J Mov Disord.  2019 Jan;12(1):27-30. 10.14802/jmd.18031.

The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases

• Way C
• Pettersson D
• Hiller A

• KMID: 2438675
• DOI: http://doi.org/10.14802/jmd.18031

Abstract

OBJECTIVE
To clarify the specificity of the "˜hot cross bun' sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism.
METHODS
The 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
Eleven 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
MSA 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).

Keyword

Multiple system atrophy; olivopontocerebellar atrophy; magnetic resonance imaging; cerebellar ataxia

MeSH Terms

Adult
Autopsy
Cerebellar Ataxia
Dementia
Hexachlorobenzene
Humans
Lewy Bodies
Magnetic Resonance Imaging
Multiple System Atrophy*
Neurologic Manifestations
Olivopontocerebellar Atrophies
Parkinsonian Disorders
Radiology Information Systems
Sensitivity and Specificity
Hexachlorobenzene