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

• Published in: Journal of movement disorders Vol. 12; no. 1; pp. 27 - 30
• Main Authors: Way, Christopher, Pettersson, David, Hiller, Amie
• Format: Journal Article
• Language: English
• Published: Korea (South) The Korean Movement Disorder Society 01.01.2019; Korean Movement Disorder Society; 대한파킨슨병및이상운동질환학회
• Subjects: cerebellar ataxia; magnetic resonance imaging; Multiple system atrophy; olivopontocerebellar atrophy; Original; 신경과학; Multiple system atrophy; cerebellar ataxia; magnetic resonance imaging; olivopontocerebellar atrophy
• ISSN: 2005-940X; 2093-4939
• DOI: 10.14802/jmd.18031

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