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Research Article: Electroencephalography distinguished anti-N-methyl-D-aspartate receptor encephalitis and Creutzfeldt-Jakob disease

Date Published: 2026-07-23

Abstract:
Creutzfeldt-Jakob disease (CJD) and anti-NMDA receptor (anti-NMDAR) encephalitis are two diseases with different progression and prognosis, so it is crucial to distinguish them early. Here we retrospectively studied the medical records of patients with CJD and anti-NMDAR encephalitis diagnosed between November 1, 2011 and March 1, 2022. More patients had seizures in anti-NMDA receptor encephalitis group than in CJD group. Anti-NMDAR encephalitis patients had much higher cerebrospinal fluid (CSF) leukocyte counts while patients with CJD had higher CSF albumin count. Extensive cortical signal abnormalities were more common in CJD, whereas limbic system signal abnormalities showed no significant trend toward higher frequency in anti-NMDAR encephalitis. Generalized Periodic Discharges (GPDs) and GPDs with triphasic morphology and A-P lag (GPDsA-P) in continuous EEG of patients with CJD were significantly different from those in anti-NMDAR encephalitis. There were no differences in periodic sharp compound waves in the continuous EEG of the two groups. In conclusion, we present distinguishing features between CJD and anti-NMDAR encephalitis, and EEG can be used as a reliable auxiliary diagnosis to distinguish between two diseases.

Introduction:
Creutzfeldt-Jakob disease (CJD) is a rare and lethal neurodegenerative disease with rapid progression ( 1 , 2 ). The most common type is chronic or subacute spongiform encephalopathy with dementia, psychiatric disorders, and Parkinsonian-like manifestations due to prion infection ( 3 ). The lack of a cure or mitigation for this disease and the fact that its course may mimic multiple diseases makes differential diagnosis extremely important. Anti-NMDA receptor encephalitis is an auto-immune disease associated with…

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