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

Edema cerebral maligno: uma manifestação neurológica atípica do lúpus eritematoso sistêmico

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.

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 unusual¹. Malignant cerebral edema is a distinct syndrome rarely observed in SLE². Severe blood-brain barrier disruption and vasculitis are the most likely pathophysiological mechanisms, and treatment includes immunotherapy².

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
  » 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
  » https://doi.org/10.1016/j.jns.2016.03.040

Publication Dates

• Publication in this collection
  07 Dec 2020
• Date of issue
  Dec 2020

History

• Received
  26 May 2020
• Reviewed
  05 June 2020
• Accepted
  15 June 2020