Introduction
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive, fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. sCJD can develop with typically neurologic symptoms like cognitive impairment, ataxia, myoclonus.
As the illness progresses, mental deterioration and motor weakness becomes worsened and people may develop systemic diseases like respiratory failure, pneumonia or other infections. They are generally the cause of death and patients die within one year.
We experienced a case of sCJD misdiagnosed as recurrent stroke because the patient with local high signals on brain diffusion-weighted magnetic resonance imaging (DWI) presented hemiparesis as initial symptoms and developed aphasia along the course.
Case report
A 70-year-old female patient who presented right hemiparesis, showed high signals in the left frontal and temporal lobe area on brain DWI. (Fig. 1)
She had no prior history and family history of brain disorder. Mental was alert and cognition was good. Muscle strength, rated using the medical research council (MRC) scale, was graded 4/4 in the right upper and lower extremities. There is no abnormal finding in complete blood cell profile, electrolytes, liver function test, renal function test at admission date. She was initially diagnosed with cerebral infarction and was started on aspirin, clopidogrel, atorvastatin.
Since twelve days after admission, her mental changed to drowsy temporarily and her MRC of right extremities got worsened as 1 to 2. DWI revealed additional high signals in the left frontal and temporal lobe and both basal ganglia area, which led to the diagnosis of recurrent stroke. (Fig. 2)
However, then six days later, she has developed Broca's aphasia and cognition got worsened as poor and presented myoclonus on right upper extremity and left lower extremity. DWI revealed high signals in both cortex and basal ganglia, particulary at putamen. (Fig. 3)
She has no family history of Creutzfeldt-Jakob disease and there is no probability of iatrogenic Creutzfeldt-Jakob disease so she was diagnosed with sCJD based on the clinical features and brain imaging.
Discussion
Variable symptoms can present as initial symptom of sCJD. However It is rare that hemiparesis presents as initial symptom and aphasia develops along the course so is suspected of a stroke like this case. sCJD can reveals high signal in not only the bilateral area but the unilateral area on brain DWI.
Any patient who presents hemiparesis with high signal in the unilateral area on brain imaging should be considered as sCJD. And this case suggests that we should consider the possibility that a patient who gets hemiparesis worsened and develops aphasia has atypical sCJD as well as recurrent stroke. |
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Figure 1. High signals in the left frontal and temporal lobe area on brain diffusion-weighted magnetic resonance imaging
Figure 2. High signals in the left frontal and temporal lobe and both basal ganglia area on brain diffusion-weighted magnetic resonance imaging
Figure 3. High signals in the both cortex and basal ganglia, particulary at putamen on brain diffusion-weighted magnetic resonance imaging
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