Case Report

A Case of Creutzfeldt-Jakob Disease with Stroke-Like Onset
Kosuke Okamoto, MD, Takato Abe, MD, PhD, and Yoshiaki Itoh, MD, PhD

Creutzfeldt-Jakob disease (CJD) is a prion disease characterized by rapidly progressive dementia that is often followed by behavioral disturbances, ataxia, myoclonus,
and akinetic mutism. The initial symptoms of CJD reportedly vary, but the onset is
usually gradual. Here, we report a case of CJD with a sudden, stroke-like onset of
right hemiparesis to alert readers that CJD can mimic a stroke during its early stage.
Key Words: Creutzfeldt-Jakob disease (CJD)—stroke mimic—sudden onset—
prion disease
© 2020 Elsevier Inc. All rights reserved.

Case Report
An 84-year-old female with no family history of neurological disease experienced weakness in her right arm
upon awakening one morning. Three days later, she also
developed weakness in her right leg. She was referred to
our hospital and was admitted 21 days after the onset of
her symptoms after her condition failed to improve. A neurological examination revealed agraphia, miscalculation,
aphasia, right tactile extinction, and right hemiplegia.
An initial cranial magnetic resonance imaging (MRI)
examination showed bilateral scattered cortical hyperintensities on fluid-attenuated inversion-recovery (FLAIR) and
diffusion-weighted images (DWI) and apparent diffusion
coefficients (ADC) hypointensity, with findings that were
similar to those for multiple ischemic strokes (Fig 1, A-D).
Cardiac ultrasonography did not reveal any embolic sources. We initially suspected a cardiac embolism and started
the administration of an anticoagulant to prevent stroke.
Over the following weeks, she became lethargic and globally aphasic and developed myoclonus almost 30 days after
onset. On follow-up MRI scans performed on day 41, the
cortical signal hyperintensities on DWI and the low
From the Department of Neurology, Osaka City University Graduate School of Medicine, Osaka City, Osaka, Japan.
Received November 25, 2019; revision received February 16, 2020;
accepted February 24, 2020.
Address correspondence to Takato Abe, MD, PhD, Department of
Neurology, Osaka City University Graduate School of Medicine,
Asahimachi 1-4-3, Abenoku, Osaka City, Osaka, Japan. E-mail:
tk-abe@umin.net.
1052-3057/$ - see front matter
© 2020 Elsevier Inc. All rights reserved.
https://doi.org/10.1016/j.jstrokecerebrovasdis.2020.104788

intensity on the ADC map were still persistent (Fig 1, E,F).
Her electroencephalogram (EEG) on day 69 showed a periodic synchronous discharge (Fig 1, G).
Her condition continued to deteriorate, and she eventually developed akinetic mutism. A real-time quakinginduced conversion test of her cerebrospinal fluid was
positive for 14-3-3 protein. No mutations in prion protein
genes were found in a gene analysis. We diagnosed her as
having Creutzfeldt-Jakob disease (CJD). She was transferred to another hospital on day 103 and died 112 days
after the onset of her symptoms.

Discussion
As this patient presented with the sudden onset of hemiparesis and the MRI findings showed areas of high-intensity
DWI with corresponding low ADC areas, a cerebrovascular
event was initially suspected. The absence of basal ganglia
hyperintense lesions are thought to be atypical for CJD at
that stage of the clinical course (Fig 1, D), although some
CJD patients do not show hyperintense lesions in the basal
ganglia, especially during the early phase.1
Only a few reports have described ADC map findings
in CJD cases with a sudden, stroke-like onset.2-4 In 2 of
these cases, ADC hypointensities correlated with DWI
subcortical hyperintensities were shown.2,3 The reduced
ADC increases over time and becomes normalized at 510 days after a cerebral infarction, eventually becoming
elevated during the chronic state,5 while DWI hyperintensities sometimes take as long as 2 months to resolve.6 In
the presently reported case, serial MRI scans showed a
persistent abnormality in the ADC map, which was atypical for cerebral ischemia and led us to a correct diagnosis.

Journal of Stroke and Cerebrovascular Diseases, Vol. 29, No. 6 (June), 2020: 104788

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K. OKAMOTO ET AL.

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Figure 1. Cranial MRI and EEG. In the cranial MRI performed at the time of admission (A-D), diffusion-weighted imaging (DWI; A, D) and a coronal FLAIR
image (B) showed bilateral scattered hyperintense areas. A low-intensity area that was correlated with a high-intensity DWI area is indicated by the white arrow
on the ADC map (C). DWI hyperintensities were not detected in the basal ganglia (D). A follow-up MRI was performed on day 41 (E, F). The high-intensity
DWI area (F) and the low-intensity ADC area (E) were still present. An electroencephalogram showed periodic synchronous discharges on the 69th day after disease onset (G). Abbreviations: ADC, apparent diffusion coefficients; MRI, magnetic resonance imaging.

The mechanism responsible for the reduced diffusion in
CJD is thought to be associated with local compromises in
energy metabolism, eg, acute strokes, after prolonged ictal
activity; this mechanism in CJD is further supported by
the impairment of mitochondrial function in dying neurons.2,7 Also, DWI hyperintensity might be due to the
deposition of prion protein, which somehow restricts the
free diffusion of water.8,9
Serial neuroradiological and laboratory examinations to
rule out CJD are recommended in stroke patients with
atypical features, such as persistent low intensities on
ADC maps.

Acknowledgments
The authors would like to thank Prof. Satoh (Nagasaki
University Graduate School of Medicine) for analyzing the
14-3-3 protein and prion genes.

Conflict of Interest
All authors have no conflict of interest for this article.

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CREUTZFELDT-JAKOB DISEASE (CJD) WITH STROKE-LIKE SYMPTOMS
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