215:(IMA), although clearly present in CBD, often manifests atypically due to the additional presence of bradykinesia and rigidity in those individuals exhibiting the disorders. The IMA symptom in CBD is characterized by the inability to repeat or mimic particular movements (whether significant or random) both with or without the implementation of objects. This form of IMA is present in the hands and arms, while IMA in the lower extremities may cause problems with walking. Those with CBD that exhibit IMA may appear to have trouble initiating walking, as the foot may appear to be fixed to floor. This can cause stumbling and difficulties in maintaining balance. IMA is associated with deterioration in the premotor cortex, parietal association areas, connecting white matter tracts, thalamus, and basal ganglia. Some individuals with CBD exhibit limb-kinetic apraxia, which involves dysfunction of more fine motor movements often performed by the hands and fingers.
204:(German, meaning "to go with") is relatively specific to CBD, and involves the active following of an experimenter's hand by the subject's hand when both hands are in direct contact. Another, rarer form of alien hand syndrome has been noted in CBD, in which an individual's hand displays an avoidance response to external stimuli. Additionally, sensory impairment, revealed through limb numbness or the sensation of prickling, may also concurrently arise with alien hand syndrome, as both symptoms are indicative of cortical dysfunction. Like most of the movement disorders, alien hand syndrome also presents asymmetrically in those diagnosed with CBD.
32:
767:
improvement based on similar drugs/dopaminergic agonists. Because of the brain's levels of inhibition, some medications have focused on creating an inhibition that would negate the effect. Many of these relaxants and anticonvulsants have some success but also have unwanted side effects. Cognitive and associative effects of CBD are also hard to treat as the effects of many of the treatments for the symptomatic conditions that ensue such as dementia, aphasia, neglect, and apraxia are not well understood.
192:, and gait disorder, with limb rigidity forming the most typical manifestation of parkinsonism in CBD. Despite being relatively indistinct, this rigidity can lead to disturbances in gait and correlated movements. Bradykinesia in CBD occurs when there is notable slowing in the completion of certain movements in the limbs. In an associated study, it was determined that, three years following first diagnosis, 71% of persons with CBD demonstrate the presence of bradykinesia.
798:
forgotten" until 1989, when
Marsden et al. used the name corticobasal degeneration. In 1989 Gibb and colleagues provided detailed clinical and pathological descriptions in a further three cases adopting the name corticobasal degeneration, after which various other names included "corticonigral degeneration with nuclear achromasia" and "cortical basal ganglionic degeneration". Although the underlying cause of CBD is unknown, the disease occurs as a result of damage to the
277:. This process involves exposing tissue samples to a silver staining material which marks for abnormalities in the tau protein and astroglial inclusions. Astroglial inclusions in CBD are identified as astrocytic plaques, which present as annularly displays of blurry outgrowths from the astrocyte. A recent study indicated that produces a high density of astrocytic plaques in the anterior portion of the frontal lobe and in the premotor area of the cerebral cortex.
547:
symptoms correlated with the disease. However, this often results in complications as these symptoms often overlap with numerous other neurodegenerative diseases. Frequently, a differential diagnosis for CBD is performed, in which other diseases are eliminated based on specific symptoms that do not overlap. However, some of the symptoms of CBD used in this process are rare to the disease, and thus the differential diagnosis cannot always be used.
293:(MAP), and is typically found in neuronal axons. However, malfunctioning of the development of the protein can result in unnatural, high-level expression in astrocytes and glial cells. As a consequence, this is often responsible for the astrocytic plaques prominently noted in histological CBD examinations. Although they are understood to play a significant role in neurodegenerative diseases such as CBD, their precise effect remains a mystery.
228:, and thus is not a true aphasia, as aphasia is related to a change in language function, such as difficulty retrieving words or putting words together to form meaningful sentences. The speech and/or language impairments in CBD result in disconnected speech patterns and the omission of words. Individuals with this symptom of CBD often lose the ability to speak as the disease progresses.
139:, and although more cognitive 36% had dementia. In another study of 36, over half had a useless/alien arm and 27% had a gait impediment. From this, we can see why CBD is hard to diagnose. Even if it can be distinguished as different from one of the other similar diseases, the varying combinations of symptoms create a difficult path to diagnosis.
297:
affinity in binding with microtubules. Because of this increased affinity, they form insoluble fibers (also called "paired helical filaments"). Microtubules themselves keep the cell and cytoskeletal structure stable. Thus, when tau proteins create unnatural configurations, microtubules become unstable, which eventually leads to cell death.
663:. Overperfusion and underperfusion relate to a comparison with the overall perfusion levels within the entire body, whereas hypoperfusion and hyperperfusion are calculated in comparison to the blood flow requirements of the tissue in question. In general, the measurements taken for CBD using SPECT are referred to as regional
148:
and the symptoms are not observed uniformly throughout the body. For example, a person exhibiting an alien hand syndrome (explained later) in one hand, will not correspondingly display the same symptom in the other hand. Predominant movement disorders and cortical dysfunctions associated with CBD include:
784:
Clinical presentation of CBD usually does not occur until age 60, with the earliest recorded diagnosis and subsequent postmortem verification being age 28. Although men and women present with the disease, some analysis has shown a predominant appearance of CBD in women. Current calculations suggest
546:
One of the most significant problems associated with CBD is the inability to perform a definitive diagnosis while an individual exhibiting the symptoms associated with CBD is still alive. A clinical diagnosis of CBD is performed based upon the specified diagnostic criteria, which focus mainly on the
236:
Psychiatric problems associated with CBD often present as a result of the debilitating symptoms of the disease. Prominent psychiatric and cognitive conditions cited in individuals with CBD include dementia, depression, and irritability, with dementia forming a key feature that sometimes leads to the
114:
Because CBD is progressive, a standard set of diagnostic criteria can be used, which is centered on the disease's evolution. Included in these fundamental features are problems with cortical processing, dysfunction of the basal ganglia, and a sudden and detrimental onset. Psychiatric and cognitive
775:
The prognosis for an individual diagnosed with CBD is death within approximately eight years, although some patients have been diagnosed in 2000 were still in relatively good condition in 2017, albeit with serious debilitation such as dysphagia, and overall limb rigidity. The partial (or total) use
762:
Because the exact cause of CBD is unknown, there is no formal treatment for the disease. Instead, treatments focus on minimizing the appearance or effect of symptoms. The most easily treatable symptom of CBD is parkinsonism, most commonly treated with dopaminergic drugs. However, there is usually
654:
analysis throughout the parts of the brain. SPECT evaluation through perfusion observation consists of monitoring blood release into different locations in tissue or organ regions, which, in the case of CBD, pertains to localized areas within the brain. Tissue can be characterized as experiencing
626:
MRI images are useful in displaying atrophied portions of neuroanatomical positions within the brain. As a result, it is especially effective in identifying regions within different areas of the brain that have been negatively affected due to the complications associated with CBD. To be specific,
613:
Developments or improvements in imaging techniques provide the future possibility for definitive clinical diagnosis prior to death. However, despite their benefits, information learned from MRI and SPECT during the beginning of CBD progression tend to show no irregularities that would indicate the
766:
Many treatments have low success rates or have not been tested thoroughly or produced frequently. For example, in relation to the motor aspect of disability, CBD has high resistance against treatments to improve dopamine intake, such as levodopa. A number of studies have reported no real levels of
559:
Progressive supranuclear palsy (PSP) is frequently the disease most often confused with CBD. Both PSP and CBD result in similar symptoms, and both display tauopathies upon histological inspection. However, it has been noted that tauopathy in PSP results in tuft-shaped astrocytes in contrast with
147:
Some of the most prevalent symptom types in people exhibiting CBD pertain to identifiable movement disorders and problems with cortical processing. These symptoms are initial indicators of the presence of the disease. Each of the associated movement complications typically appears asymmetrically
617:
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
296:
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. Tau proteins are integral in keeping microtubules stable; defective cells create four microtubule-binding repeats with increased
749:
can display a corticobasal syndrome. It has been suggested that the nomenclature of corticobasal degeneration only be used for naming the disease after it has received verification through postmortem analysis of the neuropathology. CBS patients with greater temporoparietal degeneration are more
670:
In general, SPECT reveals hypoperfusion within both the posterior regions of the frontal and parietal lobes. As in images gathered through MRI, SPECT images indicated asymmetry in the presentation of abnormalities throughout the brain. Additional studies have revealed the presence of perfusion
646:
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. Specifically affected, are dopamine transporters which are presynaptic on the nigrostriatal cells. SPECT is used to
709:
neurons, whereas IBZM is a tracer that shows an affinity for the postsynaptic neurons of the same type. Despite agreement with other imaging studies, these two SPECT methods suffer some scrutiny due to better accuracy in other imaging methods. However, β-CIT SPECT has proven to be helpful in
200:
Alien hand syndrome has been shown to be prevalent in roughly 60% of those people diagnosed with CBD. This disorder involves the failure of an individual to control the movements of their hand, which results from the sensation that the limb is "foreign". The movements of the alien limb are a
806:. Additional distinguishing neurological features of those diagnosed with CBD consist of asymmetric atrophy of the frontal and parietal cortical regions of the brain. Postmortem studies of patients diagnosed with CBD indicate that histological attributes often involve ballooning of neurons,
797:
Corticobasal syndrome was first recognized in 1967 when Rebeiz, Kolodny, and
Richardson Jr described three people with a progressive asymmetric akinetic-rigid syndrome combined with apraxia, which they named corticodentatonigral degeneration with neuronal achromasia. The condition was "mostly
634:
Functional MRI (fMRI) has been used to evaluate the activation patterns in various regions of the brain of individuals affected with CBD. Upon the performance of simple finger motor tasks, subjects with CBD experienced lower levels of activity in the parietal cortex, sensorimotor cortex, and
776:
of a feeding tube may be necessary and will help prevent aspiration pneumonia, primary cause of death in CBD. Incontinence is common, as patients often can't express their need to go, due to eventual loss of speech. Therefore, proper hygiene is mandatory to prevent urinary tract infections.
118:
Although corticobasal degeneration has a plethora of symptoms, some are more prevalent than others. In a study of 147 patients with CBD, it was found that all of them had at least one
Parkinsonian sign, 95% having two and 93% had some higher order dysfunction (cognitive impairments like
788:
Progressive supranuclear palsy (PSP) without CBD is estimated to be ten times more common. CBD represents roughly 4–6% of patients with
Parkinsonism. Average age at disease onset is about 64, with the youngest confirmed onset being at age 43. There may be a slight female predominance.
763:
only moderate improvement, and the relief from the symptom is not long-lasting. In addition, palliative therapies, including the use of wheelchairs, speech therapy, and feeding techniques, are often used to alleviate many of the symptoms that are not improved by medication.
694:(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. These studies have concluded that, in general, dopamine uptake was diminished in the caudate and the
188:(PSP). Parkinsonism in CBD is largely present in an extremity such as the arm, and is always asymmetric. It has been suggested that non-dominant arm is involved more often. Common associated movement dysfunctions that comprise parkinsonism are rigidity,
179:
The presence of parkinsonism as a clinical symptom of CBD is largely responsible for complications in developing unique diagnostic criteria for the disease. Other such diseases in which parkinsonism forms an integral diagnostic characteristic are
550:
Postmortem diagnosis provides the only true indication of the presence of CBD. Most of these diagnoses utilize the
Gallyas-Braak staining method, which is effective in identifying the presence of astroglial inclusions and coincidental tauopathy.
1402:
Hattori M, Hashizume Y, Yoshida M, Iwasaki Y, Hishikawa N, et al. (2003). "Distribution of astrocytic plaques in the corticobasal degeneration brain and comparison with tuft-shaped astrocytes in the progressive supranuclear palsy brain".
223:
Aphasia in CBD is revealed through the inability to speak or a difficulty in initiating spoken dialogue and falls under the non-fluent (as opposed to fluent or flowing) subtype of the disorder. This may be related to speech impairment such as
698:. This characteristic also has the potential to be useful in distinguishing CBD from the similar PD, as individuals having been diagnosed with PD were more likely to have a lower uptake of dopamine than in individuals with CBD.
201:
reaction to external stimuli and do not occur sporadically or without stimulation. The presence of an alien limb has a distinct appearance in CBD, in which the diagnosed individual may have a "tactile mitgehen". This mitgehen
785:
that the prevalence of CBD is approximately 4.9 to 7.3 per 100,000 people. CBD research has been limited by the rarity of the disease and the lack of research criteria. It is estimated to affect 0.6-0.9 per 100,000 per year.
728:
All of the disorders and dysfunctions associated with CBD can often be categorized into a class of symptoms that present with the disease of CBD. These symptoms that aid in clinical diagnosis are collectively referred to as
2324:
647:
detect these abnormalities in
Dopamine transporters. Given that many patients have asymmetrical loss of function and metabolism this can help differentiate patients with CBD and those with Alzheimer's.
563:
Individuals diagnosed with PD often exhibit similar movement dysfunction as those diagnosed with CBD, which adds complexity to its diagnosis. Some other neurodegenerative diseases including
814:. Much of the pioneering advancements and research performed on CBD has been completed within the past decade or so, due to the relatively recent formal recognition of the disease.
308:
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.
627:
MRI of CBD typically shows posterior parietal and frontal cortical atrophy with unequal representation in corresponding sides. In addition, atrophy has been noted in the
2069:
Fredericks CA, Lee SE (2016). "The cognitive neurology of corticobasal degeneration and progressive supranuclear palsy". In Miller, Bruce L.; Boeve, Bradley F. (eds.).
871:
1495:"Predicting amyloid status in corticobasal syndrome using modified clinical criteria, magnetic resonance imaging and fluorodeoxyglucose positron emission tomography"
1240:"Diffusion-weighted brain imaging study of patients with clinical diagnosis of corticobasal degeneration, progressive supranuclear palsy and Parkinson's disease"
687:
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
127:, neglect, etc.). In a separate study of 14 patients recorded 3 years after the onset of symptoms, many patients had high numbers of motor symptoms. 71% had
1812:
202:
305:
New diagnostic criteria known as the
Armstrong criteria were proposed in 2013, although the accuracy of these is limited and further research is needed.
1094:
Belfor N, Amici S, Boxer AL, Kramer JH, Gorno-Tempini ML, et al. (2006). "Clinical and neuropsychological features of corticobasal degeneration".
74:. CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years. It is characterized by marked
1927:, Mathias CJ, Daniel SE (January 1995). "Morphological overlap between corticobasal degeneration and Pick's disease: a clinicopathological report".
864:
115:
dysfunctions, although present in CBD, are much less prevalent and lack establishment as common indicators of the presence of the disease.
1985:
Seritan AL, Mendez MF, Silverman DH, Hurley RA, Taber KH (2004). "Functional imaging as a window to dementia: Corticobasal degeneration".
597:
1279:
Zhu MW, Wang LN, Li XH, Gui QP (April 2004). "Glial abnormalities in progressive supranuclear palsy and corticobasal degeneration".
2148:
Boeve BF (2011). "The multiple phenotypes of corticobasal syndrome and corticobasal degeneration: Implications for further study".
1880:
Scaravilli T, Tolosa E, Ferrer I (2005). "Progressive supranuclear palsy and corticobasal degeneration: Lumping versus splitting".
1005:
Koyama M, Yagishita A, Nakata Y, helloHayashi M, Bandoh M, et al. (2007). "Imaging of corticobasal degeneration syndrome".
269:
Postmortem histological examination of the brains of individuals diagnosed with CBD reveal unique characteristics involving the
875:
2411:
1048:
Rana AQ, Ansari H, Siddiqui I (2012). "The relationship between arm dystonia in corticobasal degeneration and handedness".
872:"Corticobasalganglionic Degeneration Information Page: National Institute of Neurological Disorders and Stroke (NINDS)"
2078:
851:
751:
1314:
572:
1851:
614:
presence of such a neurodegenerative disease. FDOPA PET is used to study the efficacy of the dopamine pathway.
2401:
1344:"Tau-positive glial inclusions in progressive supranuclear palsy, corticobasal degeneration and Pick's disease"
746:
290:
185:
91:
273:
in localized regions. The typical procedure used in the identification of these astroglial inclusions is the
587:
The types of imaging techniques that are most prominently utilized when studying and/or diagnosing CBD are:
2406:
701:
Other clinical tests or procedures that monitor the presence of dopamine within the brain (β-CIT SPECT and
606:
679:, basal ganglia, and pontocerebellar (from the pons to the cerebellum) locations within subjects' brains.
591:
802:, specifically marked by neuronal degeneration or depigmentation (loss of melanin in a neuron) in the
568:
95:
63:
2339:
531:
The diagnostic criteria for clinical use may result in a misdiagnosis of other tau-based diseases.
711:
576:
242:
83:
2416:
734:
564:
181:
87:
465:
impaired grammar/sentence comprehension with relatively preserved single word comprehension or
2261:
1613:
Lang AE (2005). "Treatment of progressive supranuclear palsy and corticobasal degeneration".
839:
817:
The Office of Rare
Diseases in the United States created the first criteria in 2002, and the
730:
723:
86:. Diagnosis is difficult, as symptoms are often similar to those of other disorders, such as
1813:"Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study"
257:
abnormalities within the brain and improper accumulation of the protein tau (referred to as
1292:
31:
2073:(Second ed.). Cambridge, United Kingdom: Cambridge University Press. pp. 203–6.
8:
2328:
1764:"Validation of the new consensus criteria for the diagnosis of corticobasal degeneration"
664:
157:
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1905:
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1428:
1381:
1368:
1359:
1343:
1215:
1190:
1168:
1119:
1073:
1030:
980:
238:
75:
2333:
2241:
968:
916:
2350:
2290:
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2245:
2208:
2165:
2122:
2084:
2074:
2051:
2002:
1944:
1897:
1835:
1793:
1743:
1731:
1723:
1684:
1679:
1662:
1630:
1592:
1574:
1553:
Constantinides VC, Paraskevas GP, Paraskevas PG, Stefanis L, Kapaki E (August 2019).
1526:
1472:
1420:
1373:
1296:
1261:
1220:
1160:
1111:
1065:
1022:
972:
920:
738:
212:
99:
44:
2344:
2257:
2228:
Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP (2004). "Corticobasal degeneration".
2220:
2177:
1956:
1909:
1847:
1703:
1642:
1493:
Sha SJ, Ghosh PM, Lee SE, Corbetta-Rastelli C, Jagust WJ, et al. (March 2015).
1432:
1238:
Rizzo G, Martinelli P, Manners D, Scaglione C, Tonon C, et al. (October 2008).
1172:
1123:
1034:
984:
955:
Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP (2004). "Corticobasal degeneration".
2379:
2282:
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1994:
1936:
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1783:
1775:
1715:
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1622:
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1506:
1462:
1412:
1385:
1363:
1355:
1288:
1251:
1210:
1202:
1150:
1103:
1077:
1057:
1014:
964:
912:
803:
2187:"Structural and Functional Differences between 3-Repeat and 4-Repeat Tau Isoforms"
705:
SPECT) have shown similar findings. β-CIT serves as an indicator for presynaptic
1719:
1189:
Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, et al. (August 2011).
676:
628:
67:
2265:
1570:
2355:
2118:
1811:
Litvan I, Agid Y, Goetz C, Jankovic J, Wenning GK, et al. (January 1997).
1061:
660:
103:
2161:
1762:
Alexander SK, Rittman T, Xuereb JH, Bak TH, Hodges JR, Rowe JB (August 2014).
1511:
1416:
1107:
1018:
2395:
2088:
1924:
1779:
1727:
1578:
830:
799:
656:
635:
supplementary motor cortex than those individuals tested in a control group.
71:
1663:"[Diagnoses of corticobasal syndrome and corticobasal degeneration]"
2249:
2212:
2203:
2186:
2169:
2055:
2006:
1901:
1797:
1735:
1688:
1634:
1596:
1530:
1476:
1467:
1450:
1424:
1377:
1300:
1265:
1256:
1239:
1224:
1164:
1115:
1069:
1026:
976:
924:
706:
189:
152:
128:
2294:
2126:
1948:
1940:
1839:
1552:
1449:
Alladi S, Xuereb J, Bak T, Nestor P, Knibb J, et al. (October 2007).
1191:"Clinicopathological correlations in corticobasal ganglionic degeneration"
1998:
1831:
1137:
FitzGerald DB, Drago V, Jeong Y, Chang YL, White KD, et al. (2007).
903:
Wadia PM, Lang AE (2007). "The many faces of corticobasal degeneration".
835:
2316:
1704:"Corticobasal degeneration: advances in clinicopathology and biomarkers"
2037:
603:
493:
supranuclear vertical gaze palsy or decreased vertical saccade velocity
332:
PSPS plus at least one CBS feature other than limb rigidity or akinesia
270:
253:
Neuropathological findings associated with CBD include the presence of
225:
39:
Basal ganglia along with cerebral cortex are involved in this condition
1893:
1626:
1206:
1155:
1138:
2374:
2105:
Gibb WR, Luthert PJ, Marsden CD (1989). "Corticobasal degeneration".
811:
651:
258:
254:
120:
79:
691:
672:
136:
124:
472:
1004:
807:
742:
695:
168:
162:
132:
2093:
reminiscent of classic CBS but with executive function deficits
1555:"Corticobasal degeneration and corticobasal syndrome: A review"
1237:
437:
1401:
688:
643:
560:
the ring-shaped astrocytic plaques found as a result of CBD.
456:
142:
2227:
1984:
1922:
1492:
954:
702:
350:
More likely if no genetic mutation affecting T (e.g., MAPT)
2022:"The corticobasal syndrome-Alzheimer's disease conundrum"
1761:
1136:
518:
semantic or logopenic variant primary progressive aphasia
354:
650:
SPECT studies of individuals diagnosed with CBD involve
468:
groping, distorted speech production (apraxia of speech)
2184:
1810:
1188:
1139:"Asymmetrical alien hands in corticobasal degeneration"
1879:
1660:
1093:
838:
is an inspiring account of walking
England's 630 mile
524:
granulin mutation or reduced plasma progranulin levels
347:
More likely if no family history (2 or more relatives)
231:
2068:
2019:
1987:
Journal of Neuropsychiatry and Clinical Neurosciences
1548:
1546:
1544:
1542:
1540:
1451:"Focal cortical presentations of Alzheimer's disease"
1448:
2306:
842:
with her husband who has corticobasal degeneration.
733:(CBS) or corticobasal degeneration syndrome (CBDS).
2020:Hassan A, Whitwell JL, Josephs KA (November 2011).
821:clinical diagnostic criteria were created in 2013.
461:Effortful, agrammatic speech plus at least one of:
2104:
1537:
1047:
433:alien limb phenomena (more than simple levitation)
381:alien limb phenomena (more than simple levitation)
2185:Goode BL, Chau M, Denis PE, Feinstein SC (2000).
2393:
2139:Raynor Winn (2018) The Salt Path, Penguin Books.
1757:
1755:
1753:
1656:
1654:
1652:
1315:"CBD Identification - Chemistry and Toxicology"
473:Progressive supranuclear palsy syndrome subtype
554:
1875:
1873:
1871:
1750:
1649:
1559:Clinical Parkinsonism & Related Disorders
1488:
1486:
538:criteria are proposed for clinical research.
1701:
1444:
1442:
1278:
481:axial or symmetric limb rigidity or akinesia
438:Frontal behavioural-spatial syndrome subtype
165:(ideomotor apraxia and limb-kinetic apraxia)
1231:
950:
948:
946:
944:
942:
940:
938:
936:
934:
521:structural lesion suggestive of focal cause
403:No genetic mutation affecting T (e.g. MAPT)
1980:
1978:
1976:
1974:
1972:
1970:
1968:
1966:
1868:
1483:
1397:
1395:
750:likely to have AD pathology as opposed to
598:single-photon emission computed tomography
541:
527:TDP-43 or fused in sarcoma (FUS) mutations
457:NAV of primary progressive aphasia subtype
143:Motor and associated cortical dysfunctions
30:
2273:Avila J (1992). "Microtubule functions".
2202:
2045:
1787:
1678:
1661:Shimohata T, Aiba I, Nishizawa M (2016).
1586:
1520:
1510:
1466:
1439:
1367:
1337:
1335:
1255:
1214:
1184:
1182:
1154:
1130:
902:
359:Symptoms may be symmetric or asymmetric.
2147:
1341:
1089:
1087:
1000:
998:
996:
994:
931:
717:
579:(FTD) also show commonalities with CBD.
264:
2272:
1963:
1612:
1608:
1606:
1392:
898:
896:
894:
892:
400:No family history (2 or more relatives)
338:
317:Insidious onset and gradual progression
2394:
1332:
1179:
824:
355:Possible corticobasal syndrome subtype
195:
1084:
991:
497:
248:
109:
2071:The Behavioral Neurology of Dementia
1603:
1293:10.3760/j.issn:0529-5807.2004.02.008
1096:Mechanisms of Ageing and Development
905:Parkinsonism & Related Disorders
889:
344:More likely if onset is after age 50
1041:
232:Psychiatric and cognitive disorders
13:
2062:
2026:Expert Review of Neurotherapeutics
1499:Alzheimer's Research & Therapy
1360:10.1111/j.1750-3639.1999.tb00549.x
449:behavioural or personality changes
241:such as Alzheimer's disease (AD).
82:, and is classified as one of the
14:
2428:
2302:
2150:Journal of Molecular Neuroscience
1702:Svenningsson, Per (August 2019).
852:Frontotemporal lobar degeneration
752:frontotemporal lobar degeneration
618:clinically present dysfunctions.
502:These apply to all types of CBD.
1768:J. Neurol. Neurosurg. Psychiatry
1680:10.5692/clinicalneurol.cn-000841
1050:Journal of Clinical Neuroscience
710:distinguishing CBD from PSP and
573:chronic traumatic encephalopathy
323:Meets one of the four subtypes:
2191:Journal of Biological Chemistry
2133:
2098:
2013:
1916:
1804:
1695:
1307:
1272:
779:
655:overperfusion, underperfusion,
582:
237:misdiagnosis of CBD as another
174:
747:progressive supranuclear palsy
291:microtubule-associated protein
186:progressive supranuclear palsy
92:progressive supranuclear palsy
16:Rare neurodegenerative disease
1:
2242:10.1016/s1474-4422(04)00936-6
969:10.1016/s1474-4422(04)00936-6
917:10.1016/s1353-8020(08)70027-0
857:
506:Evidence of Lewy body disease
484:postural instability or falls
275:Gallyas-Braak staining method
131:(slow movements), 64% showed
102:of CBD can only be made upon
2287:10.1016/0024-3205(92)90433-p
1720:10.1097/WCO.0000000000000707
1708:Current Opinion in Neurology
770:
757:
682:
607:positron emission tomography
390:(probable sporadic CBS) if:
372:limb myoclonus, plus one of:
300:
280:
7:
2412:Neurodegenerative disorders
1571:10.1016/j.prdoa.2019.08.005
1281:Zhonghua Bing Li Xue Za Zhi
845:
555:Overlap with other diseases
311:
10:
2433:
1062:10.1016/j.jocn.2011.10.012
792:
721:
592:magnetic resonance imaging
427:orobuccal or limb apraxia,
218:
207:
2365:
2310:
2162:10.1007/s12031-011-9624-1
1512:10.1186/s13195-014-0093-y
1417:10.1007/s00401-003-0711-4
1108:10.1016/j.mad.2005.09.013
1019:10.1007/s00234-007-0265-6
569:dementia with Lewy bodies
411:limb rigidity or akinesia
375:orobuccal or limb apraxia
366:limb rigidity or akinesia
245:can be an early feature.
96:dementia with Lewy bodies
64:neurodegenerative disease
56:Corticobasal degeneration
43:
38:
29:
25:Corticobasal degeneration
24:
2119:10.1093/brain/112.5.1171
1780:10.1136/jnnp-2013-307035
638:
430:cortical sensory deficit
378:cortical sensory deficit
84:Parkinson plus syndromes
712:multiple system atrophy
577:frontotemporal dementia
542:Clinical vs. postmortem
509:multiple system atrophy
394:Asymmetric presentation
243:Frontotemporal dementia
2204:10.1074/jbc.m007489200
621:
2402:Corticobasal syndrome
1941:10.1002/mds.870100118
1405:Acta Neuropathologica
840:South West Coast Path
731:corticobasal syndrome
724:Corticobasal syndrome
718:Corticobasal syndrome
452:visuospatial deficits
446:executive dysfunction
265:Astroglial inclusions
76:disorders in movement
2230:The Lancet Neurology
1999:10.1176/jnp.16.4.393
1832:10.1212/wnl.48.1.119
1468:10.1093/brain/awm213
1257:10.1093/brain/awn195
487:urinary incontinence
320:Lasts 1 year or more
135:, 43% reported limb
2407:Cognitive disorders
2197:(49): 38182–38189.
1250:(Pt 10): 2690–700.
1195:Annals of Neurology
825:Society and culture
735:Alzheimer's disease
665:cerebral blood flow
565:Alzheimer's disease
512:Alzheimer's disease
490:behavioural changes
196:Alien hand syndrome
182:Parkinson's disease
158:Alien hand syndrome
98:, and a definitive
88:Parkinson's disease
2366:External resources
2038:10.1586/ern.11.153
1929:Movement Disorders
1882:Movement Disorders
1667:Rinsho Shinkeigaku
1615:Movement Disorders
1143:Movement Disorders
498:Exclusion criteria
397:Onset after age 50
339:Exclusion criteria
249:Molecular features
239:cognitive disorder
110:Signs and symptoms
2389:
2388:
1894:10.1002/mds.20536
1627:10.1002/mds.20545
1342:Komori T (1999).
1207:10.1002/ana.22424
1156:10.1002/mds.21337
671:anomalies in the
213:Ideomotor apraxia
53:
52:
19:Medical condition
2424:
2308:
2307:
2298:
2269:
2224:
2206:
2181:
2140:
2137:
2131:
2130:
2113:(5): 1171–1192.
2102:
2096:
2095:
2066:
2060:
2059:
2049:
2017:
2011:
2010:
1982:
1961:
1960:
1920:
1914:
1913:
1877:
1866:
1865:
1863:
1862:
1856:
1850:. Archived from
1817:
1808:
1802:
1801:
1791:
1759:
1748:
1747:
1699:
1693:
1692:
1682:
1658:
1647:
1646:
1610:
1601:
1600:
1590:
1550:
1535:
1534:
1524:
1514:
1490:
1481:
1480:
1470:
1446:
1437:
1436:
1399:
1390:
1389:
1371:
1339:
1330:
1329:
1327:
1326:
1311:
1305:
1304:
1276:
1270:
1269:
1259:
1235:
1229:
1228:
1218:
1186:
1177:
1176:
1158:
1134:
1128:
1127:
1091:
1082:
1081:
1045:
1039:
1038:
1002:
989:
988:
957:Lancet Neurology
952:
929:
928:
900:
887:
886:
884:
883:
874:. Archived from
868:
804:substantia nigra
362:One or more of:
289:is an important
123:, sensory loss,
34:
22:
21:
2432:
2431:
2427:
2426:
2425:
2423:
2422:
2421:
2392:
2391:
2390:
2385:
2384:
2361:
2360:
2319:
2305:
2144:
2143:
2138:
2134:
2103:
2099:
2081:
2067:
2063:
2032:(11): 1569–78.
2018:
2014:
1983:
1964:
1921:
1917:
1878:
1869:
1860:
1858:
1854:
1815:
1809:
1805:
1760:
1751:
1700:
1696:
1669:(in Japanese).
1659:
1650:
1611:
1604:
1551:
1538:
1491:
1484:
1461:(10): 2636–45.
1447:
1440:
1400:
1393:
1348:Brain Pathology
1340:
1333:
1324:
1322:
1313:
1312:
1308:
1277:
1273:
1236:
1232:
1187:
1180:
1135:
1131:
1092:
1085:
1046:
1042:
1003:
992:
953:
932:
901:
890:
881:
879:
870:
869:
865:
860:
848:
827:
795:
782:
773:
760:
726:
720:
685:
677:temporal cortex
641:
629:corpus callosum
624:
585:
557:
544:
500:
475:
459:
440:
357:
314:
303:
283:
267:
251:
234:
221:
210:
198:
177:
145:
112:
104:neuropathologic
68:cerebral cortex
20:
17:
12:
11:
5:
2430:
2420:
2419:
2414:
2409:
2404:
2387:
2386:
2383:
2382:
2370:
2369:
2367:
2363:
2362:
2359:
2358:
2347:
2336:
2320:
2315:
2314:
2312:
2311:Classification
2304:
2303:External links
2301:
2300:
2299:
2281:(5): 327–334.
2270:
2236:(12): 736–43.
2225:
2182:
2142:
2141:
2132:
2097:
2079:
2061:
2012:
1962:
1915:
1867:
1803:
1749:
1714:(4): 597–603.
1694:
1648:
1602:
1536:
1482:
1438:
1391:
1331:
1319:The Drug Store
1306:
1271:
1230:
1201:(2): 327–340.
1178:
1129:
1083:
1040:
1013:(11): 905–12.
1007:Neuroradiology
990:
963:(12): 736–43.
930:
888:
862:
861:
859:
856:
855:
854:
847:
844:
826:
823:
794:
791:
781:
778:
772:
769:
759:
756:
739:Pick's disease
722:Main article:
719:
716:
684:
681:
661:hyperperfusion
640:
637:
623:
620:
611:
610:
601:
595:
584:
581:
556:
553:
543:
540:
529:
528:
525:
522:
519:
516:
513:
510:
507:
499:
496:
495:
494:
491:
488:
485:
482:
474:
471:
470:
469:
466:
458:
455:
454:
453:
450:
447:
439:
436:
435:
434:
431:
428:
424:
423:
419:
418:
417:limb myoclonus
415:
412:
408:
407:
404:
401:
398:
395:
383:
382:
379:
376:
373:
370:
367:
356:
353:
352:
351:
348:
345:
342:
335:
334:
333:
330:
327:
321:
318:
313:
310:
302:
299:
282:
279:
266:
263:
250:
247:
233:
230:
220:
217:
209:
206:
197:
194:
176:
173:
172:
171:
166:
160:
155:
144:
141:
111:
108:
66:involving the
51:
50:
47:
41:
40:
36:
35:
27:
26:
18:
15:
9:
6:
4:
3:
2:
2429:
2418:
2417:Rare diseases
2415:
2413:
2410:
2408:
2405:
2403:
2400:
2399:
2397:
2381:
2377:
2376:
2372:
2371:
2368:
2364:
2357:
2353:
2352:
2348:
2346:
2342:
2341:
2337:
2335:
2331:
2330:
2326:
2322:
2321:
2318:
2313:
2309:
2296:
2292:
2288:
2284:
2280:
2276:
2271:
2267:
2263:
2259:
2255:
2251:
2247:
2243:
2239:
2235:
2231:
2226:
2222:
2218:
2214:
2210:
2205:
2200:
2196:
2192:
2188:
2183:
2179:
2175:
2171:
2167:
2163:
2159:
2155:
2151:
2146:
2145:
2136:
2128:
2124:
2120:
2116:
2112:
2108:
2101:
2094:
2090:
2086:
2082:
2080:9781107077201
2076:
2072:
2065:
2057:
2053:
2048:
2043:
2039:
2035:
2031:
2027:
2023:
2016:
2008:
2004:
2000:
1996:
1992:
1988:
1981:
1979:
1977:
1975:
1973:
1971:
1969:
1967:
1958:
1954:
1950:
1946:
1942:
1938:
1934:
1930:
1926:
1923:Jendroska K,
1919:
1911:
1907:
1903:
1899:
1895:
1891:
1887:
1883:
1876:
1874:
1872:
1857:on 2011-07-16
1853:
1849:
1845:
1841:
1837:
1833:
1829:
1826:(1): 119–25.
1825:
1821:
1814:
1807:
1799:
1795:
1790:
1785:
1781:
1777:
1774:(8): 925–29.
1773:
1769:
1765:
1758:
1756:
1754:
1745:
1741:
1737:
1733:
1729:
1725:
1721:
1717:
1713:
1709:
1705:
1698:
1690:
1686:
1681:
1676:
1673:(3): 149–57.
1672:
1668:
1664:
1657:
1655:
1653:
1644:
1640:
1636:
1632:
1628:
1624:
1620:
1616:
1609:
1607:
1598:
1594:
1589:
1584:
1580:
1576:
1572:
1568:
1564:
1560:
1556:
1549:
1547:
1545:
1543:
1541:
1532:
1528:
1523:
1518:
1513:
1508:
1504:
1500:
1496:
1489:
1487:
1478:
1474:
1469:
1464:
1460:
1456:
1452:
1445:
1443:
1434:
1430:
1426:
1422:
1418:
1414:
1410:
1406:
1398:
1396:
1387:
1383:
1379:
1375:
1370:
1365:
1361:
1357:
1354:(4): 663–79.
1353:
1349:
1345:
1338:
1336:
1320:
1316:
1310:
1302:
1298:
1294:
1290:
1286:
1282:
1275:
1267:
1263:
1258:
1253:
1249:
1245:
1241:
1234:
1226:
1222:
1217:
1212:
1208:
1204:
1200:
1196:
1192:
1185:
1183:
1174:
1170:
1166:
1162:
1157:
1152:
1148:
1144:
1140:
1133:
1125:
1121:
1117:
1113:
1109:
1105:
1101:
1097:
1090:
1088:
1079:
1075:
1071:
1067:
1063:
1059:
1056:(8): 1134–6.
1055:
1051:
1044:
1036:
1032:
1028:
1024:
1020:
1016:
1012:
1008:
1001:
999:
997:
995:
986:
982:
978:
974:
970:
966:
962:
958:
951:
949:
947:
945:
943:
941:
939:
937:
935:
926:
922:
918:
914:
910:
906:
899:
897:
895:
893:
878:on 2009-03-23
877:
873:
867:
863:
853:
850:
849:
843:
841:
837:
833:
832:
831:The Salt Path
822:
820:
815:
813:
809:
805:
801:
800:basal ganglia
790:
786:
777:
768:
764:
755:
753:
748:
744:
740:
736:
732:
725:
715:
713:
708:
704:
699:
697:
693:
690:
680:
678:
674:
668:
666:
662:
658:
657:hypoperfusion
653:
648:
645:
636:
632:
630:
619:
615:
608:
605:
602:
599:
596:
593:
590:
589:
588:
580:
578:
574:
570:
566:
561:
552:
548:
539:
537:
532:
526:
523:
520:
517:
514:
511:
508:
505:
504:
503:
492:
489:
486:
483:
480:
479:
478:
467:
464:
463:
462:
451:
448:
445:
444:
443:
432:
429:
426:
425:
421:
420:
416:
414:limb dystonia
413:
410:
409:
405:
402:
399:
396:
393:
392:
391:
389:
388:
380:
377:
374:
371:
369:limb dystonia
368:
365:
364:
363:
360:
349:
346:
343:
340:
336:
331:
328:
325:
324:
322:
319:
316:
315:
309:
306:
298:
294:
292:
288:
278:
276:
272:
262:
260:
256:
246:
244:
240:
229:
227:
216:
214:
205:
203:
193:
191:
187:
183:
170:
167:
164:
161:
159:
156:
154:
151:
150:
149:
140:
138:
134:
130:
126:
122:
116:
107:
106:examination.
105:
101:
97:
93:
89:
85:
81:
77:
73:
72:basal ganglia
69:
65:
62:) is a rare
61:
57:
48:
46:
42:
37:
33:
28:
23:
2373:
2349:
2338:
2323:
2278:
2274:
2233:
2229:
2194:
2190:
2156:(3): 350–3.
2153:
2149:
2135:
2110:
2106:
2100:
2092:
2070:
2064:
2029:
2025:
2015:
1993:(4): 393–9.
1990:
1986:
1935:(1): 111–4.
1932:
1928:
1918:
1885:
1881:
1859:. Retrieved
1852:the original
1823:
1819:
1806:
1771:
1767:
1711:
1707:
1697:
1670:
1666:
1618:
1614:
1562:
1558:
1502:
1498:
1458:
1454:
1411:(2): 143–9.
1408:
1404:
1351:
1347:
1323:. Retrieved
1321:. 2020-08-26
1318:
1309:
1287:(2): 125–9.
1284:
1280:
1274:
1247:
1243:
1233:
1198:
1194:
1149:(4): 581–4.
1146:
1142:
1132:
1102:(2): 203–7.
1099:
1095:
1053:
1049:
1043:
1010:
1006:
960:
956:
911:: S336–S40.
908:
904:
880:. Retrieved
876:the original
866:
829:
828:
818:
816:
796:
787:
783:
780:Epidemiology
774:
765:
761:
727:
707:dopaminergic
700:
692:PET scanning
686:
669:
649:
642:
633:
625:
616:
612:
586:
583:Neuroimaging
562:
558:
549:
545:
535:
533:
530:
501:
476:
460:
441:
422:plus two of:
406:plus two of:
386:
385:
384:
361:
358:
326:Possible CBS
307:
304:
295:
286:
285:The protein
284:
274:
268:
252:
235:
222:
211:
199:
190:bradykinesia
178:
175:Parkinsonism
153:Parkinsonism
146:
129:bradykinesia
117:
113:
59:
55:
54:
1621:: S83–S91.
836:Raynor Winn
609:(FDOPA PET)
387:More likely
2396:Categories
2351:DiseasesDB
2345:D000088282
1888:: S21–S8.
1861:2010-11-21
1325:2020-10-17
882:2009-03-20
858:References
604:fluorodopa
575:(CTE) and
477:Three of:
329:FBS or NAV
271:astrocytes
255:astrocytic
226:dysarthria
2375:eMedicine
2266:201513368
2089:934020279
1925:Rossor MN
1820:Neurology
1744:169039152
1728:1350-7540
1579:2590-1125
1565:: 66–71.
819:Armstrong
812:tauopathy
771:Prognosis
758:Treatment
683:FDOPA PET
652:perfusion
301:Diagnosis
281:Tauopathy
259:tauopathy
184:(PD) and
121:acalculia
100:diagnosis
80:cognition
49:Neurology
45:Specialty
2380:neuro/77
2275:Life Sci
2262:ProQuest
2258:15324889
2250:15556806
2221:30896096
2213:10984497
2178:30307394
2170:21853287
2056:22014136
2007:15616165
1957:43288223
1910:21358152
1902:16092076
1848:30401542
1798:24521567
1736:31145128
1689:26876110
1643:45848876
1635:16092096
1597:34316603
1531:25733984
1505:(1): 8.
1477:17898010
1433:25741692
1425:12732936
1378:10517506
1301:15132848
1266:18819991
1225:21823158
1173:41998716
1165:17230447
1124:35169781
1116:16310834
1070:22705141
1035:35778587
1027:17632713
985:15324889
977:15556806
925:18267261
846:See also
673:thalamus
667:(rCBF).
536:probable
442:Two of:
312:Criteria
137:dystonia
125:dementia
70:and the
2295:1732704
2127:2478251
2047:3232678
1949:7885345
1840:9008506
1789:4112495
1588:8288513
1522:4346122
1386:3142586
1369:8098509
1216:3154081
1078:2233432
808:gliosis
793:History
743:FTDP-17
714:(MSA).
696:putamen
600:(SPECT)
571:(DLB),
341:present
219:Aphasia
208:Apraxia
169:Aphasia
163:Apraxia
133:apraxia
2293:
2264:
2256:
2248:
2219:
2211:
2176:
2168:
2125:
2087:
2077:
2054:
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1074:S2CID
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