Clinicopathological correlations in corticobasal degeneration

Lee, Suzee E.; Rabinovici, Gil D.; Mayo, Mary Catherine; Wilson, Stephen M.; Seeley, William W.; DeArmond, Stephen J.; Huang, Eric J.; Trojanowski, John Q.; Growdon, Matthew E.; Jang, Jung Yun; Sidhu, M.; See, Tricia; Karydas, Anna; Gorno‐Tempini, Maria Luisa; Boxer, Adam L.; Weiner, Michael W.; Geschwind, Michael D.; Rankin, Katherine P.; Miller, Bruce L.

doi:10.1002/ana.22424

Cited by 381 publications

(437 citation statements)

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“…Clinicopathologic correlation studies confirm that overlapping clinical phenotypes are associated with AD and FTLD spectrum pathology in multiple domains of cognition. 17,24,25 Corticobasal syndrome often due to FTLD spectrum pathology and posterior cortical atrophy often due to AD have overlapping visuospatial features; nonfluent/agrammatic primary progressive aphasia associated with FTLD spectrum pathology and logopenic primary progressive aphasia associated with AD may have overlapping language difficulties; patients with behavioral variant frontotemporal dementia and patients with AD may present with behavioral difficulty, an executive disorder, or an episodic memory deficit. Therefore, there is an urgent need to im- prove diagnostic accuracy in a quantitative manner that does not rely on clinical features in order to screen patients for clinical trials involving diseasemodifying agents.…”

Section: Resultsmentioning

confidence: 99%

White matter imaging contributes to the multimodal diagnosis of frontotemporal lobar degeneration

McMillan¹,

Brun²,

Siddiqui³

et al. 2012

Neurology

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Objective: To evaluate the distribution of white matter (WM) disease in frontotemporal lobar degeneration (FTLD) and Alzheimer disease (AD) and to evaluate the relative usefulness of WM and gray matter (GM) for distinguishing these conditions in vivo.Methods: Patients were classified as having FTLD (n ϭ 50) or AD (n ϭ 42) using autopsy-validated CSF values of total-tau:␤-amyloid (t-tau:A␤ 1-42 ) ratios. Patients underwent WM diffusion tensor imaging (DTI) and volumetric MRI of GM. We employed tract-specific analyses of WM fractional anisotropy (FA) and whole-brain GM density analyses. Individual patient classification was performed using receiver operator characteristic (ROC) curves with FA, GM, and a combination of the 2 modalities.Results: Regional FA and GM were significantly reduced in FTLD and AD relative to healthy seniors. Direct comparisons revealed significantly reduced FA in the corpus callosum in FTLD relative to AD. GM analyses revealed reductions in anterior temporal cortex for FTLD relative to AD, and in posterior cingulate and precuneus for AD relative to FTLD. ROC curves revealed that a multimodal combination of WM and GM provide optimal classification (area under the curve ϭ 0.938), with 87% sensitivity and 83% specificity. Conclusions: FTLD and AD have significant WM and GM defects. A combination of DTI and volumetric MRI modalities provides a quantitative method for distinguishing FTLD and AD in vivo.Neurology ® 2012;78:1761-1768 GLOSSARY A␤ 1-42 ϭ ␤-amyloid; AD ϭ Alzheimer disease; CC ϭ corpus callosum; CST ϭ corticospinal tract; DTI ϭ diffusion tensor imaging; DWI ϭ diffusion-weighted image; FA ϭ fractional anisotropy; FDR ϭ false discovery rate; FTLD ϭ frontotemporal lobar degeneration; GM ϭ gray matter; IFO ϭ inferior fronto-occipital; ILF ϭ inferior longitudinal fasciculus; MMSE ϭ MiniMental State Examination; MPRAGE ϭ magnetization-prepared rapid gradient echo; PBAC ϭ Philadelphia Brief Assessment of Cognition; ROC ϭ receiver operator characteristic; SLF ϭ superior longitudinal fasciculus; t-tau ϭ total-tau; TSA ϭ tractspecific analysis; UNC ϭ uncinate fasciculus; WM ϭ white matter.

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Section: Resultsmentioning

confidence: 99%

White matter imaging contributes to the multimodal diagnosis of frontotemporal lobar degeneration

McMillan¹,

Brun²,

Siddiqui³

et al. 2012

Neurology

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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%

“…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%

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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“…For example, cases of CBS have shown Alzheimer pathology, Lewy body disease, or progressive supranuclear palsy at autopsy. 5,6 CBS classically begins as a unilateral akinetic-rigid disorder with associated localizing cortical findings that may include cortical sensory loss, alien limb phenomenon, and pyramidal findings. Apraxia is frequently associated with CBS.…”

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confidence: 99%