Acute Brainstem syndrome with NMOSD and related to systemic lupus erythematosus, A Rare Case Report (PP-02)

Document Type : Poster Presentation


1 Isfahan University of medical sciences, Isfahan, Iran

2 Isfahan University of Medical Sciences, Isfahan, Iran


Introduction: Neuromyelitis optica spectrum disorders (NMOSD) are a family of syndromes, dominated by optic neuritis (ON) and myelitis, but also including brainstem syndrome. Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect the CNS. We report a case in which SLE and NMOSD subsequently occurred and presented with acute brainstem syndrome.
Case History: A 36-year-old woman with history of poorly managed SLE was brought to the emergency department with sudden onset respiratory distress and a witnessed tonic-clonic seizure. The symptoms started a few days ago with headache, blurred vision, vertigo, ataxia, nausea, and hiccups. The patient had bilateral visual loss one year ago, which recovered after a few days without treatment. On examination, clinical features include drowsiness, facial asymmetry, dysphagia and tachypnea, lower limb movement weakness, and urinary incontinence, Babinski sign. She was diagnosed lupus a long time but non-cooperative for treatment. Initial assessment revealed afebrile, tachypnea, tachycardia without meningeal irritation. She intubated and managing the frequent seizures with midazolam. Lab data showed mild leukocytosis, and ABG analysis revealed respiratory acidosis.  ANA and anti-dsDNA tests were positive. AQP4-IgG and MOG-IgG checked once but was negative. CSF analysis was normal.  Chest CT Scan displayed bilateral patchy infiltrates. Brain MRI (Figs. 1-3) showed hyper intensity in posterior of pons and midbrain in FLAIR, T2, DWI sequences. Cervical MRI was unremarkable. Based on the clinical presentation and investigation, treatment with pulse of methyl prednisolone 1gr/day for five days and then cyclophosphamide has been started. The patient showed gradual improvement in respiratory distress and regained consciousness.
Discussion: Diagnostic criteria of NMOSD was fulfilled. This rare case illustrates the importance of accurate diagnosis and targeted treatment of NMOSD when coexisting with SLE.


  • Receive Date: 14 December 2023
  • Accept Date: 14 December 2023