Imaging-Pathologic Correlation in Corticobasal Degeneration

Tokumaru, Aya M.; Saito, Yuya; Murayama, Shigeo; Kanemaru, Kazutomi; Sakiyama, Yoshio; Toyoda, M; Yamakawa, Mayumi; Terada, Hitoshi

doi:10.3174/ajnr.a1721

Cited by 36 publications

(35 citation statements)

References 21 publications

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“…Although these findings were considered to be secondary degeneration of white matter following cortical pathology [37], Tokumaru et al . suggested that the high signal intensities correlated with primary tau pathology of white matter in CBD [38]. In the present study, volume loss was not found in white matter of Cases 1 and 2, which may also correlate with the lack or mild clinical symptoms in Cases 1 and 2.…”

Section: Discussionsupporting

confidence: 47%

“…Previous results have shown that CBD patients experienced volume loss relative to controls in the motor cortex and frontal subcortical white matter [36]. In addition, fluid‐attenuated inversion recovery or high signal intensities by T2‐weighted imaging were observed in the subcortical white matter of CBD cases [37,38]. Although these findings were considered to be secondary degeneration of white matter following cortical pathology [37], Tokumaru et al .…”

Section: Discussionmentioning

confidence: 99%

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Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases

Nishida

Yoshida

Hata

et al. 2015

Neuropathology Appl Neurobio

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Section: Discussionsupporting

confidence: 47%

Section: Discussionmentioning

confidence: 99%

Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases

Nishida

Yoshida

Hata

et al. 2015

Neuropathology Appl Neurobio

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“…Hyperintense lesions in the subcortical white matter on FLAIR images is occasionally observed in CBD cases . By histopathological examination, areas overlapping with these hyperintense lesions contained myelin pallor and tau‐positive structures .…”

Section: Discussionmentioning

confidence: 94%

“…There were only two previous studies on pathologically diagnosed CBD cases showing hyperintense lesions in the deep white matter on FLAIR images . We compared the clinical course and radiological characteristics of our case with previously reported CBD cases (Table ).…”

Section: Discussionmentioning

confidence: 98%

“…On brain magnetic resonance imaging (MRI), CBD is characterized by asymmetric cortical atrophy of the frontoparietal lobe . In addition, CBD patients occasionally show subcortical white matter hyperintense lesions, which are usually observed beneath the affected area on fluid‐attenuated inversion recovery (FLAIR) images . The underlying pathophysiology is still controversial, although several studies suggest the progression of neuronal degeneration, or the accumulation of abnormally phosphorylated tau in oligodendroglia, contribute to the signal change in MRI.…”

Section: Introductionmentioning

confidence: 99%

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Corticobasal degeneration with deep white matter lesion diagnosed by brain biopsy

et al. 2020

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Corticobasal degeneration (CBD) is a rare progressive neurodegenerative disorder characterized by asymmetric presentation of cerebral cortex signs, cortical sensory disturbance and extrapyramidal signs. Herein, we report a case of a 66‐year‐old Japanese woman who presented with apraxia of the right hand. She subsequently developed postural instability and cognitive impairments that rapidly worsened. One and a half years later, the patient was wheelchair‐bound and severely demented. Brain magnetic resonance imaging revealed left dominant atrophy of the frontoparietal lobe. There was a hyperintense lesion in the deep white matter expanding toward the subcortical area on fluid‐attenuated inversion recovery (FLAIR) images. In order to rule out the possibility of an intracranial tumor such as an astrocytoma or malignant lymphoma, we performed a brain biopsy of the left frontal middle gyrus. The patient became bedridden and showed akinetic mutism 1 year after biopsy. Pathological examination revealed a large amount of 4‐repeat tau‐immunoreactive neuropil threads scattered predominantly in the corticomedullary junction and tau‐immunoreactive structures, consistent with CBD. Immunostaining for p53 showed no positive cells, and there were very few Ki‐67‐positive cells. On immunoblots of sarkosyl‐insoluble brain extracts, a major doublet of 64 and 68 kDa full‐length tau with two closely related fragments of approximately 37 kDa were detected. Based on these results, the patient was pathologically diagnosed as having CBD, excluding the possibility of tumor. Taken together with previous similar case reports, our findings indicate that a deep white matter hyperintense lesion on FLAIR images may be a useful clue to CBD, predicting rapid clinical progression with severe dementia based on severe white matter degeneration with a large amount of tau accumulation on pathological examination.

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Whole‐brain white matter disruption in semantic and nonfluent variants of primary progressive aphasia

Schwindt

Graham

Rochon

et al. 2011

Human Brain Mapping

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Semantic (svPPA) and nonfluent (nfPPA) variants of primary progressive aphasia are associated with distinct patterns of cortical atrophy and underlying pathology. Little is known, however, about their contrasting spread of white matter disruption and how this relates to grey matter (GM) loss. We undertook a structural MRI study to investigate this relationship. We used diffusion tensor imaging, tract-based spatial statistics, and voxel-based morphometry to examine fractional anisotropy (FA) and directional diffusivities in nine patients with svPPA and nine patients with nfPPA, and compared them to 16 matched controls after accounting for global GM atrophy. Significant differences in topography of white matter changes were found, with more ventral involvement in svPPA patients and more widespread frontal involvement in nfPPA individuals. However, each group had both ventral and dorsal tract changes, and both showed spread of diffusion abnormalities beyond sites of local atrophy. There was a clear dissociation in sensitivity of diffusion tensor imaging measures between groups. SvPPA patients showed widespread changes in FA and radial diffusivity, whereas changes in axial diffusivity were more restricted and proximal to sites of GM atrophy. NfPPA patients showed isolated changes in FA, but widespread axial and radial diffusivity changes. These findings reveal the extent of white matter disruption in these variants of PPA after accounting for GM loss. Further, they suggest that differences in the relative sensitivity of diffusion metrics may reflect differences in the nature of underlying white matter pathology in these two subtypes.

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Imaging-Pathologic Correlation in Corticobasal Degeneration

Cited by 36 publications

References 21 publications

Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases

Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases

Corticobasal degeneration with deep white matter lesion diagnosed by brain biopsy

Whole‐brain white matter disruption in semantic and nonfluent variants of primary progressive aphasia

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