Artículo Sobre Los Nuevos Criterios Para La Clasificación De Las Afasias Progresivas Primaria

tículoVIEWS & REVIEWS

Classification of primary progressive
aphasia and its variants

M.L. Gorno-Tempini,
MD, PhD
A.E. Hillis, MD
S. Weintraub, PhD
A. Kertesz, MD
M. Mendez, MD
S.F. Cappa, MD
J.M. Ogar, MS
J.D. Rohrer, MD
S. Black, MD
B.F. Boeve, MD
F. Manes, MD
N.F. Dronkers, PhD
R. Vandenberghe, MD,
PhD
K. Rascovsky, PhD
K. Patterson, PhD
B.L. Miller, MD
D.S. KnopmanJ.R. Hodges, MD*
M.M. Mesulam, MD*
M. Grossman, MD*

Address correspondence and
reprint requests to Dr. Maria
Luisa Gorno-Tempini, Memory
and Aging Center, Department of
Neurology, UCSF, 350 Parnassus
Avenue, Suite 905, San Francisco,
CA 94143-1207
marilu@memory.ucsf.edu

ABSTRACT

This article provides a classification of primary progressive aphasia (PPA) and its 3 main variants
toimprove the uniformity of case reporting and the reliability of research results. Criteria for the 3
variants of PPA—nonfluent/agrammatic, semantic, and logopenic—were developed by an international group of PPA investigators who convened on 3 occasions to operationalize earlier published clinical descriptions for PPA subtypes. Patients are first diagnosed with PPA and are then
divided intoclinical variants based on specific speech and language features characteristic of
each subtype. Classification can then be further specified as “imaging-supported” if the expected
pattern of atrophy is found and “with definite pathology” if pathologic or genetic data are available. The working recommendations are presented in lists of features, and suggested assessment tasks are also provided. Theserecommendations have been widely agreed upon by a
large group of experts and should be used to ensure consistency of PPA classification in
future studies. Future collaborations will collect prospective data to identify relationships
between each of these syndromes and specific biomarkers for a more detailed understanding
of clinicopathologic correlations. Neurology® 2011;76:1006–1014GLOSSARY
AD

Alzheimer disease; FTLD

frontotemporal lobar degeneration; PPA

primary progressive aphasia.

A progressive disorder of language associated with atrophy of the frontal and temporal regions
of the left hemisphere was first described in the 1890s by Pick1 and Serieux.2 In the modern
literature, Mesulam3 described a series of cases with “slowly progressive aphasia,” subsequentlyrenamed primary progressive aphasia (PPA).4 Warrington5 described a progressive disorder of
semantic memory in 1975. This condition was also described by Snowden et al.6 as semantic
dementia. In the early 1990s, Hodges and colleagues7 provided a comprehensive characterization of semantic dementia. Subsequently, Grossman et al.8 described a different form of progressive language disorder, termedprogressive nonfluent aphasia. A consensus meeting
attempted to develop criteria for these conditions in relation to frontotemporal lobar degeneration.9 For about 2 decades, cases of PPA were generally categorized as semantic dementia or
progressive nonfluent aphasia, or in some studies as “fluent” vs “nonfluent.” However, there

Editorial, page 942
e-Pub ahead of print on February 16, 2011, atwww.neurology.org.
*These authors contributed equally to this work.
From the Memory and Aging Centre (M.L.G.-T., J.M.O., K.R., B.L.M.), Department of Neurology, University of California at San Francisco, San
Francisco; Center for Mind/Brain Sciences (M.L.G.-T.), University of Trento, Trento, Italy; Department of Neurology (A.E.H.), Johns Hopkins
University, Baltimore, MD; Department ofNeurology (S.W., M.M.M.), Northwestern University, Chicago, IL; Cognitive Neurology (A.K.),
University of Western Ontario, London, Canada; Department of Neurology (M.M.), University of California at Los Angeles, Los Angeles; Universita
Vita-Salute San Raffaele (S.F.C.), Milan, Italy; Dementia Research Centre (J.D.R.), Institute of Neurology, University College London, London, UK;
Department of...