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From the Memory Aging Center (M.L.G.-T., S.M.B., J.O., B.L.M.), University of California, San Francisco, Department of Neurology, San Francisco; Vita-Salute San Raffaele University (S.M.B., V.G., A.M., D.P., V.G., S.F.C.), Department of Nuclear Medicine, IRCCS San Raffaele, Department of Clinical Neurosciences, San Raffaele Turro, National Institute of Neuroscience, Milan, Italy; University of California (J.O., N.F.D.), Davis, CA, and San Diego, CA.
Address correspondence and reprint requests to Dr. Maria Luisa Gorno-Tempini, UCSF Memory and Aging Center, 350 Parnassus Ave., Suite 506, San Francisco, CA 94143
Objective: Primary progressive aphasia (PPA) is characterized by isolated decline in language functions. Semantic dementia and progressive nonfluent aphasia are accepted PPA variants. A "logopenic" variant (LPA) has also been proposed, but its cognitive and anatomic profile is less defined. The aim of this study was to establish the cognitive and anatomic features of LPA.
Methods: Six previously unreported LPA cases underwent extensive neuropsychological evaluation and an experimental study of phonological loop functions, including auditory and visual span tasks with digits, letters, and words. For each patient, a voxel-wise, automated analysis of MRI or SPECT data were conducted using SPM2.
Results: In LPA, speech rate was slow, with long word-finding pauses. Grammar and articulation were preserved, although phonological paraphasias could be present. Repetition and comprehension were impaired for sentences but preserved for single words, and naming was moderately affected. Investigation of phonological loop functions showed that patients were severely impaired in digit, letter, and word span tasks. Performance did not improve with pointing, was influenced by word length, and did not show the normal phonological similarity effect. Atrophy or decreased blood flow was consistently found in the posterior portion of the left superior and middle temporal gyri and inferior parietal lobule.
Conclusions: Logopenic progressive aphasia (LPA) is a distinctive variant of primary progressive aphasia. Cognitive and neuroimaging data indicate that a deficit in phonological loop functions may be the core mechanism underlying the LPA clinical syndrome. Recent studies suggest that Alzheimer disease may be the most common pathology underlying the LPA clinical syndrome.
GLOSSARY: AD = Alzheimer disease; BA = Brodmann area; CDR = Clinical Dementia Rating; CVLT-MS = California Verbal Learning Test–Mental Status Edition; ECD = ethyl cysteinate dimer; FWHM = full-width at half-maximum; GM = gray matter; LPA = logopenic progressive aphasia; MMSE = Mini-Mental State Examination; PNFA = progressive nonfluent aphasia; PPA = primary progressive aphasia; Rey-O = Rey–Osterrieth; SemD = semantic dementia; VBM = voxel-based morphometry; WAB = Western Aphasia Battery; WAIS-III = Wechsler Adult Intelligence Scale, Third Edition.
e-Pub ahead of print on July 16, 2008, at www.neurology.org.
Supported by the National Institute of Neurological Disorders and Stroke (R01 NS050915), the State of California (DHS 04-35516), the National Institute on Aging (P50 AG03006, P01 AG019724), the Alzheimer’s Disease Research Center of California (03-75271 DHS/ADP/ARCC), and the Italian Ministry for University and Scientific Research (Interlink Grant to S.F.C. and M.L.G.-T.).
Disclosure: The authors report no disclosures.
marilu{at}memory.ucsf.edu
Supplemental data at www.neurology.org
Editorial, page 1218
Received September 26, 2007. Accepted in final form May 2, 2008.
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