By causing problems in vision-dependent perception, posterior cortical atrophy (PCA) results in a significant impairment of functional ability. Most cases of PCA are atypical manifestations of Alzheimer's disease. PCA is a rare disease, and accurate data about its frequency are lacking. Disease symptoms begin typically under the age of 60 years. The prerequisites for a correct diagnosis are awareness and recognition of a special clinical picture and special investigations such as cerebrospinal fluid analysis and investigations of brain metabolism. A typical set of clinical characteristics and findings of special examinations in PCA are described.

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