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Clinical and neuropathologic findings in a case of posterior cortical atrophy (PCA)
Author(s) -
Gearing Marla,
Brat Daniel J.,
Hunter Stephen B.,
Cooper Deborah S.,
Tehrani Mahtab,
Lah James J.
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a22-c
Subject(s) - posterior cortical atrophy , visual agnosia , atrophy , pathology , white matter , senile plaques , psychology , agnosia , autopsy , medicine , cortex (anatomy) , alzheimer's disease , neuroscience , dementia , magnetic resonance imaging , disease , radiology , perception
The clinical syndrome of PCA has been associated with various neuropathologic findings including Alzheimer's disease (AD), and it has been suggested that PCA may represent a variant of AD. Here we report our findings in a 65 year‐old woman who presented with complaints of poor color discrimination and visual distortions in dim light. Ophthalmologic evaluation was unremarkable. Cognitive testing showed preservation of memory, language, executive function, attention, and abstract reasoning; she had difficulty with visual reproduction and interpretation of visual stimuli. MMSE score was 28/30. MRI revealed bilateral parieto‐occipital atrophy, and PET showed biparietal hypometabolism. Visual agnosia progressed, but the patient refused subsequent cognitive testing. She died at age 69. At autopsy, the brain was moderately atrophic, with marked posterior parietal atrophy. Microscopic examination revealed severe parieto‐occipital neuronal loss and gliosis with secondary degeneration of subcortical white matter. Diffuse plaques were present throughout most of the neocortex, notably excluding the occipital cortex. Neuritic plaques were not present, and the Braak score was I; thus this case did not meet diagnostic criteria for AD. Other neurodegenerative diseases previously associated with PCA were also ruled out. Our findings in this case indicate that PCA is not a variant of AD. Supported by 1P50‐AG025688.