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J Neurol Sci. 2009 Sep 15;284(1-2):120-3. doi: 10.1016/j.jns.2009.04.021. Epub 2009 May 12.

Abnormal laughter-like vocalisations replacing speech in primary progressive aphasia.

Author information

  • 1Dementia Research Centre, Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK.

Abstract

We describe ten patients with a clinical diagnosis of primary progressive aphasia (PPA) (pathologically confirmed in three cases) who developed abnormal laughter-like vocalisations in the context of progressive speech output impairment leading to mutism. Failure of speech output was accompanied by increasing frequency of the abnormal vocalisations until ultimately they constituted the patient's only extended utterance. The laughter-like vocalisations did not show contextual sensitivity but occurred as an automatic vocal output that replaced speech. Acoustic analysis of the vocalisations in two patients revealed abnormal motor features including variable note duration and inter-note interval, loss of temporal symmetry of laugh notes and loss of the normal decrescendo. Abnormal laughter-like vocalisations may be a hallmark of a subgroup in the PPA spectrum with impaired control and production of nonverbal vocal behaviour due to disruption of fronto-temporal networks mediating vocalisation.

PMID:
19435636
[PubMed - indexed for MEDLINE]
PMCID:
PMC2729814
Free PMC Article
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