The sample consisted of eight female and six male patients; mean age at symptom onset was 63 (SD 7.7) years, and mean disease duration was 7.9 (SD 2.6) years. The most commonly reported symptom at onset included asymmetric limb clumsiness with or without rigidity (50%) or
tremor (21%). At the first neurological visit, on average 3.0 (SD 1.9) years after symptom onset, the most often encountered extrapyramidal features included unilateral limb rigidity (79%) or
bradykinesia (71%), postural imbalance (45%), and unilateral
limb dystonia (43%).
Ideomotor apraxia (64%), and to a lesser extent cortical
dementia (36%), were the most common cortical signs present at the first visit. During the course of the disease, virtually all patients developed asymmetric or unilateral akinetic rigid
parkinsonism and a gait disorder. No patient had a dramatic response to
levodopa therapy. Median survival time after onset of symptoms was 7.9 (SD 0.7) (range, 2.5-12.5) years, and, after the first
clinic visit, 4.9 (SD 0.7) (range, 0.8-10) years. Early bilateral
bradykinesia, frontal syndrome, or two out of
tremor, rigidity, and
bradykinesia, predicted a shorter survival.
CONCLUSION: