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.
Highlights
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease caused by accumulated misfolded proteins, with their deposition in the cortex, striatum, and thalamus (1)
Inclusion criteria were as follows: (1) clinical situation when the onset of CJD was initially misdiagnosed as a stroke; (2) subsequent workup indicated probable or definite CJD according to the international criteria (5); or (3) detailed clinical case description including magnetic resonance imaging (MRI), electroencephalogram (EEG), and cerebrospinal fluid (CSF) studies when available were consistent with the diagnosis of CJD
In 20 cases, sporadic CJD was diagnosed, in two cases, the disease was familial [cases 6 (6) and 7 (7)], and in another case, a mutation was identified characteristic of familial CJD, but no other cases were reported in his family [case 20 (8)] (Table 1)
Summary
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease caused by accumulated misfolded proteins (prions, PrPSc), with their deposition in the cortex, striatum, and thalamus (1). The clinical hallmarks of the disease are progressive dementia, behavioral disorders, epileptic seizures, ataxia, myoclonus, and a variety of other movement disorders (2). Stroke-like onset of CJD is considered as very rare. Only a single case has been identified in a recent series of cases from China (3). In an earlier important review 5.6% (30/532) of patients with “probable” or “definite” CJD collected in the UK from 1970 to 1993 had stroke-like onset (4).
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