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. 2024 Nov;66(11):1917-1929.
doi: 10.1007/s00234-024-03432-w. Epub 2024 Jul 22.

Conventional magnetic resonance imaging key features for distinguishing pathologically confirmed corticobasal degeneration from its mimics: a retrospective analysis of the J-VAC study

Keita Sakurai  1 , Aya M Tokumaru  2 , Mari Yoshida  3 , Yuko Saito  4   5 , Koichi Wakabayashi  6 , Takashi Komori  7 , Masato Hasegawa  8 , Takeshi Ikeuchi  9 , Yuichi Hayashi  10 , Takayoshi Shimohata  10 , Shigeo Murayama  4   11 , Yasushi Iwasaki  3 , Toshiki Uchihara  12   13 , Motoko Sakai  14 , Ichiro Yabe  15 , Satoshi Tanikawa  16 , Hiroshi Takigawa  17 , Tadashi Adachi  17 , Ritsuko Hanajima  17 , Harutoshi Fujimura  18 , Kentaro Hayashi  19 , Keizo Sugaya  19 , Kazuko Hasegawa  20 , Terunori Sano  21 , Masaki Takao  21 , Osamu Yokota  22   23 , Tomoko Miki  22   23 , Michio Kobayashi  24 , Nobutaka Arai  25 , Takuya Ohkubo  26 , Takanori Yokota  26 , Keiko Mori  27 , Masumi Ito  27 , Chiho Ishida  28 , Jiro Idezuka  29 , Yasuko Toyoshima  30   31 , Masato Kanazawa  32 , Masashi Aoki  33 , Takafumi Hasegawa  33 , Hirohisa Watanabe  34 , Atsushi Hashizume  35 , Hisayoshi Niwa  36 , Keizo Yasui  37 , Keita Ito  38 , Yukihiko Washimi  39 , Akatsuki Kubota  40 , Tatsushi Toda  40 , Kenji Nakashima  41 , Ikuko Aiba  42 ; J-VAC study group
Affiliations

Conventional magnetic resonance imaging key features for distinguishing pathologically confirmed corticobasal degeneration from its mimics: a retrospective analysis of the J-VAC study

Keita Sakurai et al. Neuroradiology. 2024 Nov.

Abstract

Purpose: Due to the indistinguishable clinical features of corticobasal syndrome (CBS), the antemortem differentiation between corticobasal degeneration (CBD) and its mimics remains challenging. However, the utility of conventional magnetic resonance imaging (MRI) for the diagnosis of CBD has not been sufficiently evaluated. This study aimed to investigate the diagnostic performance of conventional MRI findings in differentiating pathologically confirmed CBD from its mimics.

Methods: Semiquantitative visual rating scales were employed to assess the degree and distribution of atrophy and asymmetry on conventional T1-weighted and T2-weighted images. Additionally, subcortical white matter hyperintensity (SWMH) on fluid-attenuated inversion recovery images were visually evaluated.

Results: In addition to 19 patients with CBD, 16 with CBD mimics (progressive supranuclear palsy (PSP): 9, Alzheimer's disease (AD): 4, dementia with Lewy bodies (DLB): 1, frontotemporal lobar degeneration with TAR DNA-binding protein of 43 kDa(FTLD-TDP): 1, and globular glial tauopathy (GGT): 1) were investigated. Compared with the CBD group, the PSP-CBS subgroup showed severe midbrain atrophy without SWMH. The non-PSP-CBS subgroup, comprising patients with AD, DLB, FTLD-TDP, and GGT, showed severe temporal atrophy with widespread asymmetry, especially in the temporal lobes. In addition to over half of the patients with CBD, two with FTLD-TDP and GGT showed SWMH, respectively.

Conclusion: This study elucidates the distinct structural changes between the CBD and its mimics based on visual rating scales. The evaluation of atrophic distribution and SWMH may serve as imaging biomarkers of conventional MRI for detecting background pathologies.

Keywords: Asymmetry; Corticobasal degeneration; Corticobasal syndrome; Magnetic resonance imaging; Subcortical white matter hyperintensity.

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Conflict of interest statement

The authors declared that they have no competing interests.

Figures

Fig. 1
Fig. 1
Visual rating scale of midbrain atrophy. The degree of midbrain atrophy was assessed using the three-point scale (0 = normal, 1 = mild, and 2 = severe). Compared with the normal midbrain in a patient with pathologically confirmed AD (a), mild midbrain atrophy showing a slight decrease in anteroposterior and/or superoinferior diameters was observed in a patient with pathologically confirmed CBD (b). By contrast, a definite decrease in these diameters was classified as severe midbrain atrophy in a patient with pathologically confirmed PSP (c). AD, Alzheimer’s disease; CBD, corticobasal degeneration; PSP, progressive supranuclear palsy
Fig. 2
Fig. 2
Visual rating scale of asymmetric atrophy. The degree of asymmetry in the frontal, temporal, parietal, and occipital lobes, and cerebral peduncle was assessed using a three-point scale (0 = normal, 1 = mild, and 2 = severe). In contrast to no obvious asymmetry in a patient with pathologically confirmed DLB (a), mild asymmetry and severe asymmetry were observed in patients with pathologically confirmed PSP (b) and CBD (c). CBD, corticobasal degeneration; DLB, dementia with Lewy bodies; PSP, progressive supranuclear palsy
Fig. 3
Fig. 3
Evaluation of SWMH. The presence/absence of hyperintensity confined to the subcortical white matter (i.e., SWMH) was evaluated on an axial FLAIR image. In contrast to other lobes at lower convexity level (a, b), distinct SWMH of the bilateral frontal lobes at high convexity (c) was observed in a patient with pathologically confirmed CBD. CBD, corticobasal degeneration; SWMH, subcortical white matter hyperintensity
Fig. 4
Fig. 4
Flow chart showing the inclusion and exclusion of patients with pathologically confirmed CBD and those with mimics. AD, Alzheimer’s disease; CBD, corticobasal degeneration; DLB, dementia with Lewy bodies; FTLD-TDP, frontotemporal lobar degeneration with TAR DNA-binding protein of 43 kDa; GGT, globular glial tauopathy; PSP, progressive supranuclear palsy
Fig. 5
Fig. 5
A representative case of radiologically typical CBD. This 69-year-old female patient obtained an antemortem clinical diagnosis of AD. After the postmortem pathological examination, it was proved that main pathology of this patient was CBD. MRI was performed one year after the symptom onset. In contrast to mild atrophy of the callosal body (arrow, a), sagittal T1WI showed relatively preserved midbrain. Axial T1WI and FLAIR image showed right dominant asymmetric atrophy of the cerebral peduncle and frontal lobe (arrow, b). Additionally, mild SWMH was detected in the frontal lobe at high convexity (circle, c). A magnified view was exhibited to emphasize the asymmetry of the short axis in the cerebral peduncles (double sided arrows, d). a, sagittal T1WI; b, axial T1WI; c, axial FLAIR image; AD, Alzheimer’s disease; CBD, corticobasal degeneration; FLAIR, fluid-attenuated inversion recovery; SWMH, subcortical white matter hyperintensity; T1WI, T1-weighted image
Fig. 6
Fig. 6
A representative case of radiologically atypical CBD. This 67-year-old female patient obtained an antemortem diagnosis of CBD. MRI was performed four years after the symptom onset. In addition to atrophy of the callosal body (arrow, a), mild midbrain atrophy was also detected on sagittal T1WI (arrowhead, b). In contrast to right dominant asymmetric atrophy of the cerebral peduncle and frontal lobe (arrows, b, c), no obvious SWMH was noted on axial FLAIR image (c). A magnified view was exhibited to emphasize the asymmetry of the short axis in the cerebral peduncles (double sided arrows, d). a, sagittal T1WI; b, axial T1WI; c, axial FLAIR image; CBD, corticobasal degeneration; FLAIR, fluid attenuated inversion recovery; SWMH, subcortical white matter hyperintensity; T1WI, T1-weighted image
Fig. 7
Fig. 7
A representative case of CBD mimics. This 71-year-old male patient obtained a postmortem diagnosis of PSP. MRI was performed two years after the symptom onset. Sagittal and axial T1WI (a, b), and axial FLAIR image (c) showed atrophy of the midbrain and callosal body (arrow and arrowhead, a), and right dominant asymmetric atrophy of the cerebral peduncle without obvious SWMH (arrow, b). These MRI findings were very similar to those of the patient described in Fig. 6. The case of this patient was exhibited to clarify the challenges when differentiating CBD without SWMH from its mimics, especially PSP. A magnified view was exhibited to emphasize the asymmetry of the short axis in the cerebral peduncles (double sided arrows, d). a, sagittal T1WI; b, axial T1WI; c, axial FLAIR image; CBD, corticobasal degeneration; FLAIR, fluid attenuated inversion recovery; PSP, progressive supranuclear palsy; SWMH, subcortical white matter hyperintensity; T1WI, T1-weighted image
Fig. 8
Fig. 8
A representative case of CBD mimics. This 74-year-old male patient obtained a postmortem diagnosis of FTLD-TDP. MRI was performed three years after the symptom onset. The confusable atrophy of the midbrain and callosal body (arrow and arrowhead, a), asymmetric atrophy of the left cerebral peduncle (arrow, b) and frontal lobe, and SWMH in the left frontal lobe at high convexity (circle, c) were detected. The severity of bilateral temporal lobe atrophy (arrowheads, b) significantly differed from that of CBD. This patient was exhibited to clarify the different atrophy patterns in the CBD mimics group, especially the non-PSP subgroup. A magnified view was exhibited to emphasize the asymmetry of the short axis in the cerebral peduncles (double sided arrows, d). a, sagittal T1WI; b, axial T1WI; c, axial FLAIR image; CBD, corticobasal degeneration; FLAIR, fluid attenuated inversion recovery; FTLD-TDP, frontotemporal lobar degeneration with TAR DNA-binding protein of 43 kDa; SWMH, subcortical white matter hyperintensity; T1WI, T1-weighted image
Fig. 9
Fig. 9
A schema of atrophic changes and SWMH in patients with CBD and its mimics. In this scheme, asymmetry and signal change were colored in the right hemisphere, and degree of brain atrophy was colored in the left hemisphere, respectively. CBD, corticobasal degeneration; CBS, corticobasal syndrome; PSP, progressive supranuclear palsy; SWMH, subcortical white matter hyperintensity
See this image and copyright information in PMC

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