Investigations into Mechanisms of Word Finding John Hart, Jr ...

Investigations into Mechanisms of Word Finding John Hart, Jr ...

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 Picks 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, Picks 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 Picks 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

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