Materiały źródłowe

• #1 Corticobasal degeneration (corticobasal syndrome) | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/corticobasal-degeneration-corticobasal-syndrome

  A diagnosis of corticobasal degeneration (corticobasal syndrome) is made based on your symptoms, exam and testing. However, your symptoms could be due to another disease that affects the brain. Conditions that cause similar symptoms include progressive supranuclear palsy, Alzheimer’s disease, Pick’s disease or Creutzfeldt-Jakob disease. […] You may need an imaging test such as an MRI or CT scan to rule out these other conditions. Sometimes, these tests are performed every few months to look for changes in the brain. […] Positron emission tomography (PET) scans can identify brain changes related to corticobasal degeneration. However, more research needs to be done in this area. […] Your healthcare professional may test your blood or cerebrospinal fluid for amyloid and tau proteins. This can determine whether Alzheimer’s disease is the cause of your symptoms.

• #2 Corticobasal degeneration (corticobasal syndrome) – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/symptoms-causes/syc-20354767

  Corticobasal degeneration (corticobasal syndrome) can have several causes. Most commonly, the disease results from a buildup of a protein called tau in brain cells. The buildup of tau may lead to the breakdown of the cells. This can cause symptoms of corticobasal degeneration. […] Half of the people who have symptoms have corticobasal degeneration. But the second most common cause of corticobasal degeneration symptoms is Alzheimer’s disease. Other causes of corticobasal degeneration include progressive supranuclear palsy, Pick’s disease or Creutzfeldt-Jakob disease. […] People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.

• #3 Corticobasal degeneration – UpToDate

  https://www.uptodate.com/contents/corticobasal-degeneration

  Corticobasal degeneration (CBD) is a rare neurodegenerative disorder that poses significant challenges to clinical diagnosis and treatment. […] This topic will review the clinical features, pathology, diagnosis, and management of CBD. […] It has been recognized that pathologically proven CBD often begins as a cognitive or behavioral disturbance. Additionally, the characteristic cognitive and motor features are not specific to CBD, but may occur with other pathologically proven neurodegenerative disorders, including progressive supranuclear palsy (PSP), frontotemporal dementia, and Alzheimer disease (AD). […] This has prompted some experts to the use the term „corticobasal syndrome” (CBS) for cases with a clinical diagnosis, while reserving „CBD” for cases with neuropathologic confirmation.

• #4 Corticobasal degeneration | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/corticobasal-degeneration?lang=us

  Corticobasal syndrome (CBS) represents the clinical syndrome that is diagnosed clinically, while corticobasal degeneration is only reserved for the subset of cases that are pathologically confirmed as the tauopathy. […] Corticobasal degeneration has a heterogeneous clinical presentation, often making it challenging to diagnose. […] The classical corticobasal syndrome is a progressive disorder with various asymmetric movement abnormalities, myoclonus, as well as cortical signs including ideomotor apraxia and alien limb phenomenon. […] MRI is the modality of choice for assessing corticobasal degeneration, although similar findings can, only to a certain degree, be seen on CT. […] The pattern of atrophy in corticobasal degeneration may be distinguishable from that of progressive supranuclear palsy.

• #5 Criteria for the diagnosis of corticobasal degeneration – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23359374/

  Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. […] Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. […] Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset 50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. […] The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype. […] Future validation and refinement of the proposed criteria are needed.

• #6 Criteria for the diagnosis of corticobasal degeneration. | The Online Core Curriculum and Mastery (OCCAM) for Behavioral Neurology & Neuropsychiatry (BNNP)

  https://www.vumc.org/occam-bnnp/publication/criteria-diagnosis-corticobasal-degeneration

  Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. […] An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. […] Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. […] Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). […] Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset 50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. […] The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype.

• #7 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1150039-overview

  They defined four clinical phenotypes associated with CBD pathology based on these data: (1) Corticobasal syndrome, (2) Frontal behavioral-spatial syndrome (FBS), (3) Nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and (4) Progressive supranuclear palsy syndrome (PSPS). […] Based on the above phenotypes, they suggested two separate criteria for Probable CBD (at least one CBS feature, however, the differential could include FBS or naPPA) and Possible CBD (more inclusive of other tau disorders including PSPS) intended to be applied during a patient’s lifetime. […] However, in 2014 Alexander et al. disputed the utility of the above criteria after they applied them to their cohort of 33 patients followed longitudinally between 1990 and 2013. […] While the Armstrong et al. criteria did help to identify patients with CBS, they were not specific enough to identify which of these patients had CBD pathology on post-mortem examination; that is, not all patients with a CBD diagnosis clinically were found to have CBD-related pathology (4R tau in a specific distribution in neurons and glia).

• #8 Corticobasal Degeneration (CBD) – Brain Support Network

  https://www.brainsupportnetwork.org/education/corticobasal-degeneration/

  Corticobasal Degeneration (CBD) is the rarest of the four atypical parkinsonism disorders. […] The medical community now uses the term CBS or corticobasal syndrome to refer to a clinical diagnosis. CBD or corticobasal degeneration is now used for an autopsy-confirmed diagnosis. […] The diagnostic criteria listed in this section are derived from the paper Criteria for the diagnosis of corticobasal degeneration, Armstrong, et al. published in Neurology in 2013. […] The paper explains CBD in terms of four distinct types (phenotypes) of the disease. These are: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), progressive supranuclear palsy syndrome (PSPS). […] Proposed clinical phenotypes (syndromes) associated with the pathology of corticobasal degeneration include probable corticobasal syndrome, possible corticobasal syndrome, frontal behavioral-spatial syndrome, nonfluent/agrammatic variant of primary progressive aphasia, and progressive supranuclear palsy syndrome.

• #9 Corticobasal Degeneration | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/157387

  Corticobasal ganglionic degeneration (CBD) is a rare and progressive neurodegenerative disorder with diverse clinical and pathological features. The most common clinical presentation, corticobasal syndrome, includes asymmetric limb rigidity, Parkinsonism, dystonia, and cortical dysfunction, such as apraxia or sensory deficits. […] Accurate early diagnosis is essential, as it can guide patient care and facilitate the development of targeted therapies and disease-specific biomarkers. […] The clinical phenotypes were integrated to establish 2 overarching diagnostic categories: Probable CBD and Possible CBD. […] Probable CBD requires an age of onset of more than 50 years and excludes the presence of a family history or a known genetic mutation affecting tau protein. […] The clinical presentation of CBD is characterized by an asymmetric presentation of at least 2 of the following: limb rigidity or akinesia, limb dystonia, limb myoclonus, plus at least 2 of: orobuccal or limb apraxia, cortical sensory deficit, alien limb phenomenon.

• #10 Criteria for the diagnosis of corticobasal degeneration

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3590050/

  Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. […] Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. […] Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset 50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. […] The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype.

• #11 Validation of the new consensus criteria for the diagnosis of corticobasal degeneration | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/85/8/925

  Corticobasal degeneration (CBD) is a complex neurodegenerative disorder. Accurate diagnosis is increasingly important, with the advent of clinical trials of drugs aimed at modifying the underlying tau pathology. CBD often presents with a corticobasal syndrome including impairments of movement and cognition. However, patients with similar corticobasal syndromes can have neurodegenerative pathologies that are not CBD. In addition, patients with CBD may present with aphasia or behavioural change. The clinical diversity of CBD and mimicry by non-CBD pathologies hinders accurate diagnosis. […] The Armstrong criteria usefully broadens the recognised clinical phenotype of CBD but does not sufficiently improve the specificity of diagnosis to increase the power of clinical trials or targeted applications of tau-based disease-modifying therapies. Further work is required to show whether biomarkers could be more effective than clinical signs in the diagnosis of CBD. […] The new criteria continue to misdiagnose CBD mimics as CBD, and fail to identify about a third of cases with CBD, even when applied late in the course of the disease.

• #12

  https://www.brain.northwestern.edu/dementia/cbs/diagnosis.html

  CBS is diagnosed on the basis of a clinical examination by a physician, usually a neurologist or a psychiatrist. The diagnosis of CBS is made in the context of the history, cognitive examination, and neurological examination. […] Neuropathologic assessment at autopsy remains the only certain means to diagnose corticobasal degeneration (CBD), one of several neuropathologic causes of CBS. […] There are no definitive biomarkers for CBD, though some imaging modalities can provide supportive data. […] A magnetic resonance imaging (MRI) image of the brain can reveal asymmetric atrophy or shrinking in and around areas that subserve motor function. […] Functional imaging, specifically fluorodeoxyglucose-positron emission tomography (FDG-PET) or single-photon emission computed tomography (SPECT), can reveal asymmetrically decreased activity in the brain.

• #13

  https://111.wales.nhs.uk/encyclopaedia/c/article/corticobasaldegeneration/

  Corticobasal degeneration (CBD) is a rare condition that can cause gradually worsening problems with movement, speech, memory and swallowing. […] Diagnosing CBD can be difficult, as there’s no single test for it, and the condition can have similar symptoms to a number of others. […] A diagnosis of CBD will be based on the pattern of your symptoms. […] The diagnosis will usually be made by a neurologist (specialist in conditions affecting the brain and nerves). […] If you have symptoms of CBD that suggest there is something wrong with your brain, it is likely that you will be referred for a brain scan. […] Brain scans are also being developed to show the build-up of a protein called tau. This is associated with CBD. […] A person’s symptoms usually help distinguish CBD from Parkinson’s disease, but sometimes tests may be used to support the diagnosis and rule out other possible conditions.

• #14 Corticobasal degeneration (corticobasal syndrome) – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/corticobasal-degeneration-corticobasal-syndrome/

  Corticobasal degeneration (corticobasal syndrome) can be caused by several underlying pathologies. […] A diagnosis of corticobasal degeneration (corticobasal syndrome) is made based on your signs and symptoms. However, your signs and symptoms could be due to another degenerative disease such as progressive supranuclear palsy, Alzheimer’s disease, Pick’s disease and Creutzfeldt-Jakob disease. You may need an imaging test, such as a CT scan or MRI, to rule out these other conditions. […] Researchers are looking at whether positron emission tomography (PET) and single-photon emission computerized tomography (SPECT) scans can uncover changes in the brain that are consistent with corticobasal degeneration. However, more research needs to be done in this area.

• #15 Cortiocobasal Degeneration: Essential Facts For Patients

  https://www.movementdisorders.org/MDS/Resources/Patient-Education/Cortiocobasal-Degeneration.htm

  The diagnosis is based on your medical history and neurological examination. […] Because CBD signs and symptoms may be similar to other diseases, such as Parkinsons disease, it can be difficult to diagnose in the early stages. […] Even later on, diagnosis can be difficult. […] Sometimes the diagnosis is only made by autopsy. […] Scans like magnetic resonance imaging (MRI) are often useful. […] Imaging may rule out other diseases that may mimic CBD. […] Imaging may also find specific patterns of brain shrinkage, called atrophy. […] There are no blood tests for the diagnosis.

• #16 Corticobasal syndrome: a practical guide | Practical Neurology

  https://pn.bmj.com/content/21/4/276

  An MR scan of brain showed only mild involutional changes consistent with age but no perirolandic atrophy. […] Our impression was likely corticobasal syndrome. […] MR scan of brain showed left perirolandic atrophy consistent with corticobasal syndrome. […] Probable corticobasal degeneration criteria require an insidious onset and gradual progression for at least 1 year, age at onset 50 years, no similar family history or known tau mutations, and one of the clinical phenotypes outlined above. […] The estimated prevalence of corticobasal degeneration is 4.97.3 cases per 100000 population. […] The typical age of presentation is 50s70s and average lifespan from diagnosis to death is 7 years. […] Corticobasal syndrome has an insidious onset and is slowly progressive. […] Patients with dramatic presentations and/or rapidly progressive disease courses should be considered mimics.

• #17 Corticobasal syndrome: a practical guide | Practical Neurology

  https://pn.bmj.com/content/21/4/276

  Limb apraxia is among the most commonly identified signs that suggests cortical dysfunction in the corticobasal syndrome, occurring in 70%80%. […] The traditional oculomotor hallmark of clinically diagnosed corticobasal syndrome is saccade apraxia, which manifests clinically as difficulty and delay in initiating saccades towards a target. […] The typical language disturbance in corticobasal syndrome is non-fluent variant primary progressive aphasia, with slowed, effortful and/or groping (apraxia of speech) speech and grammatical errors being common. […] There are currently no proven treatments for corticobasal syndrome. […] Conditions that may initially be diagnosed as corticobasal syndrome but turn out to be something else tend to be those with subacute or chronic onset. […] The prognosis for a patient diagnosed with corticobasal syndrome depends mainly on the underlying neuropathology. […] In summary, a corticobasal syndrome diagnosis can be supported but not refuted by imaging features, while emerging techniques may direct the neurologist to the underlying pathological cause of a patient’s syndrome.

• #18

  https://www.nhs.uk/conditions/corticobasal-degeneration/

  Corticobasal degeneration (CBD) is a rare condition that can cause gradually worsening problems with movement, speech, memory and swallowing. […] There’s no single test for CBD. Instead, the diagnosis is based on the pattern of your symptoms. Your doctor will try to rule out other conditions that can cause similar symptoms, such as Parkinson’s disease or a stroke. […] You may need to have a brain scan to look for other possible causes of your symptoms, as well as tests of your memory, concentration and ability to understand language. […] You will usually see a neurologist (a specialist in conditions affecting the brain and nerves). […] Read more about how CBD is diagnosed.

• #19

  https://www.nhs.uk/conditions/corticobasal-degeneration/diagnosis/

  It can be difficult to diagnose corticobasal degeneration (CBD), as there’s no single test for it, and the condition can have similar symptoms to a number of others. […] A diagnosis of CBD will be based on the pattern of your symptoms. Your doctor will also try to rule out conditions that can cause similar symptoms, such as Parkinson’s disease, a stroke and Alzheimer’s disease. […] The diagnosis will usually be made by a neurologist (specialist in conditions affecting the brain and nerves). […] MRI scans can also detect abnormal changes to the brain that are consistent with a diagnosis of CBD, such as shrinkage of certain areas. […] Brain scans are also being developed to show the build-up of a protein called tau. This is associated with CBD. […] A person’s symptoms usually help distinguish CBD from Parkinson’s disease, but sometimes tests may be used to support the diagnosis and rule out other possible conditions. […] If it doesn’t lead to a significant improvement in your symptoms, it can help your doctor distinguish CBD from Parkinson’s disease. […] Most people with CBD have a distinct pattern of difficulties with these tests.

• #20 Corticobasal Syndrome and Corticobasal Degeneration Workup: Approach Considerations, Laboratory Studies, Imaging Studies

  https://emedicine.medscape.com/article/1150039-workup

  All patients require brain MR imaging and a serum workup for reversible/effectively treatable etiologies (although CBS and other CBD presentations are not diagnoses of exclusion). FDG-PET may be useful in selected cases. Emerging tau PET ligands can image tau pathology in vivo and are currently available on a research basis. […] MRI is helpful in evaluating the size and appearance of the midbrain if any disturbance of vertical eye movements is noted and progressive supranuclear palsy is being considered. Midbrain size should be relatively normal in typical CBD. […] Position emission tomography (PET) and single-photon emission computed tomography (SPECT) reveal asymmetric activity in both cortical (frontal-parietal) and subcortical (basal ganglia) regions. Recent FDG-PET studies in CBD presenting as CBS have shown marked asymmetric hypometabolism of the contralateral frontoparietal cortex (including the superior, middle and inferior frontal gyri, pre- and post-central gyri, superior parietal lobule and supramarginal gyrus), as well as the thalamus and caudate nuclei.

• #21

  https://www.brain.northwestern.edu/dementia/cbs/diagnosis.html

  A dopamine transporter (DaT) SPECT can be abnormal in CBD, though this study does not discriminate CBD from other pathologies, such as Parkinsons disease, Lewy Body disease, multiple system atrophy, and progressive supranuclear palsy (PSP). […] Finally, a lumbar puncture and cerebrospinal fluid analysis can diagnose underlying Alzheimers disease, another possible cause of CBS.

• #22 Corticobasal degeneration – Wikipedia

  https://en.wikipedia.org/wiki/Corticobasal_degeneration

  Despite the undoubted presence of cortical atrophy (as determined through MRI and SPECT) in individuals experiencing the symptoms of CBD, this is not an exclusive indicator for the disease. Thus, the utilization of this factor in the diagnosis of CBD should be used only in combination with other clinically present dysfunctions. […] SPECT is currently being used to try to detect CBD. With many patients of CBD, there are areas in the basal ganglia which have difficulties receiving dopamine, typically asymmetrically. […] Research has suggested that the integrity of the dopamine system in the striatum has been damaged as an effect of CBD. Current studies employing the use of FDOPA PET scanning (FDOPA PET) as a possible method for identifying CBD have focused on analyzing the efficiency of neurons in the striatum that utilize the neurotransmitter dopamine.

• #23 Corticobasal degeneration – Wikipedia

  https://en.wikipedia.org/wiki/Corticobasal_degeneration

  Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. Diagnosis is difficult, as symptoms are often similar to those of other disorders, such as Parkinson’s disease, progressive supranuclear palsy, and dementia with Lewy bodies, and a definitive diagnosis of CBD can only be made upon neuropathologic examination. […] New diagnostic criteria known as the Armstrong criteria were proposed in 2013, although the accuracy of these is limited and further research is needed. […] Recent findings in clinicopathology have made it possible to distinguish CBD from Parkinson’s and increase the accuracy of diagnosis, using developments in MRI and nuclear medicine. […] The diagnostic criteria for clinical use may result in a misdiagnosis of other tau-based diseases.

• #24 Corticobasal Degeneration | Pacific Movement Disorders

  https://www.pacificneuroscienceinstitute.org/movement-disorders/conditions/atypical-parkinsonism/corticobasal-degeneration/

  Corticobasal degeneration (CBD) is extremely rare and manifests as very asymmetric (often unilateral) parkinsonism with rigidity, apraxia (trouble coordinating the limb to accomplish a task), myoclonus (muscle jerks), dystonia, and rarely a condition known as alien limb syndrome, where the limb has complex movements of its own accord. […] The diagnosis is primarily clinical but functional imaging such as FDG-PET can elucidate the patterns of metabolic dysfunction seen in CBD. Research is being done to develop imaging that directly measures tau protein deposition, which will enable more specific and accurate diagnosis.

• #25 Corticobasal degeneration | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/corticobasal-degeneration?lang=us

  Research is ongoing regarding the use of techniques such as 3D volumetric MRI and diffusion tensor imaging. […] There are no drug therapies available to modify the course of corticobasal degeneration. Treatment is often focused on symptomatic relief. […] Clinically there is overlap with other conditions, some of which also cause corticobasal syndrome.

• #26 Corticobasal degeneration | MedLink Neurology

  https://www.medlink.com/articles/corticobasal-degeneration

  Advances in volumetric MR imaging and paired disease progression modeling are being made to aid in diagnosis. […] Asymmetry of metabolic activity in the parietal lobe contralateral to limb apraxia can also be documented by PET scans. […] The presence of at least two of the following features would move the diagnosis to probable over possible: asymmetric rigidity, limb dystonia, myoclonus, apraxia, cortical sensory deficit, alien limb phenomena. […] In utilizing neuropsychological testing, patterns of apraxia deficit may help differentiate cases of corticobasal degeneration from those with progressive supranuclear palsy.

• #27 Studies: Corticobasal Degeneration

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/research/corticobasal-degeneration.html

  The purpose of this study is to analyze the effectiveness of a procedure called a skin punch biopsy to lead to an earlier diagnosis. Skin biopsy could be a useful way to diagnose and measure the severity of these conditions. […] This is a 5-year longitudinal investigating the best method of analysis to track patients with corticobasal syndrome, corticobasal degeneration, progressive supranuclear palsy, behavioral variant of frontotemporal degeneration, primary progressive aphasia, and other forms of frontotemporal lobar degeneration with the overall goal to prepare for treatment trials. The results will provide information about the value of novel imaging techniques for diagnosis and value of novel imaging techniques in comparison to biofluid biomarkers. […] This study is investigating the best method of analysis to track patients with corticobasal syndrome, corticobasal degeneration, progressive supranuclear palsy, and oligo- or variant-progressive supranuclear palsy. The results will provide information about the value of novel imaging techniques for diagnosis and value of novel imaging techniques in comparison to biofluid biomarkers.

• #28 Corticobasal Degeneration: Symptoms, Causes, Diagnosis and Treatment

  https://medisearch.io/blog/corticobasal-degeneration

  In order to diagnose CBD, we would need to determine whether the accumulation of the abnormal tau protein occurs both in the astrocytes and neurons. But as we mentioned above, this is not possible. […] Additionally, there are no exact biomarkers for CBD. […] One way clinicians diagnose corticobasal degeneration is by ruling out other disease. […] However, recent advances offer hope in the diagnosis of CBD. […] In 2022, researchers found that there is a correlation between specific fragments of the abnormal tau protein in the brain and cerebrospinal fluid. This could be a biomarker, specific to CBD. […] Because of the difficulty in proper diagnosis of cortiocobasal degeneration, there is no effective treatment available. […] However, the results of whether these are helpful are inconsistent but interventions such as physical therapy are suggested. […] To summarize, Corticobasal degeneration is a rare neurodegenerative disorder with a diverse range of symptomatic phenotypes, making its diagnosis challenging. […] However the lack of specific biomarkers makes post-mortem diagnosis the only definitive method.

• #29 Living with CBS :: Fight Parkinson’s – Together we can

  https://www.fightparkinsons.org.au/living-with-cbs/

  Corticobasal Syndrome (CBS) is a rare progressive neurological disorder resulting from degeneration in a specific part of the brain. […] CBS is a form of Atypical Parkinsonism, meaning that some of the symptoms are similar to those experienced by people with Parkinsons disease, for example stiff muscles and slowness of movement. […] Many people are initially diagnosed with Parkinsons due to the similarity of symptoms. Treatments for Parkinsons are generally ineffective in treating CBS. […] CBS is difficult to diagnose. There are no blood tests or brain scans that can diagnose it, although tests and scans are commonly used to rule out other conditions that can cause similar symptoms. […] A limited response to Parkinsons medication is a key indicator of the need to review a diagnosis of Parkinsons. […] It is important to see a neurologist (a doctor specialising in brain conditions). People with CBS should visit their neurologist regularly for ongoing treatment and advice.

• #30 Criteria for the diagnosis of corticobasal degeneration

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3590050/

  Definition and standardization of clinical diagnostic criteria for CBD are critical, especially as potential neuroprotective therapies for tauopathies emerge. […] Thus, we propose 2 sets of criteria: a narrower, more specific one for probable CBD and a broader set for possible CBD that has less specificity for CBD pathology while still representing probable tau-based pathology. […] Given the complex clinicopathologic correlations and varying phenotypes over time, it is not surprising that developing CBD diagnostic criteria has proven challenging. […] The 4 clinical phenotypes thought to be most representative of CBD were CBS, frontal behavioral-spatial syndrome, nonfluent/agrammatic variant of primary progressive aphasia, and PSPS. […] Both sets of criteria require insidious onset and gradual progression with symptom duration of at least 1 year to exclude rapidly progressive conditions more likely to represent other pathologies (e.g., CJD).

• #31 Corticobasal Degeneration (Corticobasal Syndrome): What It Is

  https://my.clevelandclinic.org/health/diseases/22522-corticobasal-degeneration

  Corticobasal degeneration (corticobasal syndrome) is a rare brain condition that causes you to lose your ability to speak, move and remember. Symptoms are progressive, so they slowly get worse over time. […] Corticobasal degeneration may shorten your life expectancy and lead to life-threatening complications. As symptoms affect more of your body, you may find it difficult to safely maintain your independence. […] Corticobasal degeneration is different, in that you dont receive an official diagnosis until after death. This happens because a provider or pathologist can locate and confirm brain degeneration. During your lifetime, your healthcare provider will diagnose the same condition as corticobasal syndrome based on your presenting symptoms. […] To make a diagnosis, your healthcare provider will conduct a physical exam. Theyll carefully evaluate your symptoms.

• #32 Corticobasal Degeneration – EyeWiki

  https://eyewiki.org/Corticobasal_Degeneration

  CBD has a very heterogeneous clinical spectrum, and it can manifest as multiple phenotypes. […] These phenotypes broaden CBD criteria and aid in diagnosis; however, several other disorders can mimic these CBD phenotypes. […] Coupled with the lack of antemortem confirmation, CBD is commonly misdiagnosed and underdiagnosed. […] Thus, CBD diagnosis remains difficult, and diagnostic specificity and sensitivity must be refined.

• #33 Corticobasal degeneration | MedLink Neurology

  https://www.medlink.com/articles/corticobasal-degeneration

  Corticobasal degeneration is one of the atypical parkinsonian syndromes or parkinsonism-plus syndromes most often characterized by parkinsonism (typically unilateral); apraxia; cortical sensory signs; dystonia; involuntary movements, such as myoclonus; and alien limb sign. […] Although there is no cure and treatment remains symptomatic, accurate diagnosis can help with patient and family counseling and guide the treating physicians. […] Currently, no specific treatment is available for corticobasal degeneration. […] Revised clinical diagnostic criteria by Armstrong and colleagues identify clinical characteristics that can aid in the diagnosis and distinction from other parkinsonian and dementia disorders. […] The diagnosis of corticobasal degeneration is primarily a clinical one based on history and examination. However, studies such as the DaTscan can be used as supportive studies, though the DaTscan cannot distinguish between neurodegenerative parkinsonian disorders.

• #34 Unravelling Genetic Factors Underlying Corticobasal Syndrome: A Systematic Review

  https://www.mdpi.com/2073-4409/10/1/171

  We suggest a diagnostic algorithm to help in identifying potential genetic cases of CBS in order to improve the diagnostic accuracy and to better understand the still poorly defined underlying pathogenetic process. […] Identifying genetic variants underlying CBS has a great practical relevance, since it allows appropriate family counseling and the possibility to be enrolled in clinical trials with disease-modifying treatments. […] Positive family history and an earlier age at onset are the two main clues that should lead clinicians to suspect and test for genetic conditions. […] Our review of genotype–phenotype correlation in corticobasal syndrome emphasizes the importance of a complete and in-depth clinical assessment.

• #35 SciELO Brazil – Corticobasal syndrome: A diagnostic conundrum Corticobasal syndrome: A diagnostic conundrum

  https://www.scielo.br/j/dn/a/TMZQJYmKy8NmrSW9FKC9wpf/

  The majority of causes of CBS are tauopathies. […] The better characterization of clinical, neuropsychological and imaging features is important to improve antemortem diagnosis and crucial for designing therapies. […] There is growing interest in developing disease-specific biomarkers to aid the prediction of pathology in the antemortem diagnosis of neurodegenerative disorders. […] The first study to use amyloid imaging in CBS included 14 CBS patients to undergo PiB-PET imaging, four (28.6%) were PiB-positive -patients with high PiB binding, a standardized uptake ratio 1.5- and the remaining were PiB-negative (71.4%). […] There is no specific treatment for CBS, but the ability to accurately detect underlying pathology early in the course of CBS will be crucial when effective therapies are developed. […] The wide range of cognitive, behavioral and motor aspects is extremely variable between patients.

• #36 Corticobasal Degeneration (Corticobasal Syndrome): What It Is

  https://my.clevelandclinic.org/health/diseases/22522-corticobasal-degeneration

  As many conditions share similar symptoms, your provider will run tests to determine the cause. These may include: Blood tests, Imaging scans (MRI or CT scan), Neuropsychological testing to assess brain function and memory. […] There isnt an available treatment option to slow the progression of symptoms or cure the condition. […] The prognosis (outlook) is poor for corticobasal degeneration. Its a progressive disease, which means itll get worse over time. It usually happens slowly over many years. Your provider will offer treatment options to help you cope and manage symptoms. Treatment options may reduce the severity of symptoms, but it wont stop them from progressing. […] On average, many people live between six to eight years after the first symptoms of corticobasal degeneration appear. This isnt the case for everyone, and many people live much longer than this. Your healthcare provider is the best person to talk to about your situation because it may not match statistics.

• #37 SSA – POMS: DI 23022.605 – Corticobasal Degeneration – 09/16/2020

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022605

  Corticobasal Degeneration (CBD) is a rare progressive neurological disorder characterized by the degeneration of parts of the brain, including the cerebral cortex and basal ganglia. […] The diagnosis of CBD is based on: Computerized tomography (CT) or magnetic resonance imaging (MRI) scans; Single photon emission computerized tomography (SPECT) studies; or Positron emission tomography (PET) scans. […] People diagnosed with CBD experience gradual decline in motor and cognitive functioning, with some people eventually losing the ability to walk. […] The average survival time after diagnosis of CBD is approximately 8 years after diagnosis. […] There is no treatment to slow the progression of CBD. Treatment centers on the management of symptoms.

• #38 Corticobasal Degeneration

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/cbd.html

  Corticobasal degeneration (CBD) is a rare and gradually progressive neurodegenerative disorder that is difficult to diagnose and treat because its symptoms vary and are similar to those of other neurological disorders. […] Currently, there are no laboratory markers for diagnosis of CBD, but imaging and cerebrospinal studies are used to rule out other disorders. […] There are no treatments that can stop or slow the progressive nature of CBD. Treatment is targeted at reducing the symptoms, improving the quality of life and preventing complications. […] Pharmacologic therapies for the motor symptoms have limited benefit. Levodopa is usually not effective in improving the parkinsonism in CBD, but the poor responsiveness to levodopa therapy may support the diagnosis. […] There are no known therapies that could improve the cognitive disturbances patients with CBD experience. Cholinesterase inhibitors (such as donepezil, rivastigmine, or galantamine) are not useful in CBD.

• #39 Corticobasal Syndrome and Corticobasal Degeneration: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1150039-overview

  Thus, current diagnostic criteria lack specificity in identifying CBD pathology clinically (ante-mortem) and the main utility of the Armstrong et al. criteria lies in formally defining the clinical syndromes associated with CBD pathology. Future studies on biomarkers and imaging will likely be helpful in predicting CBD pathology more accurately.

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