|
|
|
|
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.The results confirm that unilateral parkinsonism unresponsive to levodopa and limb ideomotor apraxia are the clinical hallmarks of corticobasal degeneration,and only a minority of patients with corticobasal degeneration present with dementia. The study also suggests that a focal cognitive and extrapyramidal motor syndrome is indicative of corticobasal degeneration.Survival in corticobasal degeneration was shortened by the early presence of(more) widespread parkinsonian features or frontal lobe syndrome. |
|