Semantic dementia, a many-worded disorder
O'Neill, S.; Andreotti, M.; de Simone, V.
Revista de Neurologia 43(11): 685-689
2006
ISSN/ISBN: 0210-0010 PMID: 17133330 Document Number: 604966
Semantic dementia is a progressive, relatively selective disorder affecting the semantic system with involvement of the verbal and non-verbal functions. The clinical picture is well characterised despite the confusion that may be generated by the different ways of classifying it. AIM. To determine the clinical, neurolinguistic, imaging and pathological features of this progressive language disorder. Evaluation of language reveals above all the existence of semantic paraphasias, disorders affecting the comprehension of isolated words and surface dyslexia. Flow of speech, complex syntactic comprehension and grammar are preserved. Both episodic and autobiographic memory are close to normality. Both the clinical signs and symptoms and imaging studies agree on the fact that the most heavily affected area is the anteroinferomedial region of the temporal lobe on a bilateral scale but with predominance of the left-hand side. Pathologically, in most cases positive intraneuronal ubiquitin inclusions are observed like those described in motor neuron diseases. Semantic dementia constitutes a diagnosis challenge, mainly from the neuropsychological point of view. Further advances towards reaching a diagnosis would allow us to determine which area is mainly affected and, in the future, to find an effective treatment for this progressive, degenerative disorder.