Clinical, imaging and electrophysiological characteristics of Creutzfeldt-Jakob disease / 临床神经病学杂志

ABSTRACT

Objective To explore the clinical, imaging and electrophysiological characteristics of Creutzfeldt-Jakob disease ( CJD) .Methods The clinical data of 28 very probable CJD patients were analyzed retrospectively. Results Twenty-eight patients in this group, acute onset was in 5 cases, subacute onset was in 17 cases, chronic onset was in 6 cases.The initial symptoms were dizziness, gait instability in 11 cases ( 39.3%) , cognitive dysfunction in 10 cases (35.7%), visual symptoms in 3 cases (10.7%), mental symptoms, insomnia, repeated falls and barylalia in 1 case (3.6%) respectively.The clinical symptoms were progressive dementia in 28 cases (100%), myoclonus in 21 cases (75.0%), ataxia in 19 cases (67.9%), abnormal mental behavior in 7 cases (25.0%), pyramidal signs in 20 cases (71.4%), extrapyramidal symptoms in 15 cases (53.6%), visual impairment in 6 cases (21.4%), sleep disorder in 11 cases (39.3%), and aphasia in 9 cases (32.1%).Twelve patients (42.9%) had positive results with CSF 14-3-3 protein analysis.Twenty-four patients (85.7%) had MRI high signal abnormalities in caudate nucleus and putamen or/and at least two cortical regions.Eight cases (28.6%) experienced a typical periodic sharp wave complexes (PSWCs), 12 cases (42.9%) had a diffuse symmetric or asymmetric slow-wave activity, 8 cases (28.6%) showed rhythmic three-phase or delta wave, with the whole guide or forehead synchronization.Conclusion Most CJD patients are subacute or chronic onset.Initial symptoms of CJD are atypical, progressed to classical clinical characteristics presented in middle-late stages.Nearly half patients have CSF 14-3-3 protein positive.MRI DWI of mostly CJD patients have abnormal signal.EEG of nearly a third of CJD patients shows PSWCs.