Creutzfeldt-Jakob Disease With Atypical Magnetic Resonance Imaging Features.
basal ganglia high signal intensity
cortical ribboning
creutzfeld-jakob disease
diffusion-weighted images
fatal outcome
fluid attenuation inversion recovery
human prion disease
neurodegenerative disease
neurological deficit
role of mri
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
02 Nov 2020
02 Nov 2020
Historique:
entrez:
7
12
2020
pubmed:
8
12
2020
medline:
8
12
2020
Statut:
epublish
Résumé
Creutzfeld-Jakob disease (CJD) is a rare neurodegenerative condition characterized by rapid progression and fatal outcomes. Patients with progressive dementia and associated atypical features should be investigated, especially with the MRI brain for CJD. Cortical ribboning on diffusion-weighted MRI images is a very crucial diagnostic sign for CJD. Here we present a case of a 52-year-old woman admitted to the hospital after a seizure episode and two-month history of altered mental status. She presented with a 40-minute episode of status epilepticus, necessitating admission to the intensive care unit. Head CT showed no acute intracranial abnormalities, and MRI showed generalized brain atrophy. Electroencephalography (EEG) demonstrated an intermittent slowing of the left hemisphere. Two weeks after admission, she got discharged. Four days later, she presented to the hospital after being found disoriented in a park. MRI showed ventricular dilation and a questionable focus of restricted diffusion in the left thalamus posteriorly. CJD protein panel was collected. Three days after discharge, she was brought to the hospital, and CJD protein testing revealed the presence of 14-3-3 protein, elevated T-tau, and negative real-time quaking-induced conversion (RT-QuIC). The National Prion Disease Surveillance Center reviewed her case, and the CJD diagnosis was confirmed.
Identifiants
pubmed: 33282571
doi: 10.7759/cureus.11294
pmc: PMC7710345
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e11294Informations de copyright
Copyright © 2020, Qamar et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
Références
Brain. 2004 Oct;127(Pt 10):2348-59
pubmed: 15361416
N Engl J Med. 2001 May 17;344(20):1516-26
pubmed: 11357156
Neuropathology. 2017 Apr;37(2):174-188
pubmed: 28028861
Arch Neurol. 1996 Feb;53(2):162-6
pubmed: 8639066
Neurology. 2006 Aug 22;67(4):637-43
pubmed: 16924018
Arch Neurol. 2003 Jun;60(6):813-6
pubmed: 12810484
AJNR Am J Neuroradiol. 2007 Jun-Jul;28(6):1114-8
pubmed: 17569970
AJNR Am J Neuroradiol. 2005 Jun-Jul;26(6):1551-62
pubmed: 15956529
J Clin Neurosci. 2000 May;7(3):195-202
pubmed: 10833615
Handb Clin Neurol. 2017;145:393-403
pubmed: 28987186
Neurology. 2009 Jun 9;72(23):1994-2001
pubmed: 19506221
Acta Neuropathol. 2017 Apr;133(4):559-578
pubmed: 28205010
J Clin Neurol. 2016 Jan;12(1):101-6
pubmed: 26541494
Cases J. 2008 Sep 09;1(1):146
pubmed: 18782431
Neurosurg Focus. 2015 Nov;39(5):E2
pubmed: 26646926
Pract Neurol. 2017 Apr;17(2):113-121
pubmed: 28153848
Brain. 2003 Jun;126(Pt 6):1333-46
pubmed: 12764055
Neurology. 2012 Oct 2;79(14):1499-506
pubmed: 22993290
Clin Neurophysiol. 2006 May;117(5):935-51
pubmed: 16442343