Freezing of gait (FOG) is frequently observed in high level gait disorders (HLGD), but its relationship to disease progression and cognitive function is unknown. To study this relationship, episodic gait disturbances, affect and cognitive function were assessed in twenty-five patients with HLGD (mean age: 78.2 +/- 5.0 yrs). After a mean of 32.2 +/- 4.2 months, twenty-two patients were reassessed. FOG was observed in 20% of the patients at baseline and in 40% at follow-up. The presence of FOG was associated with significant mobility disturbances, functional deterioration as well as poor performance on the frontal neuropsychological assessment battery (Dementia Rating Scale - initiation sub-score) at follow-up. Depression, anxiety and fear of falling were not correlated with the presence of FOG at baseline or follow-up. These results indicate that FOG is common in HLGD, and that it is associated with significant functional disability and a specific frontal cognitive disturbance of initiation.