Differential value of brain magnetic resonance imaging in multiple system atrophy cerebellar phenotype and spinocerebellar ataxias

Kim, Min-Kyeong; Ahn, Jong Hyeon; Cho, Yoonsu; Kim, Ji Sun; Youn, Jinyoung; Cho, Jin Whan

doi:10.1038/s41598-019-53980-y

Cited by 24 publications

(42 citation statements)

References 33 publications

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“…Among patients imaged within 2 or 3 years after onset, grade 2 HCB was observed in none of those with SCA3, yielding a high specificity of 100% for distinguishing between MSA-C and SCA3. In line with the results of this study, previous studies that evaluated HCB in MSA-C and SCA including SCA2, SCA3, SCA7, and SCA8, described that grade 2 HCB was not observed in 39 patients with SCA3 within 3 years after onset [16]. In an evaluation of the HCB in adult cerebellar ataxia that included 33 patients with SCA3 (disease duration 6.3 ± 6.0 years), a pontine midline linear hyperintensity was observed 24 (72.7%), but none had the complete cruciform HCB [15].…”

Section: Discussionsupporting

confidence: 91%

“…An HCB of either grade 1 or 2 had a high sensitivity of 91.7% in MSA-C within 2 years after disease onset, and a grade 2 HCB had a sensitivity of 54.2% at 2 years and 66.7% at 3 years after disease onset. Our findings suggest that, in a case of progressive cerebellar ataxia, if an HCB is not observed on MRI within 2 years after the onset of cerebellar symptoms, it is unlikely that the diagnosis is MSA-C. A recent study showed that either a vertical or a cruciform hyperintensity was detected in 80.1% (149/186) of the patients with MSA-C within 3 years after onset [16]. This result is consistent with that of this study but had a lower frequency.…”

Section: Discussionmentioning

confidence: 58%

“…In particular, a grade 2 HCB was reportedly observed more frequently in SCA2 than in SCA3 [9,15]. Kim et al reported that grade 2 HCB was observed in 1 of 36 patients with SCA2 within 3 years after onset [16].…”

Section: Discussionmentioning

confidence: 98%

“…Finally, in some cases, the middle cerebellar peduncle was not adequately visualized well enough to be evaluated in the axial images, so that the hyperintensity in the middle cerebellar peduncle could not be evaluated in this study. A hyperintensity in the middle cerebellar peduncle on T2WI is reportedly highly specific for differentiating MSA from other neurodegenerative diseases [5,16,17,25,26]. Combining the HCB with a hyperintensity in the middle cerebellar peduncle may increase the specificity of MSA diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

et al. 2020

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Background: The "hot cross bun" (HCB) sign, a cruciform hyperintensity in the pons on magnetic resonance imaging (MRI), has gradually been identified as a typical finding in multiple system atrophy, cerebellar-type (MSA-C). Few reports have evaluated the sensitivity of an HCB, including a cruciform hyperintensity and vertical line in the pons, which precedes a cruciform hyperintensity, in the early stages of MSA-C. Moreover, the difference in frequency and timing of appearance of an HCB between MSA-C and spinocerebellar ataxia type 3 (SCA3) has not been fully investigated. Methods: This study investigated the time at which an HCB and orthostatic hypotension (OH) appeared in 41 patients with MSA-C, based on brain MRI and head-up tilt test. The MRI findings were compared with those of 26 patients with SCA3. The pontine signal findings on T2-weighted MRI were graded as 0 (no change), 1 (a vertical T2 high-intensity line), or 2 (a cruciform T2 high-intensity line), with grades 1 or 2 considered as an HCB. OH 30/15 was defined as a decrease in systolic blood pressure of > 30 mmHg or diastolic blood pressure of > 15 mmHg. Results: Among the 24 patients with MSA-C within 2 years from the onset of motor symptoms, an HCB was detected in 91.7%, whereas OH 30/15 was present in 60.0%. Among the 36 patients with MSA-C within 3 years from the onset of motor symptoms, a grade 2 HCB was detected in 66.7% of those with MSA-C but in none of those with SCA-3. Conclusions: HCB is a highly sensitive finding for MSA-C, even in the early stages of the disease. A grade 2 HCB in the early stage is an extremely specific finding for differentiating MSA-C from SCA-3.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 58%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

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Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

et al. 2020

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“…An HCB of either grade 1 or 2 had a high sensitivity of 91.7% in MSA-C within 2 years after disease onset, and a grade 2 HCB had a sensitivity of 54.2% at 2 years and of 66.7% at 3 years after disease onset. Our findings suggest that, in a case of progressive cerebellar ataxia, if an HCB is not observed on MRI by 2 years after onset of cerebellar symptoms, it is unlikely that the diagnosis is MSA-C. A recent study showed that either a vertical or a cruciform hyperintensity was detected in 80.1% (149/186) of the patients with MSA-C within 3 years after onset [16]. This result is consistent with that of this study but had a lower frequency.…”

Section: Discussionmentioning

confidence: 56%

The vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

Sugiyama

Yokota

Yamanaka

et al. 2020

Preprint

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Background The “hot cross bun” (HCB) sign, a cruciform hyperintensity in the pons on magnetic resonance imaging (MRI), has gradually been identified as a typical finding in multiple system atrophy, cerebellar-type (MSA-C). Few reports have evaluated the sensitivity of an HCB including a cruciform hyperintensity and vertical line in the pons, which precedes a cruciform hyperintensity, in the early stages of MSA-C. Moreover, the difference in frequency and timing of appearance of an HCB between MSA-C and spinocerebellar ataxia type 3 (SCA3) has not been fully investigated. Methods This study investigated the time at which an HCB and orthostatic hypotension (OH) appeared in 41 patients with MSA-C, based on brain MRI and head-up tilt test. The MRI findings were compared with those of 26 patients with SCA3. The pontine signal findings on T2-weighted MRI were graded as 0 (no change), 1 (a vertical T2 high-intensity line), or 2 (a cruciform T2 high-intensity line), with grades 1 or 2 considered as an HCB. OH 30/15 was defined as a decrease in systolic blood pressure of > 30 mmHg or diastolic blood pressure of > 15 mmHg. Results Among the 24 patients with MSA-C within 2 years from the onset of motor symptoms, an HCB was detected in 91.7%, whereas OH 30/15 was present in 60.0%. Among the 36 patients with MSA-C within 3 years from the onset of motor symptoms, a grade 2 HCB was detected in 66.7% of those with MSA-C but in none of those with SCA-3. Conclusions HCB is a highly sensitive finding for MSA-C, even in the early stages of the disease. A grade 2 HCB in the early stage is an extremely specific finding for differentiating MSA-C from SCA-3.

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Diseases of the Central Nervous System

Shimosegawa¹,

Shigemoto²,

Sato³

et al. 2023

Radiology‐Nuclear Medicine Diagnostic Imaging

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Differential value of brain magnetic resonance imaging in multiple system atrophy cerebellar phenotype and spinocerebellar ataxias

Cited by 24 publications

References 33 publications

Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

The vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

Diseases of the Central Nervous System

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