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cortical-basal ganglionic degeneration; corticobasal degeneration
Epidemiology:
- rare
- age of onset 50-70 years
Pathology:
1) aberrations in tau protein: abnormal staining in neurons & astrocytic processes
2) frontal/parietal atrophy
3) cortical cell loss
4) swollen, achromatic neurons throughout the cerebrum, most numerous in frontal regions
5) gliosis
6) degeneration of
a) basal ganglia
b) thalamus
c) midbrain
d) locus ceruleus
7) rare neuronal plaques
8) rare neurofibrillary tangles
9) decrease in blood flow to the left hemisphere, especially the parietal & premotor areas (ideomotor apraxia)
10) neuronal inclusions?
Clinical manifestations:
1) pronounced asymmetric rigidity
2) ideomotor apraxia
3) akinesia
4) cortical sensory changes - sensory loss
5) alien-limb phenomenon
6) myoclonus, limb dystonia (fixed dystonia) [6]
7) stimulus sensitive (reflex) myoclonus
8) disequilibrium
9) dysarthria
10) supranuclear gaze palsy
11) dementia (common)
- slowed processing speed
- impaired working memory
- impaired executive function
12) action tremor
13) pyramidal tract signs
14) blepharospasm
15) aphasia uncommon [6]
16) faster progression than Parkinson's disease
17) visual hallucinations uncommon [7]
Radiology:
- MRI of brain
- asymmetric cortical atrophy involving posterior frontal lobes & parietal lobes [6]
Differential diagnosis:
- frontotemporal dementia
a) sometimes clinically indistinguishable
b) considered by some a subtype of frontotemporal dementia
- amyotrophic lateral sclerosis
- distinguished by muscle weakness & upper motor neuron signs
- cognition generally preserved
Management:
1) generally no response to levodopa/carbidopa (Sinemet)
2) acetylcholinesterase inhibitors may be useful for cognitive impairment
3) SSRI's may be useful for depression
4) avoid neuroleptics
Interactions
disease interactions
Related
corticobasal syndrome (CBS)
tauopathy
General
parkinson plus syndrome
References
- Diseases of the Nervous System. Clinical Neurobiology.
Asbury, McKhann, McDonald eds. Saunders 1992 2nd ed.
- Lang & Lazano NEJM 339(15):1044-53 1998
- Bronstein J, In: Intensive Course in Geriatric Medicine &
Board Review, Marina Del Ray, CA, Sept 29-Oct 2, 2004
- Miller B, UCSF Memory & Aging Center, 2001
- Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP.
Corticobasal degeneration.
Lancet Neurol. 2004 Dec;3(12):736-43. Review.
PMID: 15556806
- Medical Knowledge Self Assessment Program (MKSAP) 17, 19
American College of Physicians, Philadelphia 2015, 2021
- Geriatric Review Syllabus, 11th edition (GRS11)
Harper GM, Lyons WL, Potter JF (eds)
American Geriatrics Society, 2022