# Presentation: 245694

## Frontotemporal Dementia

**American Academy of Neurology**
**John Hart, Jr., M.D.**

## Frontotemporal Dementia

**Definition: clinicopathologic condition consisting of deterioration of personality and cognition assoc. with prominent frontal and temporal lobe atrophy**
**Accounts for up to 3-20% of dementias**
**Third behind AD and Lewy Body Dementia in neurodegenerative dementing illnesses**

## Prevalence

**Mean age of onset 52.8 ****(Ratnavalli et al. Neurology 2002;58:1615-1621)**** **
**Male preponderance 14:3 in one study and M=F in others**
**Dementia prevalence of 81 per 100,000 (95% CI, 62.8 to 104.5) in the 45-64 year age group**
**Prevalence of AD and FTD in 45-64 age group same at 15 per 100,000 (8.4-27.0)**

## Frontotemporal Dementia

**Thus, common cause of dementia in younger population**
**Greater caregiver burden and increased dependency and health care costs**

## Frontotemporal Dementia

**Established clinical consensus criteria ****(The Lund and Manchester Groups, J Neurol Neurosurg Psychiatry 1994;57:416-418; Neary et. al, Neurology 1998;51:1546-1554):**
**Core features **
**Insidious onset and slow progression**
**Early decline of **
**Social interpersonal conduct**
**Regulation of personal conduct**
**Insight**
**Early emotional blunting**

## Frontotemporal Dementia

**Supportive features:**
**Decline in personal hygiene and grooming**
**Mental rigidity and inflexibility**
**Distractibility and impersistence**
**Hyperorality**
**Perseverative behavior**
**Speech and language **

## Frontotemporal Dementia

**Neuropsychology:**
**Impaired frontal lobe tests in absence of severe amnesia, aphasia, or visuospatial deficits**
**Imaging: **
**Atrophy or decreased uptake in the frontal or anterior temporal lobes (bilateral or unilateral) by MRI, CT, PET, SPECT**** ****(The Lund and Manchester Groups, J Neurol Neurosurg Psychiatry 1994;57:416-418; Neary et. al, Neurology 1998;51:1546-1554)**

## Frontotemporal Dementia

## Frontotemporal Dementia

## Frontotemporal Dementia

**Recent study showed of 42 FTD cases, 19 had at least 1 other family member affected ****(Chow et al., Arch Neurol. 1999;56:817-822)**
**1/3 had a positive family history in this study, but others much lower**
**Appears to be inherited in autosomal dominant fashion in those patients**
**Linked to chromosome 17 (tau gene) and chromosome 3**
**Association with motor neuron disease (ALS)**

## Pathology

**Prominent frontal and temporal lobar atrophy**
**Atrophy may be associated with Pick’s bodies, tauopathy, nonspecific superficial cortical neuron loss (DLDH)**

## Frontotemporal Dementia

## Frontotemporal Dementia

**Term is conventionally used in clinical and pathological diagnoses in standard clinical setting**
**No other specific code comes reasonably close to capturing disease**
**Dementia or AD diagnosis (290 or 331) ignores established diagnostic criteria and allows no tracking of FTD**

## Frontotemporal Dementia

**Differs from the other codes including AD, frontal dementia, Pick’s disease **
**Use of these codes inappropriate as not capture the age of onset, duration of illness, genetic factors, and impact on caregiver, society, and economics**
**AD ******** older, different duration, less clear genetics**
**Frontal dementia ******** no temporal lobe involvement, genetics differ**
**Pick’s disease ******** not capture spectrum of FTD **

## Frontotemporal Dementia

**Implications for FTD different from other dementias/AD:**
**Greater caregiver burden and increased dependency and health care costs**
**Patients see codes and think they have some other disease**

## Frontotemporal Dementia

## Frontotemporal Dementia