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Declared no potential conflict of interest.
Stefano F. CappaDepartment of Neuroclinical SciencesVita Salute University andSan Raffaele Scientific InstituteMilan, Italy
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CLINICAL HETEROGENEITY
Stefano F. Cappa
Vita-Salute San Raffaele University and
San Raffaele Scientific Institute,
Milan, Italy
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The fronto-temporal spectrum
• Behavioural variant
• Progressive aphasia
• non-fluent/agrammatic variant
• semantic variant
• (logopenic-phonological variant)
• Movement disorder variants
• FTD-MND
• CBD-PSP
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The fronto-temporal spectrum
• Behavioural variant
• Progressive aphasia
• non-fluent/agrammatic variant
• semantic variant
• (logopenic-phonological variant)
• Movement disorder variants
• FTD-MND
• CBD-PSP
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Raskovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, van Swieten JC, Seelaar H, Dopper EGP, Onyike C, Hillis A, Josephs K, Boeve BF, Kertesz A, Seeley WW, Rankin K, Johnson JJ, Gorno-Tempini ML, Rosen H, Neuhaus J, Latham C, Lee A, Kipps CM, Lillo P, Piguet O, Rohrer J, Rossor M, Galasko G, Salmon DP, Black SE, Mesulam M, Weintraub S, Diehl-Schmid J, Pasquier F, Deramecourt V, Lebert F, Pijnenburg Y, Chow TW, Manes F, Grafman J, Cappa SF, Freedman M, Grossman M, Miller BL.
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A comprehensive experimental assessment of social
cognition
bvFTD HC
N (males) 13 (10) 39 (30)
Mean age (SD) 64 (7) 63 (6)
Mean education 11.85 12.28
MMSE 25.33 28.86
Months from onset 52.50 -
FBI 22.08 -
NPI 27.58 -
CDR (range) 0.5-1
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Story-Based ToM and Empathy Task (STET)
• CARTOON-TASK: 36 comic strips
• SECTIONS:
• Theory of Mind (ToM); Empathy (Emp); Physical causation (Caus)
• N OF PERSONS:
•One (ToM1/Emp1/Caus1) or two (ToM2/Emp2/Caus2)
• SOLUTIONS:
•socially/emotionally correct; socially/emotionally wrong; completely wrong
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TOM2
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EMP2
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CAUS2
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Voxel-based morphometric study (11 bvFTD 20 HC)
• Overall gray matter density reduction in bvFTD
• Correlations between STET and gray matter density
Whole Brain
ROIs
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Behavioural results
HC
bvFTDp < 0.001
p < 0.02
p < 0.05
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p =0.055
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bvFTD vs HC
p < 0.05 corrected
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Whole Brain and ROIs
EMP and TOM
p<0.05 corrected
p<0.05
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CAUS
p<0.05 corrected
Whole Brain
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p<0.05 corrected
p<0.05
EMP
Whole Brain and ROIs
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Interpersonal Reactivity Index (IRI) (18 HC, 23 bvFTD, 14 AD)
Phantasy, Perspective Taking, Empathic considerationbvFTD < HC and AD (p < 0.001)
Personal distress inverse effect in bvFTD (p > 0.05)
** ****
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bvFTD < HC and AD (F = 18.43; p < 0.0000)
Self-Monitoring Scale (SMS) (18 HC, 23 bvFTD, 14 AD)
Social Norms Evaluation (102 HC, 23 bvFTD, 16 AD)
bvFTD < HC and AD (F=23.98; p < 0.0000)
**
**
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bvFTD mare refusals than HC for all conditions except 5-5 (p < 0.05)
Ultimatum Game Test (71 HC, 15 bvFTD)
bvFTD HC M-W U test
(valore p)
Offerta 1€ 7 % 37 % 0.0105
Offerta 2€ 9 % 42 % 0.0193
Offerta 3€ 7 % 47 % 0.0006
Offerta 4€ 24 % 59 % 0.0090
Offerta 5€ 96 % 98 % 0.5494
Offerte Eque (4/5€) 76 % 87 % 0.0254
Offerta Inique (1/2/3€) 7 % 42 % 0.0068
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The fronto-temporal spectrum
• Behavioural variant
• Progressive aphasia
• non-fluent/agrammatic variant
• semantic variant
• (logopenic-phonological variant)
• Movement disorder variants
• FTD-MND
• CBD-PSP
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Pick, 1892Lund, Manchester1987-89
Mesulam, 1982
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Gorno Tempini et al., 2004
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Agosta et al., submitted
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Minimal assessment
• Quantitative/qualitative production analysis
• Single word naming and comprehension
• Sentence repetition
• Syntactic comprehension
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Articulation Naming Word
comprehension
Repetition Syntactic
comprehension
NFPA impaired impaired-
PP
preserved impaired impaired
SD preserved impaired-
SP
impaired preserved rel. preserved
LPA preserved
(slow-
hesitant)
impaired-
PP
preserved impaired impaired
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Right temporal variant of semantic
dementia• Presentation with visuo-perceptual impairment (often
prosopoagnosia)
• Progression to widespread semantic dysfunction
• Differential diagnosis with posterior cortical atrophy and
Lewy body dementia
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The fronto-temporal spectrum
• Behavioural variant
• Progressive aphasia
• non-fluent/agrammatic variant
• semantic variant
• (logopenic-phonological variant)
• Movement disorder variants
• FTD-MND
• CBD-PSP
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FTD-MND
• MND/dementia: usually bvFTD
• MND/aphasia: usually non-fluent/agrammatic
• Cognitive disorders in non-demented MND patients
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CB-PSP syndrome
• Very low correlation with CBD pathology (Ling et al.,
2010)
• At the clinical level, there is a huge overlap between the
CB and PSP phenotype
• A careful examination of praxis is required
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Conclusions
• A correct definition of the clinical phenotype requires an
in-depth evaluation based on neuropsychology and
neuroimaging
• The clinical phenotype reflect exclusively the topography
of brain involvement by the pathological process
• The relationship between the clinical and imaging
phenotype is only probabilistic