Frontotemporal dementias

Lars Gustafson Neuropathology

Prevalence and age.characteristics Clinical features

Disordered.. .behaviour Affectiive..symptoms other.symptoms Dissolu—n ..of. language Physical.signs

Comparison ..between, ..FLD., and., .Eick's..„disease Dementia..in., mo.tor..neurone, disease Investigations

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lBra.i.n,,imag.ing

Assessment, noi£ogni.tive..imEairm,e,nt Differential. diagnosis Classification

Aeti.ology..and., pathogenesis Treatment, ..and...care Chapter References

Nosological classification of organic dementia is based on current knowledge and theories of aetiology, clinical picture, the pathological substrate, and the predominant location of brain damage. This chapter is concerned with dementia caused by a degenerative disease primarily affecting the frontal and temporal lobes called frontal-lobe dementia(1 or frontotemporal dementia (FTD ).(2) The terminology should be viewed from a historical perspective. The relationship between localized cortical atrophy within the frontal and temporal lobes in dementia and symptoms of aphasia was first reported by Pick in a series of publications, starting in 1892.(3) The pathological account of this lobar degeneration by Alzheimer in 1911 described inflated, 'ballooned' neurones (Pick cells) and argentophilic globes (Pick bodies),(4) and in the 1920s this clinicopathological entity was named Pick's disease.(5)

However, 'pure' Pick's disease is rare, and in recent decades attention has been drawn to a much larger group of frontal-lobe dementias clinically similar to Pick's disease but lacking the pathological characteristics of Pick's disease and Alzheimer's disease. (6Z and 8) The disease was named frontal-lobe degeneration of non-Alzheimer type (FLD)(6) to mark its dissimilarity to Alzheimer's disease. Alternative designations are 'dementia lacking distinctive histology' (9) and 'primary degenerative dementia without Alzheimer pathology'.(10) In 1994 the Lund-Manchester consensus delineated the clinical and neuropathological criteria for FTD, with Pick's disease, FLD, and motor neurone disease with dementia as the main constituents.(2) Other diseases, such as progressive non-fluent aphasia, (H) and FTD with parkinsonism linked to chromosome 17 (FTDP-17) are considered as belonging to the same clinicopathological spectrum. (12)

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