Neuropsychiatric Features in 36 Pathologically Confirmed Cases of Corticobasal Degeneration

Geda, Y. E.; Boeve, B. F.; Negash, Selam; Graff-Radford, Neil; Knopman, D. S.; Parisi, J. E.; Dickson, Dennis W.; Petersen, R. C.

doi:10.1176/appi.neuropsych.19.1.77

Cited by 21 publications

(31 citation statements)

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“…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%

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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Section: Discussionmentioning

confidence: 99%

Could a structural damage mimic a Parkinson plus syndrome?

Alexoudi

Zalonis²,

Stathis³

2013

Open Medicine

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“…Thus, understanding the distribution of tau pathology would likely help to explain the presence of neuropsychiatric signs and symptoms in CBS and PSPS. To date, in retrospective studies of autopsy-proven tauopathies, the clinical diagnosis of CBS -including neuropsychiatric symptoms -has accurately predicted no more than 50% of true CBD cases (Dickson et al, 2010;Geda et al, 2007;Wenning et al, 1998). Improving diagnostic accuracy is critical.…”

Section: Neuroanatomical Substrates For Corticobasal Syndrome and Promentioning

confidence: 99%

“…Another group performed a retrospective study of 36 patents with pathologically confi rmed CBD (Geda et al, 2007). Eight of these patients (22%) reported neuropsychiatric symptoms.…”

Section: Neuropsychiatric Features Of Corticobasal Syndromementioning

confidence: 99%

“…The purpose of this review is to demonstrate some of the neuropsychiatric challenges to accurately diagnose CBS and PSPS to refl ect the underlying pathology of CBD and PSP, respectively. The diagnostic dilemma is evident in studies of pathologically confi rmed CBD and PSP cases which retrospectively were misdiagnosed clinically (Geda et al, 2007;Litvan et al, 1999).…”

Section: Introductionmentioning

confidence: 99%

“…Thus, research efforts have now begun to also focus on the medical, psychological, and fi nancial stressors burdening caregivers of dementia patients (Nunnemann et al, 2012). Despite expert clinical prowess, multidisciplinary consensus diagnostic approaches, and ' classic ' presenting symptoms, antemortem clinical diagnoses do not always match pathologically confi rmed diagnoses (Geda et al, 2007;Litvan et al, 1999). Diagnostic criteria for research purposes and clinical management are being updated as rapidly as possible (Snowden et al, 2011;Sorbi et al, 2012).…”

Section: Introductionmentioning

confidence: 99%

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Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy

Bruns

Josephs

2013

International Review of Psychiatry

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Corticobasal syndrome (CBS) and progressive supranuclear palsy syndrome (PSPS) are two of the atypical Parkinsonism syndromes, in that patients exhibit rigidity, occasional tremor and postural instability, but do not symptomatically respond to dopamine replacement. CBS and PSPS can often present with complex cognitive difficulties and neuropsychiatric disturbances. Symptoms of depression, apathy, or agitation can be subtle and are often overlooked as reactions to learning a new diagnosis of Parkinsonism. These symptoms may be the earliest presenting evidence of CBS or PSPS, and these syndromes can be misdiagnosed with a primary psychiatric disorder rather than a neurodegenerative condition. Patients may be inappropriately treated with antipsychotic medications that exacerbate the extra-pyramidal motor features of the syndromes. When symptoms are considered to comprise a neurodegenerative syndrome, it may be an inaccurate diagnosis as many features of CBS and PSPS not only overlap with each other, but also with other dementia syndromes. This review discusses similarities and differences between the syndromes of CBS and PSPS in terms of neuropsychiatric features. Improved characterization of the clinical syndromes is necessary to better predict underlying pathology. Improved education about these diseases would help patients, caregivers and clinicians to anticipate symptom progression and avoid premature nursing home placement.

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Clinicopathological correlations in corticobasal degeneration

Lee

Rabinovici

Mayo

et al. 2011

Annals of Neurology

381

380

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Objective To characterize cognitive and behavioral features, physical findings and brain atrophy patterns in pathology-proven corticobasal degeneration (CBD) and corticobasal syndrome (CBS) with known histopathology. Methods We reviewed clinical and MRI data in all patients evaluated at our center with either an autopsy diagnosis of CBD (n=18) or clinical CBS at first presentation with known histopathology (n=40). Atrophy patterns were compared using voxel-based morphometry. Results CBD was associated with four clinical syndromes: progressive nonfluent aphasia (5), behavioral variant frontotemporal dementia (5), executive-motor (7), and posterior cortical atrophy (1). Behavioral or cognitive problems were the initial symptoms in 15/18 patients; less than half exhibited early motor findings. Compared to controls, CBD patients showed atrophy in dorsal prefrontal and peri-rolandic cortex, striatum and brainstem (p<0.001 uncorrected). The most common pathologic substrates for clinical CBS were CBD (35%), Alzheimer’s disease (AD, 23%), progressive supranuclear palsy (13%), and frontotemporal lobar degeneration (FTLD) with TDP inclusions (13%). CBS was associated with perirolandic atrophy irrespective of underlying pathology. In CBS due to FTLD (tau or TDP), atrophy extended into prefrontal cortex, striatum and brainstem, while in CBS due to AD, atrophy extended into temporoparietal cortex and precuneus (p<0.001 uncorrected). Interpretation Frontal lobe involvement is characteristic of CBD, and in many patients frontal, not parietal or basal ganglia symptoms, dominate early-stage disease. CBS is driven by medial peri-rolandic dysfunction, but this anatomy is not specific to one single underlying histopathology. Antemortem prediction of CBD will remain challenging until clinical features of CBD are redefined, and sensitive, specific biomarkers are identified.

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Neuropsychiatric Features in 36 Pathologically Confirmed Cases of Corticobasal Degeneration

Cited by 21 publications

References 0 publications

Could a structural damage mimic a Parkinson plus syndrome?

Could a structural damage mimic a Parkinson plus syndrome?

Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy

Clinicopathological correlations in corticobasal degeneration

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