Is this a new type of primary prosopagnosia, both progressive and apperceptive?

Figures & data

Table 1 Visual perception test for agnosia⁸

Test	Errors on first examination (percentages)	Errors 14 months later (percentages)	Tasks	(Mean ± SD)
Face recognition
Famous face naming	16 (100)	16 (100)	/16	(0.8 ± 1.95)
Famous face pointing	13 (81)	16 (100)	/16	No mean ± SD for this item
Unknown face discrimination	7 (88)	8 (100)	/8	(0.6 ± 1.12)
Unknown face matching	6 (100)	6 (100)	/6	(0.1 ± 0.27)
Perception of facial expression	5 (83)	6 (100)	/6	(0.0 ± 0.14)
Perception of sex	6 (75)	8 (100)	/8	No mean ± SD for this item
Perception of age	8 (100)	8 (100)	/8	No mean ± SD for this item
Object recognition
Picture naming	12 (75)	16 (100)	/16	(0.0 ± 0.14)
Primary visual functions
Discrimination of line length	2 (20)	6 (75)	/10	(0.0 ± 0.00)
Counting points	3 (50)	4 (67)	/6	(0.0 ± 0.00)
Perception of oblique lines	1 (17)	4 (67)	/6	(0.1 ± 0.64)
Discrimination of piling figures	6 (100)	6 (100)	/6	(0.1 ± 0.40)
Recognition of symbols
Recognition of cartographic symbols	2 (25)	8 (100)	/8	No mean ± SD for this item
Reading characters	11 (17)	–	/66
Katakana	0 (0)	6 (100)	/6	(0.0 ± 0.00)
Hiragana	2 (17)	12 (100)	/12	(0.0 ± 0.27)
Kanji	2 (17)	12 (100)	/12	(0.1 ± 0.14)
Figures	1 (8)	12 (100)	/12	No mean ± SD for this item
Words (kanji)	4 (33)	–	/12	No mean ± SD for this item
Words (kana)	2 (17)	–	/12	No mean ± SD for this item
Hemispatial neglect
Checking the center of lines	19.3% to the left	–

Notes: A dash (−) rather than a number indicates patient found task too difficult; mean ± SD of normal controls.⁸

Abbreviation: SD, standard deviation.

Figure 1 Magnetic resonance imaging coronal sections of fluid-attenuated inversion recovery images on first examination. The calcarine sulcus (arrow) and collateral sulcus (arrow head) were broader on the right than the left. The finding was temporo-parieto-occipital lobe atrophy with right-side dominance.

Figure 2 ^99mTc-ethyl cysteinate dimer SPECT using eZIS¹⁰ on first examination showed decreased blood flow in the temporo-parieto-occipital lobe with right-side dominance.

Figure 3 ¹⁸F-fluorodeoxyglucose positron emission tomography, 7 months after first examination, showing hemihypometabolism, especially from the inferior lateral temporal lobe to the lateral occipital lobe.

Figure 4 Magnetic resonance imaging coronal sections of fluid-attenuated inversion recovery images 11 months after first examination showed progression of temporo-parieto-occipital lobe atrophy with right-side dominance.

Figure 5 ^99mTc-ethyl cysteinate dimer SPECT using the eZIS¹⁰ 11 months after first examination showed decreased blood flow in the temporo-parieto-occipital lobe with right-side dominance.

Figure 6 A simple, schematic diagram of the relative loci of associative and apperceptive prosopagnosia, fluent aphasia, and pure alexia. Our own case history of PCA/apperceptive prosopagnosia relates to area D in the ventral part of the right posterior area of the brain.

Abbreviations: PCA, posterior cortical atrophy; FTLD, frontotemporal lobar degeneration.

MahoneyFIBarthelDWFunctional evaluation: the Barthel indexMd State Med J196514616514258950

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MizumuraSKumitaSStereotactic statistical imaging analysis of the brain using the easy Z-score imaging system for sharing a normal databaseRadiat Med200624754555217058152

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