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Neurology. 2014 Jan 21;82(3):239-47. doi: 10.1212/WNL.0000000000000031. Epub 2013 Dec 18.

In vivo signatures of nonfluent/agrammatic primary progressive aphasia caused by FTLD pathology.

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1
From the Memory and Aging Center (F.C., M.L.M., M.H., B.G., B.M.B., J.O., M.S., L.T.G., B.L.M., W.W.S., M.L.G.-T.) and Department of Pathology (E.J.H.), University of California, San Francisco; Department of Neurology (F.C., M.F., G.C., G.M.) and Neuroimaging Research Unit (F.C., M.F.), Institute of Experimental Neurology, Division of Neuroscience, Vita-Salute University and San Raffaele Scientific Institute, Milan, Italy; Department of Pathology and Laboratory Medicine (J.Q.T.), University of Pennsylvania, Philadelphia; Center for Aphasia and Related Disorders (N.D.), VA Northern California Health Care System, Martinez, CA; and Department of Neurology (N.D.), University of California, Davis.

Abstract

OBJECTIVE:

To identify early cognitive and neuroimaging features of sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) caused by frontotemporal lobar degeneration (FTLD) subtypes.

METHODS:

We prospectively collected clinical, neuroimaging, and neuropathologic data in 11 patients with sporadic nfvPPA with FTLD-tau (nfvPPA-tau, n = 9) or FTLD-transactive response DNA binding protein pathology of 43 kD type A (nfvPPA-TDP, n = 2). We analyzed patterns of cognitive and gray matter (GM) and white matter (WM) atrophy at presentation in the whole group and in each pathologic subtype separately. We also considered longitudinal clinical data.

RESULTS:

At first evaluation, regardless of pathologic FTLD subtype, apraxia of speech (AOS) was the most common cognitive feature and atrophy involved the left posterior frontal lobe. Each pathologic subtype showed few distinctive features. At presentation, patients with nfvPPA-tau presented with mild to moderate AOS, mixed dysarthria with prominent hypokinetic features, clear agrammatism, and atrophy in the GM of the left posterior frontal regions and in left frontal WM. While speech and language deficits were prominent early, within 3 years of symptom onset, all patients with nfvPPA-tau developed significant extrapyramidal motor signs. At presentation, patients with nfvPPA-TDP had severe AOS, dysarthria with spastic features, mild agrammatism, and atrophy in left posterior frontal GM only. Selective mutism occurred early, when general neurologic examination only showed mild decrease in finger dexterity in the right hand.

CONCLUSIONS:

Clinical features in sporadic nfvPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks. We propose that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FTLD-TDP.

PMID:
24353332
PMCID:
PMC3902758
DOI:
10.1212/WNL.0000000000000031
[Indexed for MEDLINE]
Free PMC Article
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