There are certain criteria from Rascovsky et al (2011) that indicate FTD:
l. BV- FTD
- Progressive increase in cognitive deficits (to be ascertained by own observations or external history) and / or behavioral deficits (Deuschl 2016).
ll. Possible BV- FTD
A possible BV- FTD is present if 3 of the symptoms A - F are present, persist or recur:
- A. Early onset behavioral disinhibition [one of the following symptoms (A.1.-A.3.) must be present].
- A. 1. socially inappropriate behavior
- A. 2. loss or decency of manners
- A. 3. impulsive, reckless, or thoughtless actions
- B. Early onset apathy or passivity [one of the following symptoms (B.1.-B.2.) must be present]
- B. 1. apathy
- B. 2. passivity
- C. Early loss of empathy or sympathy [one of the following symptoms (C.1.-C.2.) must be present]
- C. 1. responsiveness to the feelings and needs of others is markedly diminished
- C. 2. decreased interest in relationships, social contacts, and decrease in personal warmth
- D. Early ritualized/compulsive or perseverative stereotypic behavior [one of the following symptoms (D.1.-D.3.) must be present]
- D. 1. simple, repetitive movements
- D. 2. complex ritualized or compulsive behavior
- D. 3. speech stereotypies
- E. Changes in eating habits and hyperorality [one of the following symptoms (E.1.- E.3.) must be present]
- E. 1. change in previous food preferences
- E. 2. binge eating, increase in consumption of alcohol or nicotine
- E. 3. non-edible material is placed in the mouth and consumed
- F. Neuropsychological profile [all of the following symptoms (F.1.-F.3.) must be present]
- F. 1. deficit in tasks with an executive component
- F. 2. episodic memory is relatively preserved
- F. 3. visual-spatial performance is relatively preserved (Deuschl 2016)
lll. Probable BV- FTD:
For this, all of the following symptoms must be present to meet the criteria:
- A. The above criteria for probable BV- FTD are met.
- B. Significant progression of functional deficits (to be assessed by extraneous history or clinical scales (such as the Clinical Dementia Rating Scale or the Functional Activities Questionnaire) is evident.
- C. Imaging findings are consistent with a diagnosis of BV- FTD [one of the following features (C.1.-C.2.) must be present].
C. 1. frontal and / or anterior- temporal atrophy is evident on MRI or CT, respectively
C. 2. frontal and / or anterior- temporal shows hypoperfusion or hypometabolism in SPECT or PET, respectively (Deuschl 2016)
lV. BV- FTD with proven pathological changes of a FTLD.
Criterion A must be present along with criterion B or C:
A. The above criteria for a possible or probable BV- FTD are met.
B. Histopathological evidence of FTLD is found in a biopsy or post-mortem examination.
C. Presence of a known pathogenic mutation (Deuschl 2016).
V. Exclusion criteria for BV- FTD are:
Criteria A and B below may not be met for the diagnosis of BV- FTD. Criterion C may be positive for a possible BV- FTD and must be negative for a probable BV- FTD.
A. The pattern of deficits is better explained by another medical disorder or other nondegenerative nervous system disease.
B. The behavioral deficits are better explained by a psychiatric diagnosis.
C. Biomarkers strongly suggest other neurodegenerative processes or Alzheimer's disease (Deuschl 2016).