Abstract Details

Title Clinical and Neuroanatomical Characterization of the Semantic Behavioural Variant of Frontotemporal Dementia in a Multicentre Italian Cohort

Topic Aging, Dementia, and Behavioral Neurology

Presentation(s) P4 - Poster Session 4 (11:45 AM-12:45 PM)

Poster/Presentation
Number 9-004

Objective

To evaluate clinical, neuropsychological, neuroimaging and genetic features of an Italian cohort of semantic behavioral variant FTD (sbvFTD) patients, defined according to recently proposed guidelines, compared to semantic variant primary progressive aphasia (svPPA) and behavioral variant FTD (bvFTD) patients.

Background sbvFTD is a recently recognized neurodegenerative condition presenting with specific behavioral and semantic derangements and predominant atrophy of the right anterior temporal lobe (ATL).

Design/Methods

Fifteen sbvFTD, 63 bvFTD and 25 svPPA patients and forty age- and sex-matched controls were enrolled. Patients underwent clinical and cognitive evaluations and brain MRI on a 3T scanner. Emerging symptoms of sbvFTD patients were recorded. Grey matter atrophy was investigated using voxel-based morphometry.

Results Age at onset and disease duration were comparable among groups. The majority of sbvFTD patients developed early person-specific semantic knowledge loss and words (67%), objects semantic loss (67%), complex compulsions and rigid thought process (60%). As the disease unfolded, more behavioral symptoms emerged (apathy/inertia, loss of empathy, anxiety, suspiciousness). sbvFTD patients showed sparing of attentive and executive functions, especially compared to bvFTD and better visual naming and semantic fluency compared to svPPA. All sbvFTD patients failed at the famous face recognition test and more than 80% failed in understanding written metaphors and humor. At MRI, sbvFTD had a pattern of predominant right ATL atrophy, almost specular to the left-sided pattern of atrophy of svPPA. Three sbvFTD patients presented pathogenic genetic variants (n=1 C9orf72, n=1 MAPT, n=1 GRN).

Conclusions

We replicated the application of sbvFTD diagnostic guidelines in an independent Italian cohort, demonstrating that the presence of two out of three core diagnostic criteria (person-specific semantic knowledge loss, mental rigidity and loss of empathy), along with preserved executive functions and a predominant right ATL atrophy with sparing of frontal lobes should prompt a diagnosis of sbvFTD.

Authors/Disclosures
Edoardo G. Spinelli, MD PRESENTER	Dr. Spinelli has nothing to disclose.
Alma Ghirelli (San Raffaele Research Institute)	Ms. Ghirelli has nothing to disclose.
Elisa Canu (Ospedale San Raffaele)	The institution of Elisa Canu has received research support from Italian Ministry of Health .
Silvia Basaia	Silvia Basaia has nothing to disclose.
Veronica Castelnovo, MSc (San Raffaele Scientific Institute, Vita-Salute San Raffaele University)	Dr. Castelnovo has nothing to disclose.
Giordano Cecchetti (San Raffaele Hospital)	Giordano Cecchetti has nothing to disclose.
Elisa Sibilla	Elisa Sibilla has nothing to disclose.
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.
Sara Prioni (Fondazione IRCCS Istituto Neurologico Carlo Besta)	Sara Prioni has nothing to disclose.
Cristina Villa	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.
Federico Verde	Federico Verde has nothing to disclose.
Nicola Ticozzi, MD (Istituto Auxologico Italiano)	The institution of Dr. Ticozzi has received research support from Italian Ministry of Health . The institution of Dr. Ticozzi has received research support from AriSLA.
Vincenzo Silani, MD, FAAN (University of Milan Medical School - IRCCS Istituto Auxologico Italiano)	Dr. Silani has nothing to disclose.
Massimo Filippi, MD, FAAN (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.

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