Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships

Abstract

Only a few case reports of stroke-like onset of Creutzfeldt-Jakob disease (CJD) have previously been published. We aimed to analyze the neurological, imaging, electroencephalographic (EEG), and laboratory features of patients with this very rare phenomenon. Here, we review the clinical characteristics, onset features, and clinical course variants of stroke-like CJD in 23 such patients. The median age of the patients was 71 years (range: 56–84 years); 12 were women. In 20 patients, CJD was sporadic. Thirteen patients developed apoplexy-like onset of symptoms, whereas the others had prodromal non-specific complaints. Most often the patients manifested with pyramidal signs (n = 13), ataxia (n = 9), and aphasia (n = 8). On MRI DWI sequence, all subjects had abnormal hyperintensities in various parts of the cerebral cortex, striatum, or thalamus, while EEG detected periodic triphasic waves only in 11. CSF 14-3-3 protein and total τ-protein were abnormal in 17 of 23 cases. All patients died, median lifespan being 2 months (range: 19 days−14 months). In conclusion, a complex of clinical, radiological, and laboratory manifestations of stroke-like onset of CJD is outlined. The clinical relationships between CJD and stroke are considered, in an attempt to highlight this rare presentation of an uncommon disease.

Is There Horizontal Transmission of Creutzfeldt-Jakob Disease?

“…An important strength of our study is the existence of a national registry of CJD since 1985. Limitations of our study may include possible insufficient accuracy of CJD diagnosis [31], since autopsies are very limited due to religious reasons, possible misdiagnosis of autoimmune [32] or vascular diseases of the brain [33], as well as nonregistration of CJD in the Israeli National Registry. Improvements in the methods of diagnosis of CJD achieved during the period of the study such as discovery of PRNP gene mutation [6][7][8], elaboration T2 FLAIR and DWI MRI, CSF tests for 14-3-3 and tau protein, and real-time quakinginduced conversion (RT-QuIC) could contribute to improved diagnosis of CJD and influence the statistical results.…”

Case Report: Extremely Early Detection of Preclinical Magnetic Resonance Imaging Abnormality in Creutzfeldt–Jakob Disease With the V180I Mutation

“…In fact, the patient was initially diagnosed with cerebral infarction. Interestingly, stroke-like onset of CJD has also been reported (12), and, accordingly, cerebral infarction is an important differential diagnosis in terms of radiological and clinical aspects. In addition to cerebral infarction, many differential diagnoses for cortical DWI features mimicking CJD have been reported, including hypoxic ischemic encephalopathy, hypoglycemia, autoimmunemediated encephalopathy, encephalitis, status epilepticus, hyperammonemia, and mitochondrial disorders (13); differential diagnosis can therefore be difficult, especially in the early disease stage with no or non-specific symptoms.…”