Background: There is no biological marker of progression in early Parkinson Disease (PD). Upper limb (UL) tremor is the most common motor symptom at onset. The significance of lower limb (LL) tremor remains unknown. We report on longitudinally followed autopsy-verified PD tremor onset cases. Methods: A chart review of longitudinally followed autopsy-verified PD cases was performed. Age and mode of onset were recorded at initial evaluation. Prognosis was measured by change in Hoehn and Yahr scale while on levodopa (LD). Results: Forty-nine patients were included. Thirty-eight cases had upper limb (UL), four lower limb (LL), and seven upper and lower limb (ULL) onset tremor. UL had 86.8% response to LD, LL 50% and ULL 85.7%. Sub-analysis of UL responders found 20% mild improvement, 53.3% moderate and 26.7% marked. ULL had moderate response in 83.3% and marked in 16.7%. LL responders only had mild improvement with LD. Conclusions: Tremor onset is most common in UL, followed by ULL and then LL. LL onset tremor cases have an inferior response to LD when compared to UL and ULL cases. We plan for further pathophysiologic studies to investigate LL onset in PD.