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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2020; 78(12): 815
DOI: 10.1590/0004-282X20200100
Images in Neurology

Malignant cerebral edema: an unusual neurological manifestation of systemic lupus erythematosus

Edema cerebral maligno: uma manifestação neurológica atípica do lúpus eritematoso sistêmico
Marianna Pinheiro Moraes De Moraes 
1   Universidade Federal de São Paulo, Department of Neurology, São Paulo SP, Brazil.
,
Barbara Trapp 
2   Universidade Federal de São Paulo, Neuroradiology Subdivision, Department of Imaging Diagnosis, São Paulo SP, Brazil.
,
Flávio Moura Rezende  Filho
1   Universidade Federal de São Paulo, Department of Neurology, São Paulo SP, Brazil.
,
Victor Hugo Rocha Marussi 
2   Universidade Federal de São Paulo, Neuroradiology Subdivision, Department of Imaging Diagnosis, São Paulo SP, Brazil.
3   Beneficência Portuguesa de São Paulo, Department of Neuroradiology, São Paulo SP, Brazil.
,
Orlando Graziani Povoas Barsottini 
1   Universidade Federal de São Paulo, Department of Neurology, São Paulo SP, Brazil.
,
José Luiz Pedroso 
1   Universidade Federal de São Paulo, Department of Neurology, São Paulo SP, Brazil.
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A 46-year-old woman presented with headache and decreased level of consciousness. She had systemic lupus erythematosus (SLE) with positive antibodies. Cerebrospinal fluid (CSF) pressure was 350 mmH2O. Brain magnetic resonance imaging (MRI) revealed white matter changes and edema. MRI angiography and vessel wall imaging ruled out vasculitis ([Figure 1]). Malignant cerebral edema related to SLE was diagnosed. She had complete recovery after methylprednisolone and cyclophosphamide.

Zoom Image
Figure 1 Brain CT scan shows cerebral edema with loss of sulci and white matter hypodensity (A and B). Axial FLAIR-weighted and coronal T2-weighted brain MRI reveal bilateral, symmetrical, and diffuse hyperintense signal in white matter, with loss of cerebral sulci, characterizing cerebral edema (C, D, and E). MRI angiography is normal (F).

Several neurological manifestations have been described in SLE, including intracranial hypertension syndrome, which is unusual[1]. Malignant cerebral edema is a distinct syndrome rarely observed in SLE[2]. Severe blood-brain barrier disruption and vasculitis are the most likely pathophysiological mechanisms, and treatment includes immunotherapy[2].


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Conflict of interest:

There is no conflict of interest to declare.

Authors’ contributions:

MMPM: case report project conception, organization, execution, manuscript writing of the first draft, review and critique. BT: case report project: conception, organization, execution, manuscript writing of the first draft, review and critique. FMRF: case report project: conception, organization, execution, manuscript review and critique. VHRM: case report project conception, organization, execution, manuscript review and critique. OGPB: case report project conception, organization, execution, manuscript review and critique. JLP: case report project conception, organization, execution, manuscript review and critique.


  • References

  • 1 Alessi H, Dutra LA, Braga Neto P, Pedroso JL, Toso FF, Kayser C, et al. Neuropsychiatric lupus in clinical practice. Arq. Neuropsiquiatr. 2016 Dec;74(12):1021-30. https://doi.org/10.1590/0004-282x20160150
  • 2 Koffman L, Prayson R, Manno EM. Malignant cerebral edema related to systemic lupus erythematosus. J Neurol Sci. 2016 Mar;364:180. https://doi.org/10.1016/j.jns.2016.03.040

Address for correspondence

Orlando Graziani Povoas Barsottini

Publication History

Received: 26 May 2020

Accepted: 15 June 2020

Article published online:
07 June 2023

© 2020. 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

  • 1 Alessi H, Dutra LA, Braga Neto P, Pedroso JL, Toso FF, Kayser C, et al. Neuropsychiatric lupus in clinical practice. Arq. Neuropsiquiatr. 2016 Dec;74(12):1021-30. https://doi.org/10.1590/0004-282x20160150
  • 2 Koffman L, Prayson R, Manno EM. Malignant cerebral edema related to systemic lupus erythematosus. J Neurol Sci. 2016 Mar;364:180. https://doi.org/10.1016/j.jns.2016.03.040

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Zoom Image
Figure 1 Brain CT scan shows cerebral edema with loss of sulci and white matter hypodensity (A and B). Axial FLAIR-weighted and coronal T2-weighted brain MRI reveal bilateral, symmetrical, and diffuse hyperintense signal in white matter, with loss of cerebral sulci, characterizing cerebral edema (C, D, and E). MRI angiography is normal (F).