Predictors of Disease Progression Among Patients in The Thai National Prion Disease Surveillance Program

Among 31 patients, 42% were female with mean age of 67 years. The most common clinical presentation is rapidly progressive dementia (84%) followed by pyramidal weakness, myoclonus and cerebellar dysfunction. There are no significant differences between core clinical features between both groups. CSF RT-QuIC was tested positive in almost all sporadic CJD and non in non-prion disorders. CSF tau is higher in sporadic CJD (4,027 pg/mL vs 377 ng/mL, p-value<0.001). (Table 1) Sporadic CJD diagnosis and higher levels of CSF tau and CSF NfL reported a worse MRC score at baseline. (Figure 2A-C) Linear mixed-effects models revealed no significant interactions between CSF biomarkers and time to MRC score. (Table 2) CONCLUSION: Our study demonstrates a significant main effect of CSF tau and NfL on the MRC score but no interaction effects. Patients with higher CSF tau levels generally have worse functional status throughout the clinical course, with similar rates of decline. However, the limited power of this study may restrict the detection of interaction effects.