Materiały źródłowe

• #1 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. […] Corticobasal degeneration, also known as corticobasal syndrome, is a neurological disorder that damages and destroys brain cells. […] The deterioration (breakdown) and death of brain cells and tissue cause corticobasal degeneration. […] Researchers believe that a protein called tau plays a role in corticobasal degeneration. […] Many people with corticobasal degeneration have a genetic variant (mutation) on chromosome 17 called the H1 haplotype. […] Researchers also know that this gene isnt the only factor that could cause corticobasal degeneration. […] Medical experts dont know why some people develop corticobasal degeneration. […] The prognosis (outlook) is poor for corticobasal degeneration. […] On average, many people live between six to eight years after the first symptoms of corticobasal degeneration appear.

• #2 Corticobasal Syndrome | Memory and Aging Center

  https://memory.ucsf.edu/dementia/corticobasal-syndrome

  Corticobasal syndrome (CBS) is a condition that causes changes in movement, language skills, or both. […] The cause of CBS is unknown. Scientists know that in some people with CBS, there is a large build-up of a protein called tau. […] Others may have a large build-up of amyloid plaques similar to those seen in people with Alzheimers disease. […] As more and more proteins build up in the nerve cells, the cells lose their ability to function and eventually die. […] CBS is a disease that changes with time.

• #3 Corticobasal Degeneration | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/corticobasal-degeneration

  Corticobasal degeneration (CBD) is a rare neurological disease associated with progressive brain degeneration. […] The brain tissue of patients with CBD show cell changes that also appear in patients with two other disorders frontotemporal dementia and progressive supranuclear palsy. […] Currently, there are no known causes, such as toxins or infections. […] As an international leader in the field of dementia disorders, we are actively researching the cause and progression of corticobasal degeneration. […] Significant advances in the understanding of CBD have been made and the UCSF Memory and Aging Center is actively involved in researching the cause and course of the disease. […] The UCSF Memory and Aging Center is involved in research to better understand the cause and course of the disease, which has led to counseling, support and medications to treat symptoms.

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

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

  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.

• #5

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

  Corticobasal degeneration (CBD) is caused by increasing numbers of brain cells becoming damaged or dying over time. […] CBD occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. […] Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with CBD, it isn’t broken down properly and forms harmful clumps in brain cells. […] CBD has been linked to changes in certain genes, but these genetic links are weak and the risk to other family members is very low.

• #6

  https://www.psp.org/iwanttolearn/corticobasal-degeneration

  Corticobasal degeneration (CBD) is a rare neurodegenerative disorder that has no known cause or cure. […] In people with CBD, some groups of brain cells break down and die off. Central to that process is the abnormal folding and clumping of a protein called tau, which is a normal component of the brain. Its job is to help maintain the microtubules, which are stiff rods that function as the brain cells internal transportation and skeletal system. It appears that the cells death is caused by the tau protein clumps themselves and not by the reduced availability of tau for its normal function. The clumps technical name is neurofibrillary tangles. […] We know several reasons why tau might misfold, but we dont know which one(s) are relevant to CBD. Tau forms similar aggregates in about a dozen other diseases, including Alzheimers disease, PSP, chronic traumatic encephalopathy and about half of all cases of frontotemporal dementia. The specific disease depends on the chemical characteristics of the tau aggregate and which brain cells are involved. We dont yet understand what determines these.

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

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

  Corticobasal degeneration (CBD), a sporadic neurodegenerative 4-repeat tauopathy, is a pathologically defined entity associated with several clinical phenotypes. […] The most characteristic are frontotemporal degeneration spectrum disorders, but Alzheimer disease and rare disorders such as Creutzfeldt-Jakob disease, CNS Whipple disease, and Niemann-Pick disease type C can be associated with corticobasal syndrome. […] The diagnosis of syndromes associated with CBD pathology is based on history and physical examination, while imaging, serum, and cerebrospinal fluid studies serve an ancillary role. […] CBD can also present clinically as progressive supranuclear palsy (there is significant clinical and some neuropathological overlap between these entities), a frontal behavioral-spatial syndrome, primary progressive aphasia, or (rarely) as posterior cortical atrophy.

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

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

  Understanding of the genetic underpinnings of CBD is limited. […] Although CBD is thought to arise sporadically in the vast majority of cases, several rare mutations in the microtubule-associated protein tau (MAPT) have been implicated as causative for CBS and CBD. […] A genome-wide association study of CBD identified several new CBD susceptibility loci and demonstrated that CBD and PSP share a genetic risk factor other than MAPT at 3p22 MOBP (myelin-associated oligodendrocyte basic protein).

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

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

  The pathophysiology of CBD is primarily driven by the accumulation of 4R hyperphosphorylated tau protein, which aggregates within neurons, astrocytes, and oligodendrocytes, leading to progressive neurodegeneration. […] The resultant tau inclusions disrupt neuronal function, contributing to synaptic dysfunction, axonal transport failure, and cell death. […] Beyond tauopathy, the neurodegenerative cascade in CBD involves mitochondrial dysfunction, impaired axonal transport, inflammation, and synaptic degeneration. […] Results from genetic studies have linked CBD to the H1 haplotype of the MAPT gene, which increases the risk of tau dysfunction and aggregation. […] Despite these advances, the precise triggers of tauopathy and the full extent of pathogenic pathways in CBD remain incompletely understood, underscoring the need for further research to facilitate targeted therapeutic development.

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

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

  CBD is primarily considered an idiopathic or sporadic neurodegenerative disorder, with familial cases being exceedingly rare. […] The only well-established risk factor for CBD is advanced age. […] At its core, CBD is driven by the pathological accumulation of hyperphosphorylated 4R tau protein, which disrupts microtubule stability, leading to neurodegeneration. […] Mutations in the microtubule-associated protein tau (MAPT) gene are central to the pathogenesis of CBD due to their role in disrupting membrane-associated 4R tau function. […] The extended MAPT tau haplotype (H1) and homozygosity for H1/H1 significantly increase in pathologically confirmed CBD cases, similar to progressive supranuclear palsy (PSP). […] Evidence of broader genetic pleiotropy has also emerged, linking CBD to immune-mediated diseases such as celiac disease, suggesting immune dysfunction may play a role in its pathogenesis.

• #9 Corticobasal degeneration – Wikipedia

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

  However, malfunctioning of the development of the protein can result in unnatural, high-level expression in astrocytes and glial cells. […] Neuropathological findings associated with CBD include the presence of astrocytic abnormalities within the brain and improper accumulation of the protein tau (referred to as tauopathy). […] The presence of parkinsonism as a clinical symptom of CBD is largely responsible for complications in developing unique diagnostic criteria for the disease. […] The diagnostic criteria for clinical use may result in a misdiagnosis of other tau-based diseases. […] Because the exact cause of CBD is unknown, there is no formal treatment for the disease. […] The prognosis for an individual diagnosed with CBD is death within approximately eight years.

• #10 Corticobasal Degeneration

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

  Corticobasal degeneration (CBD) is labeled as a tauopathy because a cell protein that normally exist in the walls of the cells or nourishment pipes called tau aggregates abnormally inside the neurons and other brain cells astrocytes and oligodendroglia. […] CBD is usually a non-hereditary (sporadic) disease, although rare familial cases of CBD have been reported. […] In CBD as in other tauopathies such as PSP there are polymorphisms in the tau gene that suggest there may be a genetic basis or at least a predisposition for this disorder. […] There have been no epidemiological studies of environmental or occupational risk factors.

• #10 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. […] Although the underlying cause of CBD is unknown, the disease occurs as a result of damage to the basal ganglia, specifically marked by neuronal degeneration or depigmentation (loss of melanin in a neuron) in the substantia nigra. […] Postmortem studies of patients diagnosed with CBD indicate that histological attributes often involve ballooning of neurons, gliosis, and tauopathy. […] In recent years corticobasal degeneration has come to be understood as a tauopathy. […] This is believed due to the most common indicator of CBD being a faulty tau protein. […] The protein tau is an important microtubule-associated protein (MAP), and is typically found in neuronal axons.

• #11 Cortiocobasal Degeneration: Essential Facts For Patients

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

  Corticobasal Degeneration (CBD) is a rare, progressive neurodegenerative disease. […] The cause of CBD is unknown. CBD causes an abnormal brain aging process that progresses over time. Healthy people have a protein called tau in their brains. It is important for normal neuron function. But people with CBD have an abnormal tau protein that builds up in the brain. It appears to damage nerve cells and other brain cells in certain areas. Researchers do not know why this tau protein is abnormal in CBD. CBD is not hereditary. It has not been linked to any environmental exposures.

• #12 Corticobasal Degeneration | UK Healthcare

  https://ukhealthcare.uky.edu/kentucky-neuroscience-institute/conditions/memory-cognitive-disorders/corticobasal-degeneration

  Corticobasal degeneration is a rare progressive neurologic disorder that causes atrophy (deterioration or shrinkage) of specific areas of the brain, often the cerebral cortex and basal ganglia, due to an abnormal buildup of tau protein. […] The cause of corticobasal degeneration is not well understood, so there are no known preventive measures known to reduce the risk of developing corticobasal degeneration. […] Corticobasal degeneration typically occurs spontaneously. There does not appear to be a significant genetic or environmental cause.

• #13 Corticobasal Syndrome and Corticobasal Degeneration Clinical Presentation: History, Physical, Causes

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

  The ultimate cause of corticobasal degeneration (CBD) is currently unknown, although symptoms are thought to be associated with progression of 4-repeat tau pathology and associated neuronal loss. […] Case reports suggest that a familial predisposition may exist in some individuals with this disorder. […] H1 tau haplotype homozygosity is associated with a predisposition to develop CBD and progressive supranuclear palsy (PSP). […] Rare mutations in the MAPT gene have been associated with CBD, as have C9orf72 repeat expansions and GRN (progranulin) mutations.

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

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

  Corticobasal syndrome (CBS) is an atypical parkinsonian presentation characterized by heterogeneous clinical features and different underlying neuropathology. […] Most CBS cases are sporadic; nevertheless, reports of families and isolated individuals with genetically determined CBS have been reported. […] GRN was the most common gene involved in CBS, representing 28 out of 58 cases, followed by MAPT, C9ORF72, and PRNP. […] A significant association was found between the presence of GRN mutations and specific clinical manifestations shared with FTD, including visuospatial impairment, behavioral changes, aphasia, and language alterations. […] CBS is generally recognized as a sporadic disorder, although rare familial and isolated genetic cases have been reported. When genetically determined, CBS has been mainly described in association with mutations in the genes encoding progranulin (GRN) or microtubule-associated protein tau (MAPT).

• #15

  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. […] CBD is caused by increasing numbers of brain cells becoming damaged or dying over time. […] CBD occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. […] Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with CBD, it isn’t broken down properly and forms harmful clumps in brain cells. […] CBD has been linked to changes in certain genes, but these genetic links are weak and the risk to other family members is very low.

• #16

  https://www.aurorahealthcare.org/services/neuroscience/neurology/neurological-conditions/corticobasal-degeneration

  Corticobasal degeneration (CBD), sometimes referred to as corticobasal syndrome (CBS) is a serious brain condition where over time, you start having trouble walking and talking. […] Sometimes, genetic mutations can cause corticobasal degeneration. […] While research doesnt point to a specific cause of corticobasal degeneration, they know that it is the result of damage caused by a buildup of the protein tau. Genetics also plays a factor in the development of corticobasal degeneration. […] Even though a gene mutation causes corticobasal degeneration, genetic testing cannot predict or detect it.

• #17 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. […] The corticobasal syndrome has causes in addition to corticobasal degeneration, including progressive supranuclear palsy (PSP-CBS), frontotemporal dementia, posterior cortical atrophy variant of Alzheimer disease, and Creutzfeldt-Jakob disease. […] There are no drug therapies available to modify the course of corticobasal degeneration. Treatment is often focused on symptomatic relief. […] Unfortunately, the overall prognosis is poor, with patients demonstrating gradual neurological decline. Death occurs typically 5 to 10 years after the diagnosis is first made.

• #18 Corticobasal syndrome – Wikipedia

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

  Corticobasal syndrome (CBS) is a rare, progressive atypical Parkinsonism syndrome and is a tauopathy related to frontotemporal dementia. CBS is typically caused by the deposit of tau proteins forming in different areas of the brain. […] CBD is the pathology underlying approximately 50% of CBS cases. […] Other degenerative pathologies that can cause corticobasal syndrome include: Alzheimer’s disease, Pick’s disease with Pick bodies, Lewy body dementias, Neurofilament inclusion body disease, CreutzfeldtJakob disease, Frontotemporal degeneration due to progranulin gene mutation, Motor neuron disease inclusion dementia. […] The symptoms of classic CBS differ from CBD in that CBD also includes cognitive deficits in the executive functions.

• #19 Neuropathology and emerging biomarkers in corticobasal syndrome | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/93/9/919

  CBD accounted for less than half of patients with CBS, while PSP and AD were the most common non-CBD pathological diagnoses. […] Pathologically, CBD can be divided into three subtypes on the basis of the distribution and severity of the lesions: typical CBD, basal ganglia predominant CBD and PSP-like CBD. […] Both CBD and PSP share genetic risk factors, such as MAPT H1 haplotype. […] MAPT mutations or variants have been reported to cause various types of tau pathologies, including CBD, Picks disease, GGT and unclassifiable tauopathies. […] CBS has been reported as a phenotype of FTLD-TDP. […] A recent study reported five patients with MAPT p.P301T mutation from two pedigrees: the clinical syndrome was CBS in three patients with age of onset in the 40s in two, FTD in one patient, and primary lateral sclerosis in one patient.

• #20 Corticobasal Syndrome Stages and Treatment | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/corticobasal-syndrome

  Corticobasal syndrome is a rare degenerative neurological disease. The cause of corticobasal syndrome is not clearly known. Research has shown that people with this condition have a significant buildup of tau protein in the brain. As the protein collects in the brain nerve cells, it causes the cells to degenerate and die. Cell death is what leads to brain shrinkage (atrophy). […] Research has also discovered a buildup of amyloid plaques, similar to those found in Alzheimers disease, in some cases. Atypical Alzheimers has also been shown to be a possible cause of corticobasal syndrome. […] Corticobasal syndrome is not considered an inherited disease, although research has found a gene mutation on chromosome 17 (H1 haplotype) that may increase the production of tau protein, causing it to buildup in the nerve cells. The gene mutation may also cause a carbon atom and hydrogens to stick to the tau gene, negatively impacting its functioning.

• #20 Corticobasal Syndrome Stages and Treatment | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/corticobasal-syndrome

  It is important to note that this gene mutation impacts 3 out of every 4 people in the general population and does not necessarily indicate that corticobasal syndrome will develop. […] Research is currently investigating whether environmental factors (exposure to chemicals or toxins) or traumatic brain injuries have an impact on the development of corticobasal syndrome. Additionally, research has indicated that a gene mutation (gene 17) may also play a role in the development of corticobasal syndrome.

• #21 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. Most commonly, corticobasal degeneration is characterized by a buildup of tau in brain cells, which may lead to their deterioration and the symptoms of corticobasal degeneration. […] The second most common cause of corticobasal degeneration is atypical Alzheimer’s disease. […] Other causes include progressive supranuclear palsy, Pick’s disease and Creutzfeldt-Jakob disease.

• #22 Corticobasal Syndrome – Frontotemporal Degeneration | AFTD

  https://www.theaftd.org/what-is-ftd/corticobasal-syndrome/

  Upon autopsy, corticobasal degeneration is characterized by nerve cell loss, gliosis and atrophy (shrinkage) of the deeper layers in the posterior frontal and/or parietal lobes, and the substantia nigra. […] Different underlying protein pathologies can be found at autopsy in clinically diagnosed CBS that may be FTLD related tau protein (4R type) or TDP43 protein. Alternatively, Alzheimer’s disease pathology has been observed with amyloid plaques and tau tangles, or alpha synuclein protein pathology more typical of Lewy body disease.

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

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

  The prognosis for a patient diagnosed with corticobasal syndrome depends mainly on the underlying neuropathology (i.e. cause), the difficulty being that that cause is not easily determined during life. […] In summary, a corticobasal syndrome diagnosis can be supportedbut not refutedby imaging features, while emerging techniques may direct the neurologist to the underlying pathological cause of a patients syndromeinformation that over time will have practical relevance.

• #24 Corticobasal Syndrome | Baylor Medicine

  https://www.bcm.edu/healthcare/specialties/neurology/parkinsons-disease-and-movement-disorders/corticobasal-syndrome

  Corticobasal syndrome (CBS) is a form of atypical parkinsonism (a parkinsonism-plus syndrome), which means that it shares some features with Parkinson’s disease such as slowness of movement (bradykinesia), stiffness (rigidity), tremor, and postural instability (balance difficulties). […] CBS results in gradual loss of nerve cells (neurodegeneration) in the surface of the brain (the cerebral cortical areas) as well as deep structures (the basal ganglia). […] The cause of CBS is not yet known. Like other neurodegenerative diseases, patients with CBS accumulate misfolded proteins within specific brain cells. […] Mishandling of tau, a protein that normally acts to stabilize the cellular skeleton of neurons (nerve cells) and also accumulates in the brains of patients with Alzheimer disease, appears to play a major role but the details remain unclear. […] In some cases, there is a peripheral injury or acute central damage (e.g. brain stroke or trauma) suggesting an initial trigger for subsequent neurodegeneration, thus possibly explaining the striking and persistent asymmetry of clinical, imaging and pathological findings.

• #25 Corticobasal degeneration | Monash Health

  https://monashhealth.org/services/movement-disorders-program/understanding-movement-disorders/corticobasal-degeneration/

  Corticobasal Degeneration (CBD) is a rare and progressive neurodegenerative disorder. […] The deterioration in brain cells in several areas of the brain, including the cortex and the basal ganglia leads to the symptoms found in CBD. […] In CBD there is an abnormal build-up of the protein called tau in the brain. The abnormal tau is thought to damage nerve and brain cells in certain regions of the brain. It is not known what triggers this build-up of tau. […] It remains uncertain if there is a genetic link to CBD. Changes to the tau gene are associated with CBD. […] No environmental or infective factors have been linked to CBD to date.

• #26 Corticobasal degeneration – Parkinson’s Australia

  https://www.parkinsons.org.au/information-hub/corticobasal-degeneration/

  Corticobasal degeneration (CBD), also known as corticobasal syndrome (CBS), is a rare neurological disorder considered an Atypical Parkinson’s condition. […] Currently, the cause of Parkinson’s Corticobasal Degeneration is not known, but several different factors are thought to be involved including age, genetic and environmental influences. It is thought to be linked to abnormal cellular Tau (a naturally occurring brain protein) changes. […] The underlying cause of CBD is often associated with the buildup of the protein tau in brain cells, leading to their deterioration. This is like other neurodegenerative diseases including Alzheimer’s and PSP. […] CBD has no known genetic links. It is less common than other atypical Parkinsonian syndromes like Multiple System Atrophy (MSA) or Progressive Supranuclear Palsy (PSP).

• #27 Corticobasal Degeneration – EyeWiki

  https://eyewiki.org/Corticobasal_Degeneration

  Corticobasal degeneration (CBD) is a rare, progressive neurodegenerative disease characterized by nerve cell loss and atrophy of multiple areas of the brain. […] The exact etiology of CBD is unknown but increased tau phosphorylation and vesicle trafficking dysfunction have been implicated while multiple susceptibility loci for these factors have been discovered. […] It has been proposed that a seeding process induces the propagation of tau pathology in CBD. […] Currently, the only other known risk factor for CBD is advanced age, and there has been no evidence to suggest that environmental factors generate pathogenesis of CBD. […] The pathophysiology of CBD is not entirely known. It is hypothesized that tau dysfunction is the main driving force for CBD pathogenesis. […] This accumulation of tau is termed tau seeding and results in the recruitment of normal tau by pathological tau species, yielding tau aggregate formation. […] Tau pathology spreads to other cells. Prion-like cell-to-cell spreading has been suggested as an explanation.

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

  https://radiopaedia.org/articles/corticobasal-degeneration?embed_domain=external.radpair.com%252527%25255b0%25255d%252527%25255b0%25255dfavicon.icofavicon.icofavicon.icofavicon.icofavicon.ico&lang=gb

  Corticobasal degeneration (CBD) is an uncommon neurodegenerative disease and is one of the subset of tauopathies. […] 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. The corticobasal syndrome has causes in addition to corticobasal degeneration, including progressive supranuclear palsy (PSP-CBS), frontotemporal dementia, posterior cortical atrophy variant of Alzheimer disease, and Creutzfeldt-Jakob disease. […] The characteristic histopathological findings are neuronal loss and numerous „ballooned” achromatic neurones. […] There are no drug therapies available to modify the course of corticobasal degeneration. Treatment is often focused on symptomatic relief.

• #29 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. Depending on whom you ask, its classified as an atypical parkinsonism disorder, a non-Alzheimers dementia, or a type of frontotemporal degeneration. CBD is most closely similar to PSP in terms of symptoms and pathology. Many organizations that perform research on PSP also research CBD. […] Because CBD is so rare, research has been very limited. Most studies of autopsy-confirmed disease only include a few dozen cases. Its hard to be definitive with such small numbers. […] 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. […] Since 2013, CBD experts refer to four main types of CBD:

• #30 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 average survival time after diagnosis of CBD is approximately 8 years after diagnosis. Death is generally caused by pneumonia or other complications of severe debility such as sepsis (bacterial infection) or pulmonary embolism. […] There is no treatment to slow the progression of CBD. Treatment centers on the management of symptoms. However, drugs used to treat the symptoms of this disease do not produce any significant or sustained improvement.

• #31 Corticobasal degeneration (corticobasal syndrome) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/corticobasal-degeneration/diagnosis-treatment/drc-20475446

  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. […] Positron emission tomography (PET) scans can identify brain changes related to corticobasal degeneration. However, more research needs to be done in this area. […] There are no treatments that help slow the progression of corticobasal degeneration (corticobasal syndrome). But if your symptoms are due to Alzheimer’s disease, new medicines may be available. Your healthcare professional may recommend medicines to try to manage your symptoms. […] Corticobasal degeneration and corticobasal syndrome: A review. Clinical Parkinsonism Related Disorders. 2019; doi:10.1016/j.prdoa.2019.08.005.

• #32 Corticobasal Syndrome (CBS): Causes, Symptoms, Treatment

  https://www.healthline.com/health/parkinsons/corticobasal-syndrome

  Currently, doctors dont know of any ways to prevent CBS. More research is required to pinpoint the causes of this condition. Knowing its causes may also help us better understand how to prevent CBS. […] Because theres no treatment for CBS, it will eventually lead to serious complications. For example, difficulty swallowing can cause choking or inhaling food or liquid into your airways. This can cause pneumonia, a potentially life threatening complication. […] Because of these complications, people with CBS live an average of 6 to 8 years from the onset of symptoms. […] CBS is a rare neurodegenerative disorder that gradually worsens your physical and cognitive functions. The buildup of a toxic protein in your brain cells called tau causes CBS. […] No treatment exists for this condition, but some drugs and other therapies can help you manage its symptoms. Research on new therapies and diagnostic methods is ongoing, giving hope to people with this condition and their caregivers.

• #33 Corticobasal Degeneration CBD | Definitions, Symptoms & Advice

  https://www.pspassociation.org.uk/what-is-cbd/

  Corticobasal Degeneration (CBD) involves the degeneration of brain cells caused by the accumulation of abnormal tau proteins. […] The primary cause of Corticobasal Degeneration (CBD) is the abnormal accumulation of tau protein within the brains neurons. […] The exact cause of tau clumping in CBD is still being studied. Genetic factors may play a role, although most CBD cases appear sporadically and rarely run in families. Environmental factors and other yet-to-be-identified triggers are also thought to contribute to the development of the disease. […] Research into CBD is focused on understanding its pathophysiology, improving diagnostic accuracy, and developing effective treatments. Studies primarily investigate the abnormal accumulation of tau protein in brain cells, which is believed to be a critical factor in the development of the disease. Researchers are exploring genetic factors that may predispose individuals to CBD, as well as environmental influences that could trigger its onset.

• #34 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. […] Corticobasal degeneration is a different condition pathologically than Parkinsons disease (PD); it is known as a tauopathy, meaning that the tau protein misfolds and causes disruption of neuron function, whereas in PD the abnormal protein is called alpha-synuclein. […] Unfortunately, CBD can progress more rapidly than typical (idiopathic) PD, and patients typically lose the ability to walk or care for themselves within a few years of the onset of symptoms. […] Research is being done to develop imaging that directly measures tau protein deposition, which will enable more specific and accurate diagnosis. […] There are small studies that are enrolling to modify the tau protein but are in very early stages.

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