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

A 46-year-old woman was 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...

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Bibliographic Details
Published in:Arquivos de neuro-psiquiatria 2020-12, Vol.78 (12), p.815-815
Main Authors: De Moraes, Marianna Pinheiro Moraes, Trapp, Barbara, Rezende, Flávio Moura, Marussi, Victor Hugo Rocha, Barsottini, Orlando Graziani Povoas, Pedroso, José Luiz
Format: Article
Language:English
Subjects:
Angiography
Brain Edema - diagnostic imaging
Brain Edema - etiology
Cerebrospinal fluid
Cyclophosphamide
Edema
Humans
Images in Neurology
Immunotherapy
Lupus
Lupus Erythematosus, Systemic - complications
Magnetic resonance imaging
Methylprednisolone
Neuroimaging
Substantia alba
Systemic lupus erythematosus
Vasculitis
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Summary:A 46-year-old woman was 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. Malignant cerebral edema related to SLE was diagnosed. She had complete recovery after methylprednisolone and cyclophosphamide. Several neurological manifestations have been described in SLE, including intracranial hypertension syndrome, which is unusual. Malignant cerebral edema is a distinct syndrome rarely observed in SLE. Severe bloodbrain barrier disruption and vasculitis are the most likely pathophysiological mechanisms, and treatment includes immunotherapy.
ISSN:0004-282X
1678-4227
DOI:10.1590/0004-282X20200100