Periodic sharp wave complexes identify a distinctive phenotype in Creutzfeldt-Jacob disease. (November 2022)

• Record Type: Journal Article
• Authors: Castelli, Alessandro; Placidi, Fabio; Bonomi, Chiara Giuseppina; Di Giuliano, Francesca; Martorana, Alessandro; Pizzicannella, Giulia; Liguori, Claudio; Manfredi, Natalia; Mari, Luisa; Pagano, Andrea; Bramato, Valentina; Mercuri, Nicola Biagio; Izzi, Francesca
• Is Part Of: Clinical neurophysiology. Volume 143(2022)
• Page Start: 124
• Page End: 132
• Publication Date: 2022-11
• Subjects: PrPSc Abnormal isoform of Prion Protein -- CSF Cerebrospinal Fluid -- CJD Creutzfeldt-Jacob Disease -- DWI Diffusion Weighted Imaging -- EEG Electroencephalogram -- fCJD Familial Creutzfeldt-Jacob Disease -- FS Focal slowing -- FLAIR Fluid Attenuated Inversion Recovery -- MRI Magnetic Resonance Imaging -- M Methionine -- NPDs Non-periodic Discharges -- PSWCs Periodic Sharp Wave Complexes -- PrP Prion Protein -- RPD Rapidly Progressive Dementia -- RT-QuIC Real-Time Quaking Induced Conversion -- sCJD Sporadic Creutzfeldt-Jacob Disease -- TTA Time to admission -- V Valine; Creutzfeldt-Jacob Disease -- Rapid Progressive Dementia -- Periodic Sharp Wave Complexes -- Brain Magnetic Resonance Imaging -- 14-3-3 Protein -- Prognosis; Neurophysiology -- Periodicals; Electroencephalography -- Periodicals; Electromyography -- Periodicals; Neurology -- Periodicals; 612.8
• DOI: 10.1016/j.clinph.2022.08.025
• Languages: English
• ISSNs: 1388-2457

Abstract

Highlights: EEG criterion has the lowest sensitivity and specificity among the CJD criteria. The early presence of periodic sharp wave complexes is associated with faster evolution and reduced survival time in CJD patients. CJD patients with typical EEG findings have specific MRI and cerebrospinal fluid features. Abstract: Objective: The aim of our study was to evaluate the diagnostic and prognostic value of Electroencephalogram (EEG), brain Magnetic Resonance Imaging (MRI) and cerebrospinal fluid features, currently representing Creutzfeldt-Jacob Disease (CJD) diagnostic criteria. Methods: A retrospective study on rapidly progressive dementia patients admitted at the Neurology Clinic of the University of Rome "Tor Vergata" between 2015 and 2020 was conducted. We evaluated clinical, EEG, cerebrospinal fluid and neuroradiological findings. Results: Our analysis included 13 patients with probable CJD and 18 patients with non-CJD rapidly progressive dementia. Periodic sharp wave complexes were observed in 7/13 CJD and in 4/18 non-CJD patients (p =.151). The sub-analysis according to the EEG features revealed that CJD patients with earlier periodic sharp wave complexes had a significantly lower average survival time (p =.003), a shorter time to admission (p =.003) and lower levels of cerebrospinal fluid p-tau (p =.008) compared to CJD patients without periodic sharp wave complexes. Finally, they did not show signs of signal alteration on Fluid Attenuated Inversion Recovery images. Conclusions: Despite the lowest diagnostic specificity and sensibility among the CJD criteria, periodic sharp wave complexes could identify a distinctive phenotype hallmarked by a faster evolution, a reduced survival time and specific MRI and cerebrospinal fluid features. Significance: The early presence of the typical EEG pattern may play a prognostic role in CJD.