Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3.
Hot cross bun sign
Magnetic resonance imaging
Multiple system atrophy
Orthostatic hypotension
Spinocerebellar ataxia type 3
Journal
BMC neurology
ISSN: 1471-2377
Titre abrégé: BMC Neurol
Pays: England
ID NLM: 100968555
Informations de publication
Date de publication:
27 Apr 2020
27 Apr 2020
Historique:
received:
10
01
2020
accepted:
20
04
2020
entrez:
29
4
2020
pubmed:
29
4
2020
medline:
17
9
2020
Statut:
epublish
Résumé
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. 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. 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. 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.
Sections du résumé
BACKGROUND
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
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
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
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.
Identifiants
pubmed: 32340608
doi: 10.1186/s12883-020-01738-9
pii: 10.1186/s12883-020-01738-9
pmc: PMC7184719
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
157Références
Sci Rep. 2019 Nov 22;9(1):17329
pubmed: 31758059
JAMA Neurol. 2015 Jul;72(7):797-805
pubmed: 26010696
J Neurol Neurosurg Psychiatry. 1999 Nov;67(5):620-3
pubmed: 10519868
Mov Disord. 2012 Dec;27(14):1754-62
pubmed: 22488922
Clin Auton Res. 2018 Apr;28(2):215-221
pubmed: 29313153
Eur J Neurol. 2009 Apr;16(4):513-6
pubmed: 19187260
Cerebellum. 2008;7(2):189-97
pubmed: 18418674
Clin Neurol Neurosurg. 2017 Aug;159:29-33
pubmed: 28527404
Mov Disord. 2009 Nov 15;24(15):2272-6
pubmed: 19845011
J Neurol Neurosurg Psychiatry. 1998 Jul;65(1):65-71
pubmed: 9667563
Neurology. 2008 Aug 26;71(9):670-6
pubmed: 18725592
Brain. 2002 May;125(Pt 5):1070-83
pubmed: 11960896
Mov Disord Clin Pract. 2016 Jul 28;4(1):12-20
pubmed: 30363358
Handb Clin Neurol. 2012;103:437-49
pubmed: 21827905
J Neurol. 2002 Jul;249(7):847-54
pubmed: 12140668
Cerebellum. 2016 Dec;15(6):663-679
pubmed: 26467153
Neurology. 2000 Feb 8;54(3):697-702
pubmed: 10680806
Arch Neurol. 2005 Jun;62(6):981-5
pubmed: 15956170
Neurology. 1996 May;46(5):1470
pubmed: 8628505
J Neurol Sci. 2006 Nov 15;249(2):115-21
pubmed: 16828805
J Neuroimaging. 2006 Jan;16(1):73-7
pubmed: 16483280
J Neurol. 2015 Jun;262(6):1433-9
pubmed: 25845765
Eur J Radiol. 2012 Oct;81(10):2848-52
pubmed: 22209432
Parkinsonism Relat Disord. 2004 Aug;10(6):363-8
pubmed: 15261878
Lancet Neurol. 2018 Apr;17(4):327-334
pubmed: 29553382
J Neurol Sci. 2018 Apr 15;387:187-195
pubmed: 29571861