Difference between revisions of "Corticobasal syndrome / corticobasal degeneration"
(Created page with "'''Diagnosis''' CBS clinical criteria 111 (note that other clinical phenotypes, including FBS, naPPA, and PSP may also have CBD pathology) 1) Probable CBS :a. Must have asy...") |
|||
| Line 1: | Line 1: | ||
'''Diagnosis''' | '''Diagnosis''' | ||
| − | CBS clinical criteria | + | CBS clinical criteria (Armstrong 2013) (note that other clinical phenotypes, including FBS, naPPA, and PSP may also have CBD pathology) |
1) Probable CBS | 1) Probable CBS | ||
| Line 43: | Line 43: | ||
::iii. Alien limb phenomena (more than simple levitation) | ::iii. Alien limb phenomena (more than simple levitation) | ||
| − | - patients with CBS will often have deficits within domains of executive functioning, writing, as and visuospatial construction | + | - patients with CBS will often have deficits within domains of executive functioning, writing, as and visuospatial construction (Finger 2016) |
| − | - neuroimaging shows asymmetric frontal and parietal lobe atrophy | + | - neuroimaging shows asymmetric frontal and parietal lobe atrophy (Finger 2016 |
| − | Other possible presentations of underlying corticobasal degeneration pathology: | + | Other possible presentations of underlying corticobasal degeneration pathology: (Armstrong 2013) |
1) Frontal behavioral-spatial syndrome | 1) Frontal behavioral-spatial syndrome | ||
| Line 66: | Line 66: | ||
'''Pathology''' | '''Pathology''' | ||
| − | Proposed pathologic criteria for the diagnosis of CBD require: | + | |
| + | Proposed pathologic criteria for the diagnosis of CBD require: (Armstrong 2013) | ||
| + | |||
:a) The detection of characteristic tau-immunoreactive lesions in the neurons, glia, and cell processes of the cortex and striatum (caudate and putamen), particularly astrocytic plaques and thread-like lesions in both white matter and gray matter | :a) The detection of characteristic tau-immunoreactive lesions in the neurons, glia, and cell processes of the cortex and striatum (caudate and putamen), particularly astrocytic plaques and thread-like lesions in both white matter and gray matter | ||
| Line 78: | Line 80: | ||
== References == | == References == | ||
| + | |||
| + | Armstrong, M. J. et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 80, 496–503 (2013). https://pubmed.ncbi.nlm.nih.gov/23359374/ | ||
| + | |||
| + | Finger, E. C. Frontotemporal Dementias. Contin. Minneap. Minn 22, 464–489 (2016). https://pubmed.ncbi.nlm.nih.gov/27042904/ | ||
Revision as of 12:06, 12 June 2021
Diagnosis
CBS clinical criteria (Armstrong 2013) (note that other clinical phenotypes, including FBS, naPPA, and PSP may also have CBD pathology)
1) Probable CBS
- a. Must have asymmetric presentation
- b. Must have at least two of the following:
- i. Limb rigidity or akinesia
- ii. Limb dystonia
- iii. Limb myoclonus
- c. Must have at least two of the following:
- i. Orobuccal or limb apraxia
- ii. Cortical sensory deficit (i.e. agraphesthesia, astereognosis)
- iii. Alien limb phenomena (more than simple levitation)
2) Possible CBS
- a. Must have asymmetric presentation
- b. Must have at least one of the following:
- i. Limb rigidity or akinesia
- ii. Limb dystonia
- iii. Limb myoclonus
- c. Must have at least one of the following:
- i. Orobuccal or limb apraxia
- ii. Cortical sensory deficit
- iii. Alien limb phenomena (more than simple levitation)
- patients with CBS will often have deficits within domains of executive functioning, writing, as and visuospatial construction (Finger 2016)
- neuroimaging shows asymmetric frontal and parietal lobe atrophy (Finger 2016
Other possible presentations of underlying corticobasal degeneration pathology: (Armstrong 2013)
1) Frontal behavioral-spatial syndrome
- a. Must have at least two of the following:
- i. Executive dysfunction
- ii. Behavioral or personality changes
- iii. Visuospatial deficits
2) Non-fluent/agrammatic PPA (naPPA), see PPA chapter
3) PSP syndrome, see PSP
Pathology
Proposed pathologic criteria for the diagnosis of CBD require: (Armstrong 2013)
- a) The detection of characteristic tau-immunoreactive lesions in the neurons, glia, and cell processes of the cortex and striatum (caudate and putamen), particularly astrocytic plaques and thread-like lesions in both white matter and gray matter
- b) In conjunction with neuronal loss in focal cortical regions and in the substantia nigra
- c) Supportive features include ballooned cortical neurons, cortical atrophy, and tau-positive oligodendroglial coiled bodies
- pathology is typically 4R tau (CBD) > (Alzheimer’s pathology) > PSP, TDP-43 > 3R tau 92
References
Armstrong, M. J. et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 80, 496–503 (2013). https://pubmed.ncbi.nlm.nih.gov/23359374/
Finger, E. C. Frontotemporal Dementias. Contin. Minneap. Minn 22, 464–489 (2016). https://pubmed.ncbi.nlm.nih.gov/27042904/
