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. 2019 May;20(3-4):146-158.
doi: 10.1080/21678421.2018.1556695. Epub 2019 Jan 22.

Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates

Giulia Vinceti  1   2 , Nicholas Olney  1   3 , Maria Luisa Mandelli  1 , Salvatore Spina  1 , H Isabel Hubbard  1   4 , Miguel A Santos-Santos  1 , Christa Watson  1 , Zachary A Miller  1 , Catherine Lomen-Hoerth  3 , Paolo Nichelli  2 , Bruce L Miller  1 , Lea T Grinberg  1   5 , William W Seeley  1   5 , Maria Luisa Gorno-Tempini  1
Affiliations

Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates

Giulia Vinceti et al. Amyotroph Lateral Scler Frontotemporal Degener. 2019 May.

Abstract

Objective: Behavioral variant frontotemporal dementia (bvFTD), is commonly considered the cognitive presentation of the frontotemporal dementia-motor neuron disease (FTD-MND) spectrum disorder. We evaluated the prevalence of primary progressive aphasia in a series of pathologically confirmed cases of FTD-MND spectrum. Methods: Pathologically confirmed cases of frontotemporal lobar degeneration-motor neuron disease (FTLD-MND) were obtained from the UCSF brain bank. Cases were analyzed for presence of language impairment via retrospective chart review of research visits that include neurologic exam, in-depth cognitive testing and magnetic resonance imaging (MRI) imaging. Forty one cases were included. Thirty two were diagnosed with FTD-MND, while nine cases were diagnosed as MND-only from clinical evaluation. Results: Ten FTLD-MND cases (31%) presented with prominent or isolated language involvement consistent with a diagnosis of primary progressive aphasia (PPA), which we called progressive aphasia with motor neuron disease (PA-MND). Of these, three cases that mirrored the non-fluent variant of PPA (nfvPPA) were named nfvPA-MND. The imaging pattern of these nfvPA-MND showed atrophy strictly confined to the frontal and anterior temporal language cortical areas. Another group of seven cases that resembled patients with the semantic variant PPA (svPPA) were named svPA-MND. The group of svPPA-MND on imaging analysis showed selective atrophy of the temporal lobe and orbitofrontal cortex. Conclusions: Language impairment was a frequent phenotype of FTD-MND associated with focal atrophy patterns within the language networks. This data suggest patients with FTD-MND can present quite often with language phenotype of nfvPPA and svPPA, as opposed to exclusive bvFTD symptoms.

Keywords: Neuropathology; amyotrophic lateral sclerosis; dementia; frontotemporal dementia; imaging; motor neuron disease; primary progressive aphasia.

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Figures

Figure 1.
Figure 1.
Flowchart of case selection on pathological criteria and classification by clinical diagnosis.
Figure 2.
Figure 2.
Time from disease onset to first motor symptom in nfvPA-MND, svPA-MND, bvFTD-MND, and ALS group.
Figure 3.
Figure 3.
Voxel-based morphometry analysis of gray matter. The main groups were compared to healthy controls group. Results are shown on a template in MNI space (a) PA-MND, 10 subjects, at p < 0.05 FWE (b) bvFTD-MND, 22 subjects, at p < 0.001 FWE C ALS, 9 subjects, at p < 0.001 uncorrected. PA-MND: progressive aphasia with motor neuron disease; bvFTD-MND: behavioral variant frontotemporal dementia with motor neuron disease; ALS: amyotrophic lateral sclerosis.
Figure 4.
Figure 4.
Voxel-based morphometry analysis of gray matter in each clinical PA-MND phenotype relative to controls. (a) nfvPA-MND and (b) svPA-MND). Results are shown in MNI space at p < 0.05 FWE. NfvPA-MND: non-fluent progressive aphasia with motor neuron disease; svPA-MND: semantic progressive aphasia with motor neuron disease.
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