RT Journal Article
SR Electronic
T1 Cortical‐basal ganglionic degeneration
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 1203
OP 1203
DO 10.1212/WNL.40.8.1203
VO 40
IS 8
A1 D. E. Riley
A1 A. E. Lang
A1 A. Lewis
A1 L. Resch
A1 P. Ashby
A1 O. Hornykiewicz
A1 S. Black
YR 1990
UL http://n.neurology.org/content/40/8/1203.abstract
AB 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.