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Abstract Details

Converging Longitudinal Patterns of Atrophy in Clinical Variants of Frontotemporal Lobar Degeneration
Aging, Dementia, and Behavioral Neurology
Aging and Dementia Posters (7:00 AM-5:00 PM)
073
To assess longitudinal patterns of atrophy shown by magnetic resonance imaging (MRI) in the cortical and subcortical grey matter (GM) of patients affected by different clinical variants of the frontotemporal lobar degeneration (FTLD) spectrum.
The progression of FTLD is accompanied by a widespread disruption of GM that extends well beyond frontal and temporal cortical regions.
Fifty-nine patients, including 26 with behavioral variant of frontotemporal dementia (bvFTD), 10 non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA), 12 semantic variant of PPA (svPPA), and 11 motor neuron disease (MND), in the absence of known pathogenic mutations, underwent MRI on a 3T scanner at 6-month intervals for one year. Thirty-three healthy controls underwent the same protocol. 3D T1-weighted MRI sequences were analyzed using voxel-based morphometry to assess the longitudinal evolution of GM atrophy in patients, compared with HC.
At baseline, severe diffuse atrophy of frontotemporal cortical regions and basal ganglia was found in bvFTD, nfvPPA and svPPA groups, whereas MND did not show significant GM atrophy. At 6-month follow-up, bvFTD and PPA groups showed significant progression of atrophy in the insular (bvFTD, nfvPPA and svPPA) and anterior cingulate cortex (bvFTD and nfvPPA), bilaterally, as well as in the left caudate nucleus and middle temporal cortex (svPPA). At 12-month follow-up, similar patterns of atrophy progression were found, with the additional involvement of the superior frontal cortical gyri in nfvPPA, bilaterally, and right hippocampus in svPPA. No significant progression of atrophy was found in MND patients.

Our data suggest that atrophy of insular and anterior cingulate regions closely reflects the progression of neurodegeneration across the behavioral and linguistic presentations of FTD, in contrast with a substantial sparing of GM in MND. Measures of cingulo-insular-striatal network degeneration are promising candidate biomarkers of disease progression in FTD.
Authors/Disclosures
Edoardo G. Spinelli, MD
PRESENTER 		Dr. Spinelli has nothing to disclose.
Silvia Basaia Silvia Basaia has nothing to disclose.
Camilla Cividini, MSc (San Raffaele Scientific Institute, Vita-Salute San Raffaele University) Ms. Cividini has nothing to disclose.
No disclosure on file
Giuseppe Magnani Giuseppe Magnani has nothing to disclose.
Francesca Caso, MD (Universita' Vita Salute San Raffaele) Dr. Caso has nothing to disclose.
Paola Caroppo Paola Caroppo has nothing to disclose.
No disclosure on file
Lucio Tremolizzo (UNIMIB) Lucio Tremolizzo has nothing to disclose.
Ildebrando H. Appollonio, MD (Neurology Section, Dept. of Medicine and Surgery, University of Milano Bicocca) Dr. Appollonio has nothing to disclose.
Vincenzo Silani, MD, FÂ鶹´«Ã½Ó³»­ (University of Milan Medical School - IRCCS Istituto Auxologico Italiano) Dr. Silani has nothing to disclose.
No disclosure on file
Massimo Filippi, MD, FÂ鶹´«Ã½Ó³»­ (Ospedale San Raffaele, Neuroimaging Research Unit) Dr. Filippi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion, Almirall, Biogen, Merck, Novartis, Roche, Sanofi. Dr. Filippi has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion, Biogen, Bristol-Myers Squibb, Merck, Novartis, Roche, Sanofi, Sanofi-Aventis, Sanofi-Genzyme, Takeda. Dr. Filippi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bayer, Biogen, Celgene, Chiesi Italia SpA, Eli Lilly, Genzyme, Janssen, Merck-Serono, Neopharmed Gentili, Novartis, Novo Nordisk, Roche, Sanofi, Takeda, and TEVA. Dr. Filippi has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Filippi has received research support from Biogen Idec, Merck-Serono, Novartis, Roche, the Italian Ministry of Health, the Italian Ministry of University and Research, and Fondazione Italiana Sclerosi Multipla.
Federica Agosta (San Raffaele Scientific Institute) Federica Agosta has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Philips. Federica Agosta has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier INC.