Stroke-Like Presentation in a Case of Creutzfeldt-Jakob Disease

Szabo, Kristina; Achtnichts, Lutz; Grips, Eva; Binder, Johannes; Gerigk, Lars; Hennerici, Michael G.; Gass, Achim

doi:10.1159/000080109

Cited by 15 publications

(7 citation statements)

References 16 publications

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“…Rapidly progressive cognitive dysfunction, focal myoclonus, EEG evolution to periodic sharp wave complexes, bilateral cortical DWI hyperintensities on brain MRI and 14‐3‐3 protein in the CSF were present in this case and were consistent with diagnostic criteria of sCJD 7,8 . Although a subacute onset is typical for sCJD, a stroke‐like onset has been recognized as a rare sCJD presentation 9–11 . In our case, this severe neurological picture was entirely driven by a COVID‐19‐ associated cytokine storm in both the CNS and bloodstream and was fully reversed by immunotherapy, leading to a complete neurological recovery.…”

Section: Discussionsupporting

confidence: 73%

“… 7 , 8 Although a subacute onset is typical for sCJD, a stroke‐like onset has been recognized as a rare sCJD presentation. 9 , 10 , 11 In our case, this severe neurological picture was entirely driven by a COVID‐19‐ associated cytokine storm in both the CNS and bloodstream and was fully reversed by immunotherapy, leading to a complete neurological recovery.…”

Section: Discussionmentioning

confidence: 58%

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COVID‐19‐associated immune‐mediated encephalitis mimicking acute‐onset Creutzfeldt‐Jakob disease

Beretta

Stabile

Balducci

et al. 2021

Ann Clin Transl Neurol

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We report a subtype of immune-mediated encephalitis associated with COVID-19, which closely mimics acute-onset sporadic Creutzfeldt-Jakob disease. A 64-year-old man presented with confusion, aphasia, myoclonus, and a silent interstitial pneumonia. He tested positive for SARS-CoV-2. Cognition and myoclonus rapidly deteriorated, EEG evolved to generalized periodic discharges and brain MRI showed multiple cortical DWI hyperintensities. CSF analysis was normal, except for a positive 14-3-3 protein. RT-QuIC analysis was negative. High levels of pro-inflammatory cytokines were present in the CSF and serum. Treatment with steroids and intravenous immunoglobulins produced EEG and clinical improvement, with a good neurological outcome at a 6-month follow-up.

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Section: Discussionsupporting

confidence: 73%

Section: Discussionmentioning

confidence: 58%

COVID‐19‐associated immune‐mediated encephalitis mimicking acute‐onset Creutzfeldt‐Jakob disease

Beretta

Stabile

Balducci

et al. 2021

Ann Clin Transl Neurol

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“…In our subject (case 23), as in some others [cases 2 (23), 9 (11), 15 (19), 16 (13), and 18 (14)], stroke-like CJD was initially diagnosed clinically as true vascular disease of the brain and CT or MRI imaging have suggested acute or chronic cerebrovascular disease. However, further clinical deterioration which occurred within 3-4 weeks forced the revision or repeat of MRI, which now revealed DWI cortical or subcortical hyperintensities and/or elevated CSF τ -protein or positive 14-3-3 protein.…”

Section: Discussionsupporting

confidence: 50%

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

et al. 2021

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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.

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“…The topic has been recently reviewed [134]. The "acute" neurological features mainly include motor signs (most often hemiparesis) [135,136], visual (such as blurred vision and visual field deficit) [25] and speech disturbances [124,136].…”

Section: Stroke-like Onsetmentioning

confidence: 99%

Unusual Clinical Presentations Challenging the Early Clinical Diagnosis of Creutzfeldt-Jakob Disease

Baiardi

Capellari

Stella

et al. 2018

JAD

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The introduction of prion RT-QuIC, an ultrasensitive specific assay for the in vivo detection of the abnormal prion protein, has significantly increased the potential for an early and accurate clinical diagnosis of Creutzfeldt-Jakob disease (CJD). However, in the clinical setting, the early identification of patients with possible CJD is often challenging. Indeed, CJD patients may present with isolated symptoms that remain the only clinical manifestation for some time, or with neurological syndromes atypical for CJD. To enhance awareness of unusual disease presentations and promote earlier diagnosis, we reviewed the entire spectrum of atypical early manifestations of CJD, mainly reported to date as case descriptions or small case series. They included sensory either visual or auditory disturbances, seizures, isolated psychiatric manifestations, atypical parkinsonian syndromes (corticobasal syndrome, progressive supranuclear palsy-like), pseudobulbar syndrome, isolated involuntary movements (dystonia, myoclonus, chorea, blepharospasm), acute or subacute onsets mimicking a stroke, isolated aphasia, and neuropathy. Since CJD is a rare disease and its clinical course rapidly progressive, an in-depth understanding and awareness of early clinical features are mandatory to enhance the overall diagnostic accuracy in its very early stages and to recruit optimal candidates for future therapeutic trials.

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Stroke-Like Presentation in a Case of Creutzfeldt-Jakob Disease

Cited by 15 publications

References 16 publications

COVID‐19‐associated immune‐mediated encephalitis mimicking acute‐onset Creutzfeldt‐Jakob disease

COVID‐19‐associated immune‐mediated encephalitis mimicking acute‐onset Creutzfeldt‐Jakob disease

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

Unusual Clinical Presentations Challenging the Early Clinical Diagnosis of Creutzfeldt-Jakob Disease

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