The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases
- Author:
Christopher WAY
1
;
David PETTERSSON
;
Amie HILLER
Author Information
- Publication Type:Original Article
- Keywords: Multiple system atrophy; olivopontocerebellar atrophy; magnetic resonance imaging; cerebellar ataxia
- MeSH: 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
- From:Journal of Movement Disorders 2019;12(1):27-30
- CountryRepublic of Korea
- Language:English
- 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).
