Pontine calcification in late stage cerebellar multiple system atrophy: a marker of synucleinopathy neurodegeneration?

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 calcification (Figure 2). Multiple system atrophy is a neurodegenerative disorder included in the synucleinopathies group1. The cerebellar form, MSA-C, usually causes pontocerebellar atrophy and a “hot cross bun” sign in the pons, due to selective loss of myelinated transverse pontocerebellar fibers and raphe1,2. This prospective observation demonstrates that pontine calcification may be part of the neurodegeneration process in MSA-C.