Disease The “hot cross bun” sign refers to pontine cruciform hyperintensity on long TR sequences, which can be observed in multiple-system atrophy, spinocerebellar atrophy types 2 and 3, and in parkinsonism secondary to vasculitis. It has not been previously demonstrated in variant Creutzfeldt-Jakob disease (vCJD), to our knowledge. A 16-year-old girl, diagnosed with vCJD at age 14 years and de...

1. Wenning GK, Colosimo G, Geser F, Poewe W. Multiple system atrophy [review]. Lancet Neurol. 2004;3:93—103. 2. Sitburana O, Ondo WG. Brain magnetic resonance imaging (MRI) in parkinsonian disorders. Parkinsonism Relat Disord. 2009;15:165—74. 3. Pereiro I, Arias M, Requena I. Signo de santiaguiño en la atrofia multisistémica. Neurologia. 2010;25:336—7. 4. Shrivastava A. The hot cross bun sign. ...

A 78-year-old woman presented with progressive ataxia, dysautonomia (hypotension and urinary incontinence), and pyramidal signs that started 17 years ago. Brain MRI performed 15 years ago showed pontocerebellar atrophy and the “hot cross bun” sign (Figure 1). Multiple system atrophy, cerebellar form (MSA-C) was diagnosed. A routine brain CT scan performed two years ago disclosed pontine calcifi...