Hereditary Prosopagnosia: the First Case Series

Grueter, Martina; Grueter, Thomas; Bell, Vaughan; Horst, J.; Laskowski, Wolfgang; Sperling, Karl; Halligan, Peter W.; Ellis, Hadyn D.; Kennerknecht, Ingo

doi:10.1016/s0010-9452(08)70502-1

Cited by 125 publications

(103 citation statements)

References 44 publications

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“…We have observed a similar pattern in two German families (Grueter 2004;Grueter et al 2007). However, as long as molecular genetic data are not available, it cannot be definitely excluded that some of them might be normal transmitters.…”

Section: Discussionsupporting

confidence: 79%

“…No common procedure has been published. In an initial study, we did attempt to assess the diagnoses more objectively (Grueter et al 2007). Eight out of 38 hereditary prosopagnosics from seven families agreed with in-depth testing.…”

Section: Discussionmentioning

confidence: 99%

“…However, the familial segregation pattern was not studied in anymore detail. Recently, we could show in all 38 German families studied that the segregation pattern of congenital prosopagnosia is fully compatible with an autosomal-dominant mode of inheritance (Grueter et al 2007). We could further show that the inborn form-in the absence of any traumatic event-is very common, with a minimal prevalence of 2.5% .…”

Section: Introductionmentioning

confidence: 97%

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Hereditary prosopagnosia (HPA): the first report outside the Caucasian population

et al. 2006

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Prosopagnosia (PA) or face blindness is characterized by a deficiency in identifying familiar faces. Almost all reports are single cases or collections of unrelated patients who acquired prosopagnosia after brain injuries, strokes or atrophy of at least the right occipito-temporal cortex. Until 2001, the inborn form-in the absence of any brain lesions-was described in fewer than 20 probands exclusively of Caucasian origin. We recently found that in the German Caucasian population, congenital prosopagnosia has a very high prevalence of at least 2.5% and that it is genetically determined. It is best described by autosomaldominant inheritance in the more than 50 families investigated. We therefore introduced the term nonsyndromic hereditary PA for the congenital form of a monosymptomatic or isolated PA. This surprisingly high frequency in the Caucasian population prompted us to extend our search to other ethnic groups. We performed a questionnaire-based screening among 198 native Indian students at Banaras Hindu University in Varanasi. In a then selected subset, we found after further detailed diagnostic interviews one Bengali female student with visual agnosia for face recognition only. Several other members of her large family reported the same impairment of face recognition. The segregation pattern of PA in this family is also compatible with autosomal-dominant inheritance.

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Section: Discussionsupporting

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

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Hereditary prosopagnosia (HPA): the first report outside the Caucasian population

et al. 2006

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“…The diagnosis of hereditary prosopagnosia was based on a standardized semistructured interview (Tables S3 and S4) (41,42), which has been validated with objective face recognition tests in previous studies (41,43).…”

Section: Methodsmentioning

confidence: 99%

Simulation of talking faces in the human brain improves auditory speech recognition

Kriegstein

Doğan

Grüter

et al. 2008

Proc. Natl. Acad. Sci. U.S.A.

137

192

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Human face-to-face communication is essentially audiovisual. Typically, people talk to us face-to-face, providing concurrent auditory and visual input. Understanding someone is easier when there is visual input, because visual cues like mouth and tongue movements provide complementary information about speech content. Here, we hypothesized that, even in the absence of visual input, the brain optimizes both auditory-only speech and speaker recognition by harvesting speaker-specific predictions and constraints from distinct visual face-processing areas. To test this hypothesis, we performed behavioral and neuroimaging experiments in two groups: subjects with a face recognition deficit (prosopagnosia) and matched controls. The results show that observing a specific person talking for 2 min improves subsequent auditory-only speech and speaker recognition for this person. In both prosopagnosics and controls, behavioral improvement in auditory-only speech recognition was based on an area typically involved in face-movement processing. Improvement in speaker recognition was only present in controls and was based on an area involved in face-identity processing. These findings challenge current unisensory models of speech processing, because they show that, in auditory-only speech, the brain exploits previously encoded audiovisual correlations to optimize communication. We suggest that this optimization is based on speaker-specific audiovisual internal models, which are used to simulate a talking face.fMRI ͉ multisensory ͉ predictive coding ͉ prosopagnosia H uman face-to-face communication works best when one can watch the speaker's face (1). This becomes obvious when someone speaks to us in a noisy environment, in which the auditory speech signal is degraded. Visual cues place constraints on what our brain expects to perceive in the auditory channel. These visual constraints improve the recognition rate for audiovisual speech, compared with auditory speech alone (2). Similarly, speaker identity recognition by voice can be improved by concurrent visual information (3). Accordingly, audiovisual models of human voice and face perception posit that there are interactions between auditory and visual processing streams (Fig. 1A) (4, 5).Based on prior experimental (6-8) and theoretical work (9-12) we hypothesized that, even in the absence of visual input, the brain optimizes auditory-only speech and speaker recognition by harvesting predictions and constraints from distinct visual face areas (Fig. 1B).Experimental studies (6,8) demonstrated that the identification of a speaker by voice is improved after a brief audiovisual experience with that speaker (in contrast to a matched control condition). The improvement effect was paralleled by an interaction of voice and face-identity sensitive areas (8). This finding suggested that the associative representation of a particular face facilitates the recognition of that person by voice. However, it is unclear whether this effect also extends to other audiovisual dependencies in human ...

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“…In just the last few years, however, several new lines of evidence point to a specific role of genes in determining the neural machinery of face perception. First, a congenital disorder in face perception, developmental prosopagnosia, has been shown to run in families (76,77). Second, face-perception ability is heritable (i.e., more strongly correlated for identical than fraternal twins), and this effect is independent of the heritability of domain-general abilities like IQ or global attention (78,79).…”

mentioning

confidence: 99%

Functional specificity in the human brain: A window into the functional architecture of the mind

Kanwisher

2010

Proc. Natl. Acad. Sci. U.S.A.

720

616

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Is the human mind/brain composed of a set of highly specialized components, each carrying out a specific aspect of human cognition, or is it more of a general-purpose device, in which each component participates in a wide variety of cognitive processes? For nearly two centuries, proponents of specialized organs or modules of the mind and brain-from the phrenologists to Broca to Chomsky and Fodor-have jousted with the proponents of distributed cognitive and neural processing-from Flourens to Lashley to McClelland and Rumelhart. I argue here that research using functional MRI is beginning to answer this long-standing question with new clarity and precision by indicating that at least a few specific aspects of cognition are implemented in brain regions that are highly specialized for that process alone. Cortical regions have been identified that are specialized not only for basic sensory and motor processes but also for the high-level perceptual analysis of faces, places, bodies, visually presented words, and even for the very abstract cognitive function of thinking about another person's thoughts. I further consider the as-yet unanswered questions of how much of the mind and brain are made up of these functionally specialized components and how they arise developmentally.brain imaging | modularity | functional MRI | fusiform face area U nderstanding the nature of the human mind is arguably the greatest intellectual quest of all time. It is also one of the most challenging, requiring the combined insights not only of psychologists, computer scientists, and neuroscientists but of thinkers in nearly every intellectual pursuit, from biology and mathematics to art and anthropology. Here, I discuss one currently fruitful component of this grand enterprise: the effort to infer the architecture of the human mind from the functional organization of the human brain.The idea that the human mind/brain is made up of highly specialized components began with the Viennese physician Franz Joseph Gall (1758-1828). Gall proposed that the brain is the seat of the mind, that the mind is composed of distinct mental faculties, and that each mental faculty resides in a specific brain organ. A heated debate on localization of function in the brain raged over the next century (SI Text), with many of the major figures in the history of neuroscience weighing in (Broca, Brodmann, and Ferrier in favor, and Flourens, Golgi, and Lashley opposed). By the early 20th century, a consensus emerged that at least basic sensory and motor functions reside in specialized brain regions.The debate did not end there, however. Today, a century later, two questions are still fiercely contested. First, how functionally specialized are regions of the brain? The concept of functional specialization is not all or none but a matter of degree; a cortical region might be only slightly more engaged in one mental function than another, or it might be exclusively engaged in a single mental function. Many neuroscientists today challenge the strong (exclusive) version of ...

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Hereditary Prosopagnosia: the First Case Series

Cited by 125 publications

References 44 publications

Hereditary prosopagnosia (HPA): the first report outside the Caucasian population

Hereditary prosopagnosia (HPA): the first report outside the Caucasian population

Simulation of talking faces in the human brain improves auditory speech recognition

Functional specificity in the human brain: A window into the functional architecture of the mind

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