EEG observations in probable sporadic CJD

Mundlamurri, Ravindranadh Chowdary; Shah, Rutul; Adiga, Manjunath; Chatterjee, Aparijita; Gautham, Bhargava; Kenchaiah, Raghavendra; Asranna, Ajay; Mahadevan, Anita; Kulanthaivelu, Karthik; Sinha, Sanjib

doi:10.4103/aian.aian_672_20

Cited by 7 publications

(1 citation statement)

References 17 publications

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“…A characteristic MRI finding is gray matter hyperintensity with diffusion restriction (preferentially affecting the basal ganglia and the cortex), corresponding to the pathology of spongiform degeneration with vacuole formation in the neuropril [8,10]. An EEG (Figure 2) is another helpful but less sensitive tool, with only 66% of patients demonstrating the typical periodic sharp wave complexes (PSWC), most of which were asymmetric and seen in the right hemisphere [11], as was the finding in our patient. Several CSF biomarkers are also found to be of diagnostic utility in CJD.…”

Section: Discussionsupporting

confidence: 55%

Slow to Respond: A Rapidly Progressive Case of Sporadic Creutzfeldt-Jakob Disease

Kaur,

Lam,

Singh

et al. 2024

Cureus

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Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder caused by prion proteins. In about 85% of patients, CJD occurs as a sporadic disease with no recognizable pattern of transmission. Sporadic CJD (sCJD) can present with rapid cognitive and functional decline, memory deficits, myoclonus, pyramidal and extrapyramidal signs, and visual deficits. The large spectrum of phenotypic variability has made the recognition of prion diseases difficult, and given the rare incidence, it is not uncommon for it to be missed as a potential diagnosis.We present a highly unusual case of a 76-year-old woman with rapidly progressive sCJD who died within five weeks of presentation. Our case demonstrates a typical sequence of symptoms, with rapidly progressive dementia and cerebellar signs at disease onset and myoclonus later in the disease course.

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Section: Discussionsupporting

confidence: 55%

Slow to Respond: A Rapidly Progressive Case of Sporadic Creutzfeldt-Jakob Disease

Kaur,

Lam,

Singh

et al. 2024

Cureus

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The comparative analysis between sporadic and genetic Creutzfeldt-Jakob disease in China

Li,

Chen,

Zou

et al. 2024

Acta Neurol Belg

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Prion therapeutics: Lessons from the past

Shim

Sharma

2022

Prion

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Prion diseases are a group of incurable zoonotic neurodegenerative diseases (NDDs) in humans and other animals caused by the prion proteins. The abnormal folding and aggregation of the soluble cellular prion proteins (PrP C ) into scrapie isoform (PrP Sc ) in the Central nervous system (CNS) resulted in brain damage and other neurological symptoms. Different therapeutic approaches, including stalling PrP C to PrP Sc conversion, increasing PrP Sc removal, and PrP C stabilization, for which a spectrum of compounds, ranging from organic compounds to antibodies, have been explored. Additionally, a non-PrP targeted drug strategy using serpin inhibitors has been discussed. Despite numerous scaffolds being screened for anti-prion activity in vitro , only a few were effective in vivo and unfortunately, almost none of them proved effective in the clinical studies, most likely due to toxicity and lack of permeability. Recently, encouraging results from a prion-protein monoclonal antibody, PRN100, were presented in the first human trial on CJD patients, which gives a hope for better future for the discovery of other new molecules to treat prion diseases. In this comprehensive review, we have re-visited the history and discussed various classes of anti-prion agents, their structure, mode of action, and toxicity. Understanding pathogenesis would be vital for developing future treatments for prion diseases. Based on the outcomes of existing therapies, new anti-prion agents could be identified/synthesized/designed with reduced toxicity and increased bioavailability, which could probably be effective in treating prion diseases.

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EEG observations in probable sporadic CJD

Cited by 7 publications

References 17 publications

Slow to Respond: A Rapidly Progressive Case of Sporadic Creutzfeldt-Jakob Disease

Slow to Respond: A Rapidly Progressive Case of Sporadic Creutzfeldt-Jakob Disease

The comparative analysis between sporadic and genetic Creutzfeldt-Jakob disease in China

Prion therapeutics: Lessons from the past

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