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November 1997

Frontotemporal Degeneration, Pick Disease, and Corticobasal Degeneration: Three Entities or 1?

Author Affiliations

London, Ontario

Arch Neurol. 1997;54(11):1429. doi:10.1001/archneur.1997.00550230092025

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The brain functions with bewildering complexity, but fails in only a few ways. This makes prognosis easier but classification difficult. Diverse etiologies may manifest in a similar manner, given the limited pathological repertoire of the brain.

Neary rightfully emphasizes the need to distinguish pathological patterns from clinical syndromes and histological changes. Clinical syndromes depend on the part of the brain involved, but some pathological conditions may be silent and some histological changes may be clinically irrelevant. Kertez highlights the emerging similarities among the several syndromes, suggesting Pick complex as an umbrella label and a tau protein abnormality as an underlying disturbance. This is conceptually appealing, but similarities are no guarantee of common origin, as demonstrated by some of the spinocerebellar degenerations, where different genotypes lead to similar phenotypes.

The regional involvement of all the syndromes in question is so striking that one wonders whether there are embryological clues to these

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