Abstract
Although there are no reports of pathological laughing and crying being observed in
patients with Creutzfeldt–Jakob disease (CJD), the author experienced three patients
with CJD with prion protein gene codon180 mutation (V180I CJD) who showed this characteristic
clinical finding. This finding was observed from the early disease stage in all 3
patients and continued for several months. Startle reaction was also remarkable in
all patients, although myoclonus was generally mild. The dissociation between the
startle reaction and myoclonus was suspected to be another feature of V180I CJD. The
pathological laughing and crying co-occured with the startle reaction and stopped
right before the onset of akinetic mutism, and the degree of both symptoms was almost
parallel during this period. On the basis of MRI and autopsy findings, pathological
laughing and crying was suspected of being induced by the widespread cerebral cortical
involvement that is characteristic of V180I CJD. From the present observations, the
author speculated that pathological laughing and crying may be a comparatively frequent
observation in V180I CJD patients.
Keywords
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Article info
Publication history
Published online: June 04, 2012
Accepted:
May 9,
2012
Received in revised form:
May 7,
2012
Received:
March 24,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
