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Corticobasal Ganglionic Degeneration
05 November 2004
Age: 50-70
36% sensitivity for diagnosis Best predictors for first visit: limb dystonia, asymmetric Parkinsonism, ideomotor apraxia, absence of gait/balance disturbance, limb dystonia unresponsive to L-Dopa Best predictors in later stages: delayed onset of gait/balance disturbance, ideomotor apraxia, cognitive disturbance, focal myoclonus.
Movement Disorders 100% Akinesia, rigidity 86% Postural instability 79% Postural, action tremor 57% Limb dystonia 54% Focal reflex myoclonus 18% Orolingual dyskinesia 18% Athetosis 7% Limb chorea 4% Rest tremor 4% Axial dystonia Cerebral cortical signs 71% Cortical sensory loss (neglect) 71% Apraxia 50% Alien limb 46% Frontal lobe reflexes 43% Dementia 21% Dysphasia Others 64% Hyperreflexia 46% Upgoing plantars 46% Impaired ocular motion 36% Dysarthria 21% Dysphagia 7% Dysphonia
Investigations CT/MRI: asymmetry of sulci in parietal region FDG PET: asymmetry in cortical & subcortical regions HMPAO SPECT: perfusion asymmetry of parietal cortical regions IBZM SPECT: reduced basal ganglia uptake Oculography: abnormal horizontal saccades Abnormal SSEP or motor evoked potentials
TREATMENT 15% some improvement with L-Dopa initially Clonazepam for myoclonus Clonazepam, anticholinergics & baclofen for dystonia Baclofen for pyramidal signs. |
