J Korean Geriatr Psychiatry.  2007 Dec;11(2):55-61.

Frontotemporal Lobar Degeneration

• Shin IS

Abstract

Frontotemporal lobar degeneration (FTLD) is a progressive dementia with prominent neuropsychiatric features, aphasia or both. FTLD predominantly affects the frontal and anterior part of temporal cortex. FTLD is classified into frontotemporal dementia (FTD), progressive nonfluent aphasia (PA), and semantic dementia (SD). FTLD is estimated to account for 20% of cases of degenerative dementia with presenile onset. This disease typically has onset in the mid- or early fifties. FTD is characterized by behavioral change and executive dysfunction, PA features a progressive nonfluent aphasia. SD is characterized by a progressive semantic aphasia and associative agnosia. Structural imaging shows atrophy of the frontal lobe and the anterior portion of the temporal lobe, bilaterally symmetric or asymmetric. Pathologically, FTLD can be classified into tau-positive pathology, tau-negative, ubiquitin positive pathology, dementia lacking distinctive histology. At present, there are no specific pharmacological therapies approved for use in any of the FTLD syndrome.

Keyword

Frontotemporal lobar degeneration; Frontotemporal dementia; Semantic dementia; Progressive nonfluent aphasia

MeSH Terms

Agnosia
Aphasia
Atrophy
Dementia
Frontal Lobe
Frontotemporal Dementia
Frontotemporal Lobar Degeneration*
Pathology
Primary Progressive Nonfluent Aphasia
Temporal Lobe
Ubiquitin
Ubiquitin