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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(11): 833-834
DOI: 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 Pedroso
1   Universidade Federal de São Paulo, Unidade de Ataxia, Departamento de Neurologia, São Paulo SP, Brasil
2   Hospital Israelita Albert Einstein, São Paulo SP, Brasil
,
Orlando G. Barsottini
1   Universidade Federal de São Paulo, Unidade de Ataxia, Departamento de Neurologia, São Paulo SP, Brasil
2   Hospital Israelita Albert Einstein, São Paulo SP, Brasil
,
Marcos Knobel
2   Hospital Israelita Albert Einstein, São Paulo SP, Brasil
,
Benjamin Wolf Handfas
2   Hospital Israelita Albert Einstein, São Paulo SP, Brasil
,
Antonio J. Rocha
3   Faculdade de Ciências Médicas da Santa Casa de São Paulo, Divisão de Radiologia, São Paulo SP, Brasil
,
Elias Knobel
2   Hospital Israelita Albert Einstein, São Paulo SP, Brasil
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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]).

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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.
Zoom Image
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 group[1]. 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 raphe[1],[2]. This prospective observation demonstrates that pontine calcification may be part of the neurodegeneration process in MSA-C.



Publication History

Received: 10 December 2016

Accepted: 12 July 2017

Article published online:
01 September 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

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  • 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.45.1706