The taxonomy of primary progressive aphasia
It walks and quacks like a duck … but which duck?
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Taxonomy—the science of classification—helps to create a sense of order out of chaos. In the 18th century, Linnaeus1 published his great Systema Naturae, organizing everything in nature into 3 kingdoms, then subdividing it into classes, orders, genera, and species—everything in its own logical place. Medicine has its own disease-based taxonomy, but only when the discriminating features of a clinical disorder are crystal clear can the clinician use the classification to make accurate diagnoses, provide prognoses, and develop rational treatments.
Classification of neurodegenerative disorders has presented a particular challenge. To deal with diagnostic uncertainty, and to facilitate clinical trials, scientific studies, and patient care, Delphi panels of experts have hammered out consensus criteria, setting standards for the classification of these conditions. Best known is the 1984 report of the National Institute of Neurological and Communicative Disorders and Stroke–Alzheimer's Disease and Related Disorders Association Task Force on Alzheimer disease (AD),2 cited more than 13,000 times. Subsequent expert panels have published criteria for vascular dementia,3 progressive supranuclear palsy,4 Lewy body dementia,5 and frontotemporal dementia.6
In this issue of Neurology®, another panel of 20 experts has published consensus criteria for the Classification of Primary Progressive Aphasia and its Variants.7 First described more …
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