Abstract

DONOSO S, Archibaldo; GONZALEZ V, Rafael; GONZALEZ R, Pablo  and  PUGIN A, Angela. Semantic dementia: Report of two cases. Rev. chil. neuro-psiquiatr. [online]. 2008, vol.46, n.4, pp. 280-287. ISSN 0717-9227.  http://dx.doi.org/10.4067/S0717-92272008000400005.

Frontotemporal dementia is a neurodegenerative condition that presents with a number of distinct behavioral phenotypes. One of them is semantic dementia (SD), where exists a profound impairment for semantic knowledge related to atrophy of temporal poles. Pathologically, in most cases positive intraneuronal ubiquitin and tau negative inclusions are observed. SD is characterized by fluent, effortless, grammatical speech which lacks informational content, with limited and repetitive content, as well as semantic paraphasias. Also, patients may present with associative visual agnosia, surface dyslexia or dysgraphia, behavioral alterations. Both episodic and autobiographical memory are close to normality. Two female patients with fluent progressive aphasia are reported; they failed in a simple test of semantic association (to point to one of four objects with lesser relation to others). Autobiographical memory was fair. SD can be wrongly diagnosed as left-sided variant of Alzheimer's disease; absence of episodic amnesia and parietal defects may be useful for clinical diagnosis.

Keywords : Semantics; frontotemporal dementia; semantic dementia; progressive fluent aphasia.