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Cortical-Basal Ganglionic Degeneration
Neurol 40:1203-1212, Riley,D.E.,et al, 1990
See this aricle in Pubmed

Article Abstract
We report our experience with 15 patients believed to have cortical-basal ganglionic degeneration.The clinical picture is distinctive,comprising features referable to both cortical and basal ganglionic dysfunction. Characteristic manifestations include cortical sensory loss,focal reflex myoclonus,"alien limb"phenomena,apraxia,rigidity and akinesia,a postural- action tremor,limb dystonia,hyperreflexia,and postural instability.The asymmetry of symptoms and signs is often striking.Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. Postmortem studies in 2 patients revealed the characteristic pathologic features of swollen,poorly staining(achromatic)neurons and degeneration of cerebral cortex and substantia nigra.Biochemical analysis of 1 brain showed a severe,diffuse loss of dopamine in the striatum.This condition is more frequent than previously believed,and the diagnosis can be predicted during life on the basis of clinical findings.However,as with other" degenerative"diseases of the nervous system,a definitive diagnosis of cortical-basal ganglionic degeneration requires confirmation by autopsy.
 
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alien hand syndrome
apraxia
apraxia of eye movements
bradykinesia
cortical-basal ganglionic degeneration
dementia
dyskinesia,buccal lingual facial
dysphagia
dystonia
gaze palsy
gaze palsy,supranuclear
hyperreflexia
imbalance
levitation
myoclonic jerks
neuropathology
rigidity
sensory loss
sensory loss,cortical
tremor

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