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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(05): 324-325
DOI: 10.1590/0004-282X20170037
IMAGES IN NEUROLOGY

The many faces of demyelinating diseases: acute disseminated encephalomyelitis and Guillain-Barré syndrome in the same patient

As várias faces das doenças desmielinizantes: encefalomielite aguda disseminada e síndrome de Guillain-Barré no mesmo paciente
Régis Augusto Reis Trindade
1   Hospital de Clínicas de Porto Alegre (HCPA), Radiologia e Diagnóstico por Imagem, Porto Alegre RS, Brasil;
,
Amália Izaura Nair Medeiros Klaes
1   Hospital de Clínicas de Porto Alegre (HCPA), Radiologia e Diagnóstico por Imagem, Porto Alegre RS, Brasil;
,
Juliana Ávila Duarte
2   Hospital de Clínicas de Porto Alegre (HCPA), Ressonância Magnética, Porto Alegre RS, Brasil.
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Acute disseminated encephalomyelitis and Guillain-Barré syndrome represent distinct demyelinating diseases that share an autoimmune pathogenesis. A history of viral infection or vaccination are essential for the diagnosis[1],[2],[3].

A 26-month-old boy presented with fever 10 days after vaccination (inactivated polio vaccine and tetravalent), progressive drowsiness, lower limb strength/sensory loss and urinary retention. The cerebrospinal fluid showed mild pleocytosis and an elevated total protein concentration; it was negative for infections. The initial MRI study was compatible with acute disseminated encephalomyelitis, with no spine abnormalities ([Figure 1]).

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Figure 1 Brain MRI revealed white matter hyperintensities lesions in T2/fluid- attenuated inversion recovery (FLAIR) (A, B) in the cerebral hemispheres and cerebellum, without restriction to water molecules diffusivity or gadolinium enhancement, suggestive of acute disseminated encephalomyelitis . Sagittal T1 SPIR post-gadolinium (C), without cauda equina root enhancement.

Ten days later, after therapy with corticosteroids, he experienced acute paraplegia. Follow-up brain and spine MRI scans demonstrated partial regression of brain lesions and nerve root thickening with intense enhancement extending along the cauda equina, compatible with Guillain-Barré syndrome. ([Figure 2]).

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Figure 2 Follow-up MRI one week after steroids, showed partial recovery of the brain lesions; axial (A) and sagittal T1 SPIR post-gadolinium (B), enhancement of the cauda equina roots (white arrow), compatible with Guillain-Barré Syndrome.


Publication History

Received: 31 August 2016

Accepted: 03 February 2017

Article published online:
05 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 Okumura A, Ushida H, Maruyama K, Itomi K, Ishiguro Y, Takahashi M et al. Guillain-Barré syndrome associated with central nervous system lesions. Arch Dis Child. 2002;86(4):304-6. https://doi.org/10.1136/adc.86.4.304
  • 3 Mohamed RR, Jan MM. Co-morbid Guillain-Barré syndrome and acute disseminated encephalomyelitis. Neurosciences (Riyadh). 2013;18(2):166-8.