RESEARCH SUPPORT, NON-U.S. GOV'T
Cortical-basal ganglionic degeneration.
Neurology 1990 August
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.
Full text links
Trending Papers
Management of Heart Failure With Preserved Ejection Fraction in Elderly Patients: Effectiveness and Safety.Curēus 2023 Februrary
EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.Annals of the Rheumatic Diseases 2023 March 17
What's New in the Treatment of Non-Alcoholic Fatty Liver Disease (NAFLD).Journal of Clinical Medicine 2023 Februrary 27
BTS clinical statement on aspiration pneumonia.Thorax 2023 Februrary
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
Read by QxMD is copyright © 2021 QxMD Software Inc. All rights reserved. By using this service, you agree to our terms of use and privacy policy.
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app