Evidence map›Paper›PMID 34060706›Full record

ArticleEuropean journal of neurology2021

Sporadic Creutzfeldt-Jakob disease with extremely long 14-year survival period.

Izaro Kortazar-Zubizarreta, Rebeca Ruiz-Onandi, Arrate Pereda, Yerai Vado, Gonzalo González-Chinchon, Hasier Eraña, Guiomar Perez de Nanclares, Joaquín Castilla

Open access · hybridAbstract readCase Reports
In one paragraph

Article in European journal of neurology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
0.3field-weighted citation impact, top 44% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

5 citing papers in PubMed, 6 citations in OpenAlex.

  1. Review
  2. Article
  3. Article
  4. Article
  5. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors at 4 institutions in 1 country.

Izaro Kortazar-ZubizarretaDepartment of Neurology, Bioaraba Health Research Institute, Araba University Hospital-Txagorritxu, Vitoria-Gasteiz, Spain.ORCID 0000-0002-8107-660X
Rebeca Ruiz-OnandiDepartment of Pathology, Bioaraba Health Research Institute, Araba University Hospital-Txagorritxu, Vitoria-Gasteiz, Spain.ORCID 0000-0003-3667-4333
Arrate PeredaMolecular (Epi)Genetics Laboratory, Bioaraba Health Research Institute, Araba University Hospital, Vitoria-Gasteiz, Spain.ORCID 0000-0002-5442-7873
Yerai VadoMolecular (Epi)Genetics Laboratory, Bioaraba Health Research Institute, Araba University Hospital, Vitoria-Gasteiz, Spain.ORCID 0000-0003-4156-9159
Gonzalo González-ChinchonDepartment of Neurology, Bioaraba Health Research Institute, Araba University Hospital-Txagorritxu, Vitoria-Gasteiz, Spain.ORCID 0000-0001-5474-8717
Hasier ErañaCentre for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.ORCID 0000-0001-8776-4211
Guiomar Perez de NanclaresMolecular (Epi)Genetics Laboratory, Bioaraba Health Research Institute, Araba University Hospital, Vitoria-Gasteiz, Spain.ORCID 0000-0002-2424-5294
Joaquín CastillaCentre for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain.ORCID 0000-0002-2216-1361
Hospital Txagorritxu · ESHospital Universitario Araba · ESEuskadiko Parke Teknologikoa · ESIkerbasque · ES

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

background and purposeSporadic Creutzfeldt-Jakob disease is a rapidly progressing and highly variable neurodegenerative disease with heterogeneous clinical presentation and a median survival time from diagnosis to death of 4-6 months.

methodsWe report a rare case of a 61-year-old woman with a history of initially rapidly progressive dementia, with subsequent development of pyramidal and extrapyramidal signs and with an unusually long survival period of 14 years. Initial magnetic resonance imaging evaluation, single-photon emission computed tomography, and electroencephalogram did not show relevant alterations.

resultsThe postmortem examination of the brain showed diffuse spongiform change, gliosis, and neuronal loss along with abnormal immunostaining of prion protein in the grey matter, especially in the cerebellum. Indirect PRNP genetic analysis was negative.

conclusionsThis case is, to our knowledge, the sporadic Creutzfeldt-Jakob disease patient with the longest survival period ever documented. This surprisingly long duration highlights the importance of histopathological confirmation with brain autopsies for suspected cases, as the disease can easily be misdiagnosed in such slowly progressing cases.

Indexed as

Creutzfeldt-Jakob SyndromeNeurodegenerative DiseasesPrionsBrainFemaleHumansMagnetic Resonance ImagingMiddle AgedPrionsCreutzfeldt-Jakob diseaselong survivalneuropathologysporadic CJD

Identifiers

PMID34060706
PMCPMC8457091
OpenAlexW3165334782

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.