Background and purpose: The aim of our study is to review the relationship between NCSE and sCJD. Creutzfeldt-Jakob disease (CJD) is the most common form of human prion disease. Electroencephalography (EEG)-detected changes such as periodic sharp wave complexes, superimposable to those seen in non-convulsive epileptic status (NCSE), have only rarely been described at CJD onset, especially in sporadic CJD (sCJD) cases.
Methods: We describe clinical, EEG, cerebrospinal fluid (CSF) and neuroimaging findings of a confirmed case of sCJD with tau pathology, initially diagnosed as NCSE. We performed a literature review in PubMed of previous publications on both sCJD and NCSE.
Results: An 82-year-old woman with no medical history presented with a 2-week rapidly progressive neurological disorder, with motor aphasia, myoclonus, pyramidalism, and left posterior alien hand. EEG showed periodic sharp waves on right frontal regions, so anti-epileptic treatment was started. CSF results were normal. Brain magnetic resonance imaging demonstrated hyperintensity of the right cerebral cortex in diffusion sequences. Due to suspected new-onset refractory status epilepticus (NORSE), corticosteroid treatment was started, without clinical improvement. Necropsy results confirmed sCJD with tau pathology. The literature review identified 14 references including a total of 18 cases with NCSE as the presenting symptom of sCJD; the clinical and results in complementary tests were compiled into a table.
Conclusions: Sporadic CJD should be considered in the differential diagnosis of patients with rapid cognitive decline and EEG changes consistent with NCSE. The wide heterogeneity in the etiology of NCSE, including autoimmune disorders, especially NORSE, suggests immunotherapy should be initiated based on a good risk-benefit balance. Some cases of sCJD, such as the present case with tau pathology, may mimic this clinico-electrical course.
Keywords: Creutzfeldt-Jakob disease; non-convulsive; periodic sharp wave complexes; status epilepticus; tau.
© 2020 European Academy of Neurology.