Akiko Ishiwata, Chikako Nito and Kazumi Kimura
Posterior cortical atrophy (PCA) is a neurodegenerative disorder with cerebral atrophy in the parietal, occipital, or
occipitotemporal cortices, and is characterized by visuospatial and visuoperceptual impairments. The majority of the
disorder is compatible pathologically with Alzheimer’s disease (AD). However, many patients show no memory loss
until late stages of the disease. We encountered a case where a combination of several imaging modalities, in
conjunction with symptoms and neuropsychological exams, led not only to a diagnosis of PCA but also identified its
underlying etiology. A 62-year-old Japanese man presented to ophthalmology with visual impairment and without
abnormalities in his visual acuity or visual fields. Alexia was identified, particularly pertinent to reading Kanji, at the
neurology clinic. His initial Mini-Mental State Examination (MMSE) revealed a score of 27/30 points with delayed
recall (-2) and constructional apraxia (-1). The score dropped to 25 points six months later. Brain MRI demonstrated
occipital atrophy. Brain single photon emission computed tomography (SPECT) showed hypoperfusion in the medial
and lateral occipital lobes. The myocardial meta-iodobenzylguanidine (MIBG) scintigraphy showed no evidence of
myocardial postganglionic sympathetic dysfunction. Posterior cortical atrophy (PCA) was diagnosed in conjunction
with the symptoms, including alexia and visual impairment. He developed symmetric parkinsonism one year later.
Lewy body dementia (DLB) was thus considered as the underlying etiology for the PCA. Additionally, alexia pertinent
to reading Kanji has not been reported before. We herein report this rare case.