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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282XOn-line version ISSN 1678-4227

Arq. Neuro-Psiquiatr. vol.75 no.11 São Paulo Nov. 2017

http://dx.doi.org/10.1590/0004-282x20170142 

Images in Neurology

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

Calcificação da ponte na atrofia de múltiplos sistemas forma cerebelar avançada: um marcador de neurodegeneração para as sinucleinopatias?

José Luiz Pedroso1  2 

Orlando G. Barsottini1  2 

Marcos Knobel2 

Benjamin Wolf Handfas2 

Antonio J. Rocha3 

Elias Knobel2 

1Universidade Federal de São Paulo, Unidade de Ataxia, Departamento de Neurologia, São Paulo SP, Brasil

2Hospital Israelita Albert Einstein, São Paulo SP, Brasil

3Faculdade de Ciências Médicas da Santa Casa de São Paulo, Divisão de Radiologia, São Paulo SP, Brasil

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).

Figure 1 MRI performed two years after disease onset. (A) Sagittal T1-weighted shows pontocerebellar atrophy; (B) Axial T1-weighted depicted the “hot cross bun” sign; (C) Axial gradient-echo showed no evidence of calcification in the pons. 

Figure 2 Brain CT performed 15 years after disease onset. Marked pontine calcification, centered on transverse pontocerebellar fibers and raphe. 

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.

References

1. Gilman S, Low PA, Quinn N, Albanese A, Ben-Shlomo Y, Fowler CJ et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci. 1999;163(1):94-8. https://doi.org/10.1007/BF02309628Links ]

2. Nemoto H, Konno S, Nakazora H, Wakata N, Kurihara T. Pontine calcification in a case with cerebellar type of multiple system atrophy: incidental or related to synucleinopathy. Intern Med. 2006;45(9):667-8. https://doi.org/10.2169/mtemalmedicine.45.1706Links ]

Received: December 10, 2016; Accepted: July 12, 2017

Correspondence: José Luiz Pedroso; Departamento de Neurologia da UNIFESP; Rua Pedro de Toledo, 650, CEP 04039-002; São Paulo SP, Brasil. E-mail: jlpedroso.neuro@gmail.com

Conflict of interest: There is no conflict of interest to declare.

Creative Commons License This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.