Frontotemporal Dementia History

From 1892 to 1906, Arnold Pick reported a series of cases in which patients developed cognitive and behavioral abnormalities in association with focal frontal and temporal

TABLE 15.4. Fact Summary—Frontotemporal Dementia

Typical age of onset 55-62 years

Sex ratio Men = women

Primary clinical/behavioral features Progressive disinhibition of behavior and loss of executive functioning Primary brain regions affected Frontal lobes and anterior temporal lobes

Neuropathology 1. Astrocytosis with or without Pick cells and Pick bodies

2. Microvacuolar pathology Neurochemistry Loss of serotonin

Primary treatment Selective serotonin reuptake inhibitors (proposed)

lobe atrophy. In 1911, Alzheimer discovered argyrophilic intraneuronal inclusions in similar cases and named these structures Pick bodies. In 1926, Pick's students Onari and Spatz coined the term Pick's disease to describe patients with frontotemporal atrophy and Pick bodies.

Interest in this subject waned until the 1980s when groups in Manchester, England (Neary et al., 1986), and Lund, Sweden (Brun, 1987; Gustafson, 1987), rediscovered a dementia stemming from focal frontal atrophy. These groups found on microscopic examination that the characteristic changes of Alzheimer's disease were absent in their patients and that multiple histological pictures (including Pick bodies in a minority of patients) could be found in patients with frontal atrophy. The Lund and Manchester groups coined the term frontotemporal dementia (FTD) to describe the syndrome seen in these patients and developed clinical criteria for the diagnosis of this illness (Brun et al., 1994; revised by Neary et al., 1998).

Mesulam (1982) renewed interest in cases of focal left temporal lobe atrophy with his description of primary progressive aphasia (PPA), a dysfluent language disorder. Snowden et al. (1989) described a third frontotemporal syndrome, semantic dementia (SD), a fluent language disorder marked by a loss of ability to appreciate word meaning and primarily associated with temporal lobe atrophy. More detailed descriptions of these three syndromes are available in Snowden et al. (1996) (see Table 15.4).

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