Neurological Sciences

, Volume 40, Issue 5, pp 1073–1075 | Cite as

A new neurobehavioral phenotype of familial Creutzfeldt–Jakob disease: impaired theory of mind

  • Anna Rita GiovagnoliEmail author
  • Giulia Tallarita
  • Annalisa Parente
  • Angelo Del Sole
  • Giuseppe Di Fede
Letter to the Editor

Dear Editor-in-Chief,

Creutzfeldt–Jakob disease (CJD) is a neurodegenerative condition related to proteinaceous particles (prions) leading to neuron loss, astrocytic reactions, and spongiform changes. About 5–15% of cases are familial (fCJD), while up to 95% are sporadic and few cases are linked to iatrogenic transmission or bovine spongiform encephalopathy [1]. The phenotypes are heterogeneous between and within these groups. Common symptoms are dementia, ataxia, myoclonus, hallucinations, and insomnia. Dementia involving language, executive and visual functions has been associated with frontal, temporal, or parietal degeneration [2]. However, its fast clinical course usually prevents to characterize specific neurobehavioral phenotypes linked to defined CJD forms.

Theory of mind (ToM), a main aspect of social cognition, allows to represent and understand one’s own and others’ mental states [3]. The basal ganglia, cerebellum, amygdala, prefrontal medial and inferior cortex, orbital...


Compliance with ethical standards

Conflict of interests

The authors declare that they have no competing interests.

Ethical publication statement

We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. The study has been performed in accordance with the 1964 Declaration of Helsinki and its later amendments. The patient gave informed consent to the clinical and instrumental examinations.


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Copyright information

© Springer-Verlag Italia S.r.l., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Diagnostics and Applied TechnologyFondazione IRCCS Istituto Neurologico Carlo BestaMilanItaly
  2. 2.Nuclear Medicine Unit, ASST Santi Paolo e Carlo and Department of Health SciencesUniversity of MilanMilanItaly

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