Intracranial hypertension as an initial clinical manifestation of Systemic Lupus Erythematosus

Abstract

Background: Systemic Lupus Erythematosus (SLE) is an auto-immune systemic disorder that may affect multiple organ systems including central nervous system. Although involvement of the nervous system and eye is not uncommon in SLE, the presence of intracranial hypertension (IH) with papilledema in SLE a rare occurrence and it requires high level of suspicion to be diagnosed, especially when the combination of IH and papilledema is the initial presentation of SLE. Case presentation: A 21-year-old female presented with a month long history of progressively deteriorating bilateral frontotemporal headache of throbbing quality. In neurological examination the only finding was bilateral papilledema with mild peripheral visual field Constriction. A brain Magnetic Resonance Imaging (MRI), MR angiography and venography showed no abnormal findings. Cerebrospinal fluid (CSF) analysis revealed a high opening pressure (320 mm H2O) with normal CSF indices. She also had normocytic, normochromic anemia, mild thrombocytopenia, increased quantitative CRP, ESR and LE cell was also reported. Upon further work-up, it was revealed that she had positive ANA and Anti-ds-DNA, decreased level of complement C3 and C4. She, with SLE, was treated with corticosteroid which resulted in significant improvement in patient's manifestation of IH. Conclusions: The association of IH and SLE has already been established and one should be aware of this coexistence and in IH cases, SLE should also be considered as an underlying cause and subsequently appropriate diagnostic tests should be performed. Keywords: SLE, IIH, Papilledema