Table 1.
International Consensus Criteria for Behavioral-Variant Frontotemporal Dementia (FTD)a
| Neurodegenerative disease |
| Must be present for any FTD clinical syndrome |
| Shows progressive deterioration of behavior and/or cognition by observation or history |
| Possible behavioral-variant FTD |
| Three of the features (A–F) must be present; symptoms should occur repeatedly, not just as a single instance: |
| A.Early behavioral disinhibition |
| B.Early apathy or inertia |
| C.Early loss of sympathy or empathy |
| D.Early perseverative, stereotyped, or compulsive/ritualistic behavior |
| E.Hyperorality and dietary changes |
| F.Neuropsychological profile: executive function deficits with relative sparing of memory and visuospatial functions |
| Probable behavioral-variant FTD |
| All the following criteria must be present to make the diagnosis: |
| A.Meets criteria for possible behavioral-variant FTD |
| B.Significant functional decline |
| C.Imaging results consistent with behavioral-variant FTD (frontal and/or anterior temporal atrophy on CT or MRI or frontal hypoperfusion or hypometabolism on SPECT or PET) |
| Definite behavioral-variant FTD |
| Criteria A and either B or C must be present to make the diagnosis: |
| A.Meets criteria for possible or probable behavioral-variant FTD |
| B.Histopathological evidence of frontotemporal lobar degeneration on biopsy at postmortem |
| C.Presence of a known pathogenic mutation |
| Exclusion criteria for behavioral-variant FTD |
| Criteria A and B must both be answered negatively; criterion C can be positive for possible behavioral-variant FTD but must be negative for probable behavioral-variant FTD: |
| A.Pattern of deficits is better accounted for by other nondegenerative nervous system or medical disorders |
| B.Behavioral disturbance is better accounted for by a psychiatric diagnosis |
| C.Biomarkers strongly indicative of Alzheimer's disease or other neurodegenerative process |
a
Adapted with permission from Piguet et al.2
Abbreviations: CT=computed tomography, MRI=magnetic resonance imaging, PET=positron emission tomography, SPECT=single-photon emission computed tomography.