal region (Meadows, 1974a;Cohn et al, 1974). In the two exceptions, the left sided lesions are, respectively, a superficial gliosis in the parietal region (Pevzner et al, 1962), and a tumour invading through the corpus callosum to the ventricular wall (Hecaen et al, 1957).…”