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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.
Insights
The hot cross bun sign (HCBS) is not exclusive to multiple system atrophy (MSA). While common in MSA, other conditions like hereditary cerebellar ataxia also present with this neuroimaging finding.
Area of Science:
- Neurology
- Radiology
- Neuroimaging
Background:
- The 'hot cross bun' sign (HCBS) is a neuroimaging finding observed on MRI scans.
- Its diagnostic specificity for multiple system atrophy (MSA) in patients with cerebellar ataxia or parkinsonism requires clarification.
Purpose of the Study:
- To determine the specificity of the hot cross bun sign (HCBS) for diagnosing multiple system atrophy (MSA).
- To investigate alternative etiologies associated with the HCBS in adult patients presenting with cerebellar ataxia or parkinsonism.
Main Methods:
- Retrospective review of radiologic information systems using keywords related to HCBS and MSA.
- Identification of cases with HCBS by a neurologist and neuroradiologist.
- Neurological assessment and review by two neurologists to determine the etiology of symptoms in identified subjects.
Main Results:
- Eleven cases with the HCBS were identified.
- Multiple system atrophy (MSA) was the most frequent etiology (6/11 cases: 4 probable, 2 possible).
- Non-MSA etiologies accounted for 5/11 cases, including hereditary cerebellar ataxia (3/11) and probable dementia with Lewy bodies (1/11).
Conclusions:
- The hot cross bun sign (HCBS) is not pathognomonic for multiple system atrophy (MSA).
- A significant proportion of patients with HCBS do not have MSA; hereditary cerebellar ataxia is a notable alternative diagnosis.
- Further investigation is warranted to fully understand the differential diagnosis of the HCBS in neurodegenerative diseases.
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).