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geri:dementia:corticobasal-degeneration-cbd [on June 2, 2019]
 geri:dementia:corticobasal-degeneration-cbd [on February 9, 2022] (current)
psychdb [Differential Diagnosis]
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 ===== Primer ===== ===== Primer =====
-**Corticobasal Degeneration** (CBD) is an neurodegenerative disorder, classified as an atypical parkinsonian syndrome (also known as one of the Parkinson'​s-plus syndromes). CBD was previously classified as a progressive asymmetric movement disorder characterized by akinesia, rigidity, dystonia, focal myoclonus, ideomotor apraxia, and/or alien-limb phenomena. However, it is increasingly seen as a disorder with cognitive and behavioural disturbances as well. The diagnosis of CBD remain challenging because there is considerable overlap between CBD pathology and other neurodegenerative disorders ​(including [[geri:​dementia:​progressive-supranuclear-palsy-psp|progressive supranuclear palsy]] (PSP), [[geri:​dementia:​alzheimers|]],​ and [[geri:​dementia:​frontotemporal|frontotemporal dementia]]).+**Corticobasal Degeneration** (CBD) is an neurodegenerative disorder, classified as an atypical parkinsonian syndrome (also known as one of the Parkinson'​s-plus syndromes). CBD was previously classified as a progressive asymmetric movement disorder characterized by akinesia, rigidity, dystonia, focal myoclonus, ideomotor apraxia, and/or alien-limb phenomena. However, it is increasingly seen as a disorder with cognitive and behavioural disturbances as well. The diagnosis of CBD remain challenging because there is considerable overlap between CBD pathology and other neurodegenerative disorders including [[geri:​dementia:​progressive-supranuclear-palsy-psp|progressive supranuclear palsy]] (PSP), [[geri:​dementia:​alzheimers|]],​ and [[geri:​dementia:​frontotemporal|frontotemporal dementia]].
  
 ===== Diagnostic Criteria ===== ===== Diagnostic Criteria =====
 ==== Corticobasal Syndrome (CBS) ===== ==== Corticobasal Syndrome (CBS) =====
-Since CBD pathology overlaps with other neurodegenerative disorders, it is helpful to first think of CBD on spectrum of neurodegenerative syndromes. ​The term Corticobasal //​Syndrome//​ (not Corticobasal Degeneration),​ includes other neurodegenerative disorders such as [[geri:​dementia:​progressive-supranuclear-palsy-psp|progressive supranuclear palsy]] (PSP), [[geri:​dementia:​alzheimers|]],​ and [[geri:​dementia:​frontotemporal|frontotemporal dementia]] (FTD) pathology.+Since CBD pathology overlaps with other neurodegenerative disorders, it is helpful to first think of CBD on spectrum of neurodegenerative syndromes. ​Thus the term **Corticobasal //​Syndrome//​** (not Corticobasal Degeneration),​ includes other neurodegenerative disorders such as [[geri:​dementia:​progressive-supranuclear-palsy-psp|progressive supranuclear palsy]] (PSP), [[geri:​dementia:​alzheimers|]],​ and [[geri:​dementia:​frontotemporal|frontotemporal dementia]] (FTD) pathology.
  
 <panel type="​info"​ title="​Proposed clinical phenotypes (syndromes) associated with the pathology of corticobasal degeneration"​ subtitle="​Armstrong,​ Melissa J., et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 80.5 (2013): 496-503." ​ no-body="​true">​ <panel type="​info"​ title="​Proposed clinical phenotypes (syndromes) associated with the pathology of corticobasal degeneration"​ subtitle="​Armstrong,​ Melissa J., et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 80.5 (2013): 496-503." ​ no-body="​true">​
-^ Syndrome ​                                                    ​^ Features ​                                                                                                                                                                                                                       ^ +^ Syndrome ​                                                                                                      ​^ Features ​                                                                                                                                                                                                                                         ^ 
-^ Probable corticobasal syndrome ​                              ​| Asymmetric presentation of 2 of: a) limb rigidity or akinesia, b) limb dystonia, c) limb myoclonus ​plus 2 of: d) orobuccal or limb apraxia, e) cortical sensory deficit, f) alien limb phenomena (more than simple levitation) ​ | +^ Probable corticobasal syndrome ​                                                                                ​| Asymmetric presentation of ''​2'' ​of:\\ a) limb rigidity or akinesia\\ b) limb dystonia\\ c) limb myoclonus\\ Plus ''​2'' ​of:\\ d) orobuccal or limb apraxia\\ e) cortical sensory deficit\\ f) alien limb phenomena (more than simple levitation) ​ | 
-^ Possible corticobasal syndrome ​                              ​| May be symmetric: 1 of: a) limb rigidity or akinesia, b) limb dystonia, c) limb myoclonus ​plus 1 of: d) orobuccal or limb apraxia, e) cortical sensory deficit, f) alien limb phenomena (more than simple levitation) ​          | +^ Possible corticobasal syndrome ​                                                                                ​| May be symmetric: ​''​1'' ​of:\\ a) limb rigidity or akinesia\\ b) limb dystonia\\ c) limb myoclonus\\ Plus ''​1'' ​of:\\ d) orobuccal or limb apraxia\\ e) cortical sensory deficit\\ f) alien limb phenomena (more than simple levitation) ​          | 
-^ Frontal behavioral-spatial syndrome ​                         | Two of: a) executive dysfunction, b) behavioral or personality changes, c) visuospatial deficits ​                                                                                                                               | +^ Frontal behavioral-spatial syndrome ​                                                                           | ''​2'' ​of:\\ a) executive dysfunction\\ b) behavioral or personality changes\\ c) visuospatial deficits ​                                                                                                                                           | 
-^ Nonfluent/​agrammatic variant of [[geri:​dementia:​primary-progressive-aphasia-ppa|primary progressive aphasia]] ​ | Effortful, agrammatic speech plus at least one of: a) impaired grammar/​sentence comprehension with relatively preserved single word comprehension,​ or b) groping, distorted speech production (apraxia of speech) ​              ​| +^ Nonfluent/​agrammatic variant of [[geri:​dementia:​primary-progressive-aphasia-ppa|primary progressive aphasia]] ​ | Effortful, agrammatic speech plus at least ''​1'' ​of:\\ a) impaired grammar/​sentence comprehension with relatively preserved single word comprehension,​ or \\ b) groping, distorted speech production (apraxia of speech) ​                         | 
-^ [[geri:​dementia:​progressive-supranuclear-palsy-psp|Progressive supranuclear palsy]] (PSP) syndrome ​               | Three of: a) axial or symmetric limb rigidity or akinesia, b) postural instability or falls, c) urinary incontinence, d) behavioral changes, e) supranuclear vertical gaze palsy or decreased velocity of vertical saccades ​    ​|+^ [[geri:​dementia:​progressive-supranuclear-palsy-psp|Progressive supranuclear palsy]] (PSP) syndrome ​            ​| ''​3'' ​of:\\ a) axial or symmetric limb rigidity or akinesia\\ b) postural instability or falls\\ c) urinary incontinence\\ d) behavioral changes\\ e) supranuclear vertical gaze palsy or decreased velocity of vertical saccades ​                ​|
 </​panel>​ </​panel>​
 +
 +<callout type="​tip"​ title="​Definitions"​ icon="​true">​
 +  * Alien hand syndrome - the patient does not recognize the actions of their hand and cannot control movement of the hand or arm
 +  * [[neurology:​approach-apraxia|Apraxia]] – the loss of the ability to make familiar, purposeful movements, manifested as difficulty using familiar objects or doing familiar things.
 +  * Visual-spatial impairment – an individual with corticobasal degeneration may have difficulty orienting objects in space.
 +</​callout>​
  
 ==== Corticobasal Degeneration (CBD) ===== ==== Corticobasal Degeneration (CBD) =====
-After thinking about the spectrum of symptoms that a patient may have, and ruling out other diagnoses on the differential,​ the following criteria can be used, as proposed by the 1st International CBD Investigators Meeting in 2013.[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC3590050/​|Armstrong,​ M. J., Litvan, I., Lang, A. E., Bak, T. H., Bhatia, K. P., Borroni, B., ... & Josephs, K. A. (2013). Criteria for the diagnosis of corticobasal degeneration. Neurology, 80(5), 496-503.]])]+After thinking about the spectrum of symptoms that a patient may have, and ruling out other diagnoses on the differential,​ the following criteria can be used to determine whether a patient has corticobasal degeneration, as proposed by the 1st International CBD Investigators Meeting in 2013.[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC3590050/​|Armstrong,​ M. J., Litvan, I., Lang, A. E., Bak, T. H., Bhatia, K. P., Borroni, B., ... & Josephs, K. A. (2013). Criteria for the diagnosis of corticobasal degeneration. Neurology, 80(5), 496-503.]])]
  
 <panel type="​info"​ title="​Diagnostic Criteria for Corticobasal Degeneration"​ subtitle="​Armstrong,​ Melissa J., et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 80.5 (2013): 496-503."​ footer="​CBD = corticobasal degeneration;​ CBS = corticobasal syndrome; FBS = frontal behavioral-spatial syndrome; NAV = nonfluent/​agrammatic variant of primary progressive aphasia; PSPS = progressive supranuclear palsy syndrome."​ no-body="​true">​ <panel type="​info"​ title="​Diagnostic Criteria for Corticobasal Degeneration"​ subtitle="​Armstrong,​ Melissa J., et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 80.5 (2013): 496-503."​ footer="​CBD = corticobasal degeneration;​ CBS = corticobasal syndrome; FBS = frontal behavioral-spatial syndrome; NAV = nonfluent/​agrammatic variant of primary progressive aphasia; PSPS = progressive supranuclear palsy syndrome."​ no-body="​true">​
-|                                                         ^ Clinical research criteria for probable sporadic CBD                  ^ Clinical criteria for possible CBD                                             ​^ +|                                                         ^ Clinical research criteria for probable sporadic CBD                    ^ Clinical criteria for possible CBD                                               ​^ 
-^ Presentation ​                                           | Insidious onset and gradual progression ​                              ​| Insidious onset and gradual progression ​                                       | +^ Presentation ​                                           | Insidious onset and gradual progression ​                                ​| Insidious onset and gradual progression ​                                         | 
-^ Minimum duration of symptoms (year) ​                    | 1                                                                     ​| 1                                                                              | +^ Minimum duration of symptoms (year) ​                    | 1                                                                       ​| 1                                                                                | 
-^ Age at onset (year) ​                                    | ≥ 50                                                                  | No minimum ​                                                                    ​| +^ Age at onset (year) ​                                    | ≥ 50                                                                    | No minimum ​                                                                      ​| 
-^ Family history (2 or more relatives) ​                   | Exclusion ​                                                            ​| Permitted ​                                                                     | +^ Family history (2 or more relatives) ​                   | Exclusion ​                                                              ​| Permitted ​                                                                       | 
-^ Permitted phenotypes (see previous table for criteria) ​ | 1) Probable CBS or 2) FBS or NAV plus at least one CBS feature (a–f) ​ | 1) Possible CBS or 2) FBS or NAV or 3) PSPS plus at least one CBS feature b–f  | +^ Permitted phenotypes (see previous table for criteria) ​ | 1) Probable CBS or 2) FBS or NAV plus at least ''​1'' ​CBS feature (a–f) ​ | 1) Possible CBS or 2) FBS or NAV or 3) PSPS plus at least ''​1'' ​CBS feature b–f  | 
-^ Genetic mutation affecting tau (e.g. - MAPT)            | Exclusion ​                                                            ​| Permitted ​                                                                     |+^ Genetic mutation affecting tau (e.g. - MAPT)            | Exclusion ​                                                              ​| Permitted ​                                                                       |
 </​panel>​ </​panel>​
  
 +==== Signs and Symptoms ====
 +  * Individuals with CBD can have symptoms similar to [[geri:​parkinsons|Parkinson'​s disease]] including axial rigidity (making it hard to turn around in bed), swallowing difficulties (with swallowing saliva, clearing throat, choking, and coughing), and visual difficulties such as blurry vision and difficulty scanning the environment.
 +  * Language and speech changes are increasingly being recognized as features of CBD.[([[https://​link.springer.com/​article/​10.1007/​s00415-019-09463-1|Peterson,​ K. A., Patterson, K., & Rowe, J. B. (2021). Language impairment in progressive supranuclear palsy and corticobasal syndrome. Journal of neurology, 268(3), 796-809.]])]
 ===== Pathophysiology ===== ===== Pathophysiology =====
 Pathological findings in CBD patients have shown astrocytic abnormalities in the brain as well as accumulation of tau protein. Thus, CBD is considered a tauopathy. Pathological findings in CBD patients have shown astrocytic abnormalities in the brain as well as accumulation of tau protein. Thus, CBD is considered a tauopathy.
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   * **[[geri:​dementia:​alzheimers|Alzheimer'​s disease (AD)]]**   * **[[geri:​dementia:​alzheimers|Alzheimer'​s disease (AD)]]**
     * Subsequent emergence of asymmetric motor/​sensory signs, hyperreflexia,​ gait abnormalities,​ parkinsonism,​ falls, urinary incontinence,​ and extraocular movement abnormalities may suggest a CBD over AD, especially if ther are more than three of these signs and symptoms[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC5373780/​|Day,​ G. S., Lim, T. S., Hassenstab, J., Goate, A. M., Grant, E. A., Roe, C. M., ... & Morris, J. C. (2017). Differentiating cognitive impairment due to corticobasal degeneration and Alzheimer disease. Neurology, 88(13), 1273-1281.]])]     * Subsequent emergence of asymmetric motor/​sensory signs, hyperreflexia,​ gait abnormalities,​ parkinsonism,​ falls, urinary incontinence,​ and extraocular movement abnormalities may suggest a CBD over AD, especially if ther are more than three of these signs and symptoms[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC5373780/​|Day,​ G. S., Lim, T. S., Hassenstab, J., Goate, A. M., Grant, E. A., Roe, C. M., ... & Morris, J. C. (2017). Differentiating cognitive impairment due to corticobasal degeneration and Alzheimer disease. Neurology, 88(13), 1273-1281.]])]
 +  * **Neuroleptic-induced [[meds:​antipsychotics:​eps|parkinsonism]]** (i.e. - extrapyramidal symptoms)
 +    * In these cases of potential confounding effects from medications such as antipsychotics,​ the clinician should try to elicit a clear history if the Parkinsonian symptoms developed before or after the initiation of antipsychotics.
 +===== Investigations =====
 +==== Neuroimaging ====
 +MRI shows frontal-parietal atrophy
 ===== Resources ===== ===== Resources =====
   * [[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC5619264/​|Parmera,​ J. B., Rodriguez, R. D., Studart Neto, A., Nitrini, R., & Brucki, S. M. D. (2016). Corticobasal syndrome: A diagnostic conundrum. Dementia & Neuropsychologia,​ 10(4), 267]]   * [[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC5619264/​|Parmera,​ J. B., Rodriguez, R. D., Studart Neto, A., Nitrini, R., & Brucki, S. M. D. (2016). Corticobasal syndrome: A diagnostic conundrum. Dementia & Neuropsychologia,​ 10(4), 267]]