Cognitive and motor assessment in autopsy-proven corticobasal degeneration

Murray, Rachael Z.; Neumann, Manuela; Forman, Mark S.; Farmer, Jennifer M.; Massimo, Lauren; Rice, Aaron N.; Miller, Bruce L.; Johnson, Julene K.; Clark, Chris; Hurtig, Howard I.; Gorno‐Tempini, Maria Luisa; Lee, V. M.-Y.; Trojanowski, John Q.; Grossman, Murray

doi:10.1212/01.wnl.0000259519.78480.c3

Cited by 206 publications

(246 citation statements)

References 54 publications

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“…3 Despite various clinical diagnostic criteria (table e-1 on the Neurology ® Web site at www.neurology.org), 4-10 the pathology of CBD is predicted antemortem in only 25% to 56% of cases. [11][12][13][14][15][16][17] Additionally, while these clinical criteria continue to be widely applied and cited, they reflect CBS alone and not the more recently recognized behavioral presentations of CBD.…”

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confidence: 99%

Criteria for the diagnosis of corticobasal degeneration

et al. 2013

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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). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. 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. When first described, "corticodentatonigral degeneration with neuronal achromasia" was considered a distinct clinicopathologic entity, 1 eventually termed corticobasal degeneration (CBD). 2Clinicopathologic studies have since revealed that the originally described clinical features of CBD, now called corticobasal syndrome (CBS), are often due to other pathologies. As a pathologic diagnosis, CBD is characterized by widespread deposition of hyperphosphorylated 4-repeat tau in neurons and glia, the latter as astrocytic plaques, in specific topographic areas. 3 Despite various clinical diagnostic criteria (table e-1 on the Neurology ® Web site at www.neurology.org), 4-10 the pathology of CBD is predicted antemortem in only 25% to 56% of cases.11-17 Additionally, while these clinical criteria continue to be widely applied and cited, they reflect CBS alone and not the more recently recognized behavioral presentations of CBD.

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confidence: 99%

Criteria for the diagnosis of corticobasal degeneration

et al. 2013

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“…Other criteria that exclude a CBD diagnosis are the autonomic disturbances, the emergence of early vertical gaze palsy and the presence of structural damage on imaging studies [7]. It is a matter of conflict whether early dementia belongs to the exclusion criteria, as occasionally it appears as the prominent symptom of the disease [8,12,13,29]. The main features of cognitive decline include apraxia and especially ideomotor apraxia and nonfluent aphasia.…”

Section: Discussionmentioning

confidence: 99%

“…As they exhibit vertical supranuclear gaze palsy, early falls and symmetrical bradykinesia, this subgroup has been also described as having CBD-Richardson's syndrome. Other clinical manifestations of the syndrome that often lead to misdiagnosis are the asymmetrical tremulous parkinsonism with early postural instability, early amnestic signs and progressive non-fluent aphasia with behavioral or cognitive variant of frontotemporal degeneration [1,[10][11][12][13][14]. The sensitivity in predicting CBD remains low and varies from 26, 3% to 56% in different studies [1,[12][13][14].…”

Section: Discussionmentioning

confidence: 99%

“…In CBD dementia, neuropsychological deficits are reflected by functional criteria and not the white matter lesions of MRI as long as atrophy has not occurred [26][27][28]. However, post-mortem studies have demonstrated that frontal lobe involvement is characteristic of CBD, as frequently frontal symptoms such as behavioral and cognitive disorders predominate at the disease initial stages [12,13,29].…”

Section: Discussionmentioning

confidence: 99%

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Could a structural damage mimic a Parkinson plus syndrome?

Alexoudi

Zalonis²,

Stathis³

2013

Open Medicine

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AbstractCorticobasal syndrome (CBS) is a progressive disease that includes a heterogeneous neuropathological spectrum. In the majority of cases, corticobasal degeneration (CBD) is the underlying disease. Clinical heterogeneity is reflected in the clinical presentation of the syndrome and consequently misdiagnosis is common. Our case is of interest because the symptoms, the poor response to L-dopa and the I123 Ioflupane CIT SPECT (DAT-SCAN) results were typical for CBD. However, the magnetic resonance imaging appearance suggested the possibility of a disease of vascular etiology. A 63-year-old woman presented in our outpatient clinic with symptoms of progressive clumsiness in the right arm when performing movements demanding fine motor skills, and mild speech difficulties. The brain magnetic resonance imaging (MRI) report described ischemic lesions in the left subcortical parietal areas, in the centrum semiovale, in periventricular white matter, cingulated gyrus bilaterally and moderate ventricle dilatation. A comprehensive neuropsychological study suggested mixed cortical and subcortical involvement. A DAT-SCAN examination showed a diminished dopamine transporter uptake in the left lenticular nucleus, suggesting parkinsonisn. For this patient, CBS-CBD was the most probable diagnosis, whereas vascular CBS was not excluded. This case report illustrates the low diagnostic sensitivity in predicting CBD and the overlap with other related neurodegenerative diseases. Finally, it demonstrates that in CBS, different underlying pathologies can be found at the same individual.

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“…32 Motor abnormalities may be absent in as many as approximately 50% of patients at the time of presentation with cognitive deficits, although during the course of the disease the majority of patients will show motor features. Approximately 30% of patients with CBS will develop alien limb syndrome, in which one arm may levitate or assume involuntary postures or movements, and 25% will have cortical sensory deficits.…”

Section: Neuroimagingmentioning

confidence: 99%