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JMD > Epub ahead of print
   [Epub ahead of print]
The ‘Hot Cross Bun’ Sign is Not Always Multiple System Atrophy: Etiologies of 11 Cases
Christopher Way1 , David Pettersson2, Amie Hiller3,4
1Parkinson’s Institute and Clinical Center, Sunnyvale, CA, USA
2Department of Diagnostic Radiology, Neuroradiology, Oregon Health & Sciences University, Portland, OR, USA
3Department of Neurology, Movement Disorders, Portland Veterans Administration, Parkinson’s Disease Research, Education and Clinical Center, Portland, OR, USA
4Department of Neurology, Parkinson Center & Movement Disorders Program, Oregon Health & Sciences University, Portland, OR, USA
Corresponding Author: Christopher Way ,Tel: +1-408-734-2800, Fax: +1-408-734-9208, Email: cway@parkinsonsinstitute.org
Received: June 26, 2018;   Revised: September 14, 2018;   Accepted: October 15, 2018.
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ABSTRACT

Objective
aaTo clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism.
Methods
aaThe radiologic information systems at an academic center and affiliated veterans’ hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient’s neurologic symptoms.
Results
aaEleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11).
Conclusion
aaMSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11). Key WordsaaMultiple system atrophy; olivopontocerebellar atrophy; magnetic resonance imaging; cerebellar ataxia.
Key Words: Multiple system atrophy; olivopontocerebellar atrophy; magnetic resonance imaging; cerebellar ataxia
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