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Reduplicative paramnesia as an atypical form of presentation of a Heidenhain variant of Creutzfeldt-Jakob disease: A case report

Published online by Cambridge University Press:  05 September 2022

Saul Martinez-Horta Open the ORCID record for Saul Martinez-Horta [Opens in a new window] ,
Clara Naima Marzal-Espí ,
Silvia Cusó-García ,
Ignacio Aracil-Bolaños  and
Jaime Kulisevsky
Saul Martinez-Horta*
Affiliation:
Movement Disorders Unit, Neurology Department, Sant Pau Hospital, Barcelona, Spain Universitat Autònoma de Barcelona (U.A.B.), Medicine Department, Barcelona, Spain Institut d´Investigacions Biomèdiques Sant Pau (IIB-Sant Pau), Barcelona, Spain Centro de Investigación en Red-Enfermedades Neurodegenerativas (CIBERNED), Spain
Clara Naima Marzal-Espí
Affiliation:
Neurology Department, Fundació Hospital Sant Joan de Déu, Martorell, Barcelona, Spain
Silvia Cusó-García
Affiliation:
Neurology Department, Fundació Hospital Sant Joan de Déu, Martorell, Barcelona, Spain
Ignacio Aracil-Bolaños
Affiliation:
Movement Disorders Unit, Neurology Department, Sant Pau Hospital, Barcelona, Spain Universitat Autònoma de Barcelona (U.A.B.), Medicine Department, Barcelona, Spain Institut d´Investigacions Biomèdiques Sant Pau (IIB-Sant Pau), Barcelona, Spain Centro de Investigación en Red-Enfermedades Neurodegenerativas (CIBERNED), Spain
Jaime Kulisevsky
Affiliation:
Movement Disorders Unit, Neurology Department, Sant Pau Hospital, Barcelona, Spain Universitat Autònoma de Barcelona (U.A.B.), Medicine Department, Barcelona, Spain Institut d´Investigacions Biomèdiques Sant Pau (IIB-Sant Pau), Barcelona, Spain Centro de Investigación en Red-Enfermedades Neurodegenerativas (CIBERNED), Spain
*
Corresponding author: Saul Martinez-Horta, email: smartinezho@santpau.cat
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Abstract

A previously healthy woman began to present recurrent episodes of reduplicative paramnesia within her home and later structured visual hallucinations. The case was initially oriented as an incipient vascular dementia. Detailed anamnesis and neuropsychological examination suggested a rapidly progressive pattern of neuropsychological deficits mostly attributable to parieto-occipital disturbances with some component of fronto-temporal involvement. Subsequently, cerebellar symptoms were added. Although the initial imaging studies were inconclusive, the MRI performed during follow-up showed a series of findings compatible with a prion disease. Based on the neuropsychological and clinical features and the imaging pattern, the diagnosis of Heidenhain Variant of Creutzfeldt-Jakob disease was established. This is the first report of a Heidenhain Variant of Creutzfeldt-Jakob disease presenting as a reduplicative paramnesia as the first manifestation of this disease.

Keywords

Reduplicative paramnesiaCreutzfeldt-JakobHeidenhainCJDDementia
Type
Case Report
Information
Journal of the International Neuropsychological Society , Volume 29 , Issue 6 , July 2023 , pp. 615 - 619
Copyright
Copyright © INS. Published by Cambridge University Press, 2022

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