Sporadic Creutzfeldt-Jakob disease with tau pathology mimicking new-onset refractory non-convulsive status epilepticus: Case report and review of the literature.
Creutzfeldt-Jakob disease
non-convulsive
periodic sharp wave complexes
status epilepticus
tau
Journal
European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311
Informations de publication
Date de publication:
04 2021
04 2021
Historique:
received:
13
07
2020
accepted:
29
10
2020
pubmed:
3
11
2020
medline:
13
8
2021
entrez:
2
11
2020
Statut:
ppublish
Résumé
The aim of our study is to review the relationship between NCSE and sCJD. Creutzfeldt-Jakob disease (CJD) is the most common form of human prion disease. Electroencephalography (EEG)-detected changes such as periodic sharp wave complexes, superimposable to those seen in non-convulsive epileptic status (NCSE), have only rarely been described at CJD onset, especially in sporadic CJD (sCJD) cases. We describe clinical, EEG, cerebrospinal fluid (CSF) and neuroimaging findings of a confirmed case of sCJD with tau pathology, initially diagnosed as NCSE. We performed a literature review in PubMed of previous publications on both sCJD and NCSE. An 82-year-old woman with no medical history presented with a 2-week rapidly progressive neurological disorder, with motor aphasia, myoclonus, pyramidalism, and left posterior alien hand. EEG showed periodic sharp waves on right frontal regions, so anti-epileptic treatment was started. CSF results were normal. Brain magnetic resonance imaging demonstrated hyperintensity of the right cerebral cortex in diffusion sequences. Due to suspected new-onset refractory status epilepticus (NORSE), corticosteroid treatment was started, without clinical improvement. Necropsy results confirmed sCJD with tau pathology. The literature review identified 14 references including a total of 18 cases with NCSE as the presenting symptom of sCJD; the clinical and results in complementary tests were compiled into a table. Sporadic CJD should be considered in the differential diagnosis of patients with rapid cognitive decline and EEG changes consistent with NCSE. The wide heterogeneity in the etiology of NCSE, including autoimmune disorders, especially NORSE, suggests immunotherapy should be initiated based on a good risk-benefit balance. Some cases of sCJD, such as the present case with tau pathology, may mimic this clinico-electrical course.
Sections du résumé
BACKGROUND AND PURPOSE
The aim of our study is to review the relationship between NCSE and sCJD. Creutzfeldt-Jakob disease (CJD) is the most common form of human prion disease. Electroencephalography (EEG)-detected changes such as periodic sharp wave complexes, superimposable to those seen in non-convulsive epileptic status (NCSE), have only rarely been described at CJD onset, especially in sporadic CJD (sCJD) cases.
METHODS
We describe clinical, EEG, cerebrospinal fluid (CSF) and neuroimaging findings of a confirmed case of sCJD with tau pathology, initially diagnosed as NCSE. We performed a literature review in PubMed of previous publications on both sCJD and NCSE.
RESULTS
An 82-year-old woman with no medical history presented with a 2-week rapidly progressive neurological disorder, with motor aphasia, myoclonus, pyramidalism, and left posterior alien hand. EEG showed periodic sharp waves on right frontal regions, so anti-epileptic treatment was started. CSF results were normal. Brain magnetic resonance imaging demonstrated hyperintensity of the right cerebral cortex in diffusion sequences. Due to suspected new-onset refractory status epilepticus (NORSE), corticosteroid treatment was started, without clinical improvement. Necropsy results confirmed sCJD with tau pathology. The literature review identified 14 references including a total of 18 cases with NCSE as the presenting symptom of sCJD; the clinical and results in complementary tests were compiled into a table.
CONCLUSIONS
Sporadic CJD should be considered in the differential diagnosis of patients with rapid cognitive decline and EEG changes consistent with NCSE. The wide heterogeneity in the etiology of NCSE, including autoimmune disorders, especially NORSE, suggests immunotherapy should be initiated based on a good risk-benefit balance. Some cases of sCJD, such as the present case with tau pathology, may mimic this clinico-electrical course.
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
1385-1391Informations de copyright
© 2020 European Academy of Neurology.
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