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Review
. 2018 Apr 25;18(1):54.
doi: 10.1186/s12883-018-1055-y.

Creutzfeldt-Jakob disease with Alzheimer pathology, presenting with status epilepticus following repeated partial seizures: a case report and literature review

Keita Miyake  1 , Takashi Hara  2 , Etsuko Oshima  1 , Kiyohiro Kawada  1 , Hideki Ishizu  3 , Yuko Yamauchi  3 , Katsuya Satoh  4 , Tetsuyuki Kitamoto  5 , Shintaro Takenoshita  1 , Seishi Terada  6 , Norihito Yamada  1
Affiliations
Review

Creutzfeldt-Jakob disease with Alzheimer pathology, presenting with status epilepticus following repeated partial seizures: a case report and literature review

Keita Miyake et al. BMC Neurol. .

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease. Common first symptoms are dementia, cerebellar ataxia, visual disturbance, and psychiatric symptoms. Seizure as the first symptom of CJD is a very rare finding.

Case presentation: We experienced an elderly woman who presented initially with status epilepticus following repeated partial seizures in the course of Alzheimer disease (AD) dementia. Anti-convulsive therapy had no effect. Autopsy revealed definite CJD with AD pathology.

Coclusions: This is the first reported CJD case presenting with status epilepticus in the course of AD dementia.

Keywords: Alzheimer disease; Creutzfeldt-Jakob disease; Epilepsy; Seizure; Status epilepticus.

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Conflict of interest statement

Ethics approval and consent to participate

This case report was approved by the Ethical Committee of the Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Zikei Institute of Psychiatry. Autopsy and gene analysis were carried out after written informed consent was obtained from the daughter of the patient.

Consent for publication

Written informed consent was obtained from the daughter of the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Medical history of the patient displayed on the timeline
Fig. 2
Fig. 2
Head MRI (axial, diffusion-weighted image) of the patient. Diffusion-weighted brain images showed high intensity lesions, separately in the cortex of the left temporo-parietal lobe, right parietal lobe, left frontal lobe, left insula, and left basal ganglia
Fig. 3
Fig. 3
Electroencephalogram of the patient. Electroencephalogram showed periodic synchronous discharge
See this image and copyright information in PMC

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