Pathological heterogeneity of clinically diagnosed corticobasal degeneration

Doran, Mark; Plessis, Daniel G. du; Enevoldson, T P; Fletcher, Nicholas; Ghadiali, E; Larner, A. J.

doi:10.1016/s0022-510x(03)00232-6

Cited by 69 publications

(34 citation statements)

References 41 publications

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“…One example would be the patient with pathologically proven PSP, who at the time of our first assessment did not fulfil either the NINDS (lack of falls within the first year) or the NNIPPS (lack of supranuclear gaze palsy) criteria. This confirms that such criteria have a relatively low sensitivity, especially early in the disease course [2,[27][28][29]. In our series, this might have been the case in up to 19%.…”

Section: Discussionsupporting

confidence: 86%

“Atypical” atypical parkinsonism: Critical appraisal of a cohort

Hirschbichler

Erro

Ganos

et al. 2017

Parkinsonism & Related Disorders

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Background: Atypical parkinsonian conditions such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal syndrome (CBS) and Dementia with Lewy bodies (DLB) comprise 10-15% of parkinsonian syndromes. Misdiagnosis with Parkinson disease (PD) and within the entities is common, given the absence of reliable biomarkers.However a correct diagnosis is not only important in clinical practice, but also crucial for any trial attempting to identify biomarkers or new treatments. Methods:Consecutive patients, who were either referred with a diagnosis of a particular AP by a neurologist, or where a movement disorder specialist at Queen Square considered such a diagnosis, were included and the medical records were reviewed retrospectively. We applied each set of current diagnostic research criteria to the respective cohort to see which features fit in and if there are atypical features "outside" the classic definition. Results:Sixty-nine patients were recruited clinically presenting with one of the following phenotypes: 14 MSA, 24 PSP, 19 CBS and 12 DLB. Up to 49% showed additional "atypical"features and approximately 10% eventually received an alternative diagnosis, in half of whom this was based on genetic testing. Conclusions:In a subset of our patients, despite the final diagnosis of an AP being maintained, there were additional "atypical" features. It remains to be seen if these reflect the clinical heterogeneity of APs, or should prompt a search for an alternative diagnosis. A change in terminology using phenotypic descriptors (e.g. MSA syndrome) rather than aetiological labels, as already applied for CBS, could therefore be a consideration for all the APs.

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Section: Discussionsupporting

confidence: 86%

“Atypical” atypical parkinsonism: Critical appraisal of a cohort

Hirschbichler

Erro

Ganos

et al. 2017

Parkinsonism & Related Disorders

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“…[18][19][20][21] A variety of underlying neuropathologic features have also been reported. Recent reports clarified the presence of many phenotypes of CBD neuropathologically and clinically.…”

Section: Discussionmentioning

confidence: 99%

Imaging-Pathologic Correlation in Corticobasal Degeneration

Tokumaru¹,

Saito²,

Murayama³

et al. 2009

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE:The clinical diagnosis of corticobasal degeneration (CBD) is often difficult due to varied clinical manifestations. In 4 patients with neuropathologically confirmed CBD, characteristic imaging findings and correlations with neuropathologic features were evaluated. Furthermore, imaging findings in CBD were compared with neuropathologically confirmed progressive supranuclear palsy (PSP) for a differential diagnosis.

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“…6,7 In 2003, Boeve et al 8 and Doran et al 7 independently suggested the terminology of 'corticobasal syndrome' or 'CBS' be used for suspected cases of CBD but without pathological confirmation, in order to emphasise the pathological heterogeneity of this syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…AD as one of the pathological substrates of CBS is well-described, 6,7,9,10 even with signs thought typical of CBD such as the alien limb phenomenon. 9 In one recent pathological series of 21 cases with ante-mortem clinical diagnosis of CBD, only five patients had CBD pathology and another five had AD.…”

Section: Discussionmentioning

confidence: 99%

CSF biomarkers and the diagnosis of variant forms of Alzheimer's disease

Wojtowicz¹,

Schott²,

Larner³

2017

Prog. Neurol. Psychiatry

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The differential diagnosis of progressive neurodegenerative disorders may sometimes be challenging, despite longitudinal assessment and access to traditional modalities of structural and functional neuroimaging. In this article, the authors describe a patient tentatively diagnosed with corticobasal syndrome in whom investigation with cerebrospinal fluid biomarkers led to diagnostic revision and new therapeutic options.

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Pathological heterogeneity of clinically diagnosed corticobasal degeneration

Cited by 69 publications

References 41 publications

“Atypical” atypical parkinsonism: Critical appraisal of a cohort

“Atypical” atypical parkinsonism: Critical appraisal of a cohort

Imaging-Pathologic Correlation in Corticobasal Degeneration

CSF biomarkers and the diagnosis of variant forms of Alzheimer's disease

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