Early recognition of the prognosis in parkinsonism is important for both the management and studies aimed at preventing progression of disease. Less favorable prognosis is reported in early-onset postural instability and gait difficulty (PIGD) than in the tremor-onset cases, but the reasons for this are unknown. In 70 autopsy-verified cases, 11 (15.7%) had PIGD, and 34 (49%) had tremor onset. Improvement on levodopa was more common in the tremor-than the PIGD-onset cases (p < 0.05). The majority of tremor-onset cases had Lewy body disease, while the majority of PIGD-onset cases had other forms of pathology. Survival was shorter in the PIGD- than the tremor-onset cases (p < 0.05).