The Clinical Manifestations And Polysomnography(PSG) Characteristics Of Patients With Sporadic Creutzfeldt-jakob Disease

Objective :To analyse the sleep disorders and other clinical manifestations,polysomnography(PSG)features of patients with sporadic CreutzfeldJakob disease(s CJD),and to study the characteristics of sleep disorders in patients with s CJD.Methods:9 cases with probable diagnosis of s CJD have been retrospectively reviewed.The sleep-wake symptoms and other clinical manifestations,14-3-3 protein in cerebrospinal fluid(CSF),magnetic resonance imaging(MRI),electroencephalography,PSG and genetic analysis were conducted.Result :All of the 9 cases presented typical clinical manifestations of rapidly progressive cognitive impairment(RPD),pyramidal and extrapyramidal symptoms and akinetic mutism.Sleep-wake symptoms were observed in almost all patients,mainly including hypersomnia,insomnia.Two thirds of the cases had positive 14-3-3 protein in CSF.The polymorphisms of codon 129 and 219 of all cases were MM and EE.The vast majority of cases have increased signals intensity changes on DWI,known as “DWI ribbon-like signals”,in the early stage of the disease.Increasing slow wave in the background were observed in all cases,and periodic sharp wave complexes(PSWC)was observed in 4/9 of the cases.5 cases completed PSG,showing severe sleep disorder,reduction in total sleep time,and significantly reduced sleep efficiency.Non-rapid eye movement(NREM)stage3 and rapid eye movement(REM)sleep could be lost.Conclusion:Sleep-wake symptoms are common in patients with s CJD and can occur early in the stages of disease.PSG analysis in patients with s CJD has special characteristics,which can be used for the differential diagnosis of other neurodegenerative diseases.