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The onset of Creutzfeldt-Jakob disease (CJD) is usually around the age of 60, but younger patients have been described as well. Our study characterizes the demographic and clinical features of young-onset CJD patients. The CJD Israeli National Database was reviewed, and the patients were divided into groups of young (&lt;40-year-old) (Y|) and older disease onset (&gt;40-year-old) (O). Each group was further divided into sporadic (sCJD) and genetic (gCJD) patients. Clinical and demographic parameters were compared between the groups The study included 731 patients (Y- 18 patients, O- 713 patients). MRI showed classical features more often in the older population (O-76.9%, Y-36%,  p  = 0.006). Rapidly progressive dementia as a presenting feature was more common in the older group (O = 58%, Y = 27.7%,  p  = 0.019) whereas cerebellar onset (gait instability, dysarthria) was more common in the younger group (O = 6.7%, Y = 27.7%,  p  = 0.036)). Among gCJD patients, rapidly progressive dementia was commonly seen in older patients (O = 54%, Y = 21%  p  = 0.008) whereas cerebellar symptoms were seen in young patients (O = 7%, Y = 30%  p  = 0.01) Typical MRI findings were seen in 37% of young people compared to 87% of older patients ( p  = 0.002). No significant differences were between young and older patients in the sCJD group. Young-onset gCJD patients have unique disease features including less typical brain MRI changes, a lower prevalence of dementia, and a higher prevalence of cerebellar signs at disease onset.

The epidemiological and clinical characteristics of patients with young-onset genetic Creutzfeldt-Jakob disease