Unmasking Lupus: A Case of Idiopathic Intracranial Hypertension in a Young Male
Maria Chacon1, Linker Viñan Paucar2, Jose Vasconez2, Milena Davila2, Carlos Rodriguez Alarcon2, Claudia Vera2, Rocio Santibanez2
1Universidad de Especialidades Espíritu Santo, 2Universidad Católica Santiago de Guayaquil
Objective:
To describe a case of idiopathic intracranial hypertension (IIH) as the first manifestation of systemic lupus erythematosus (SLE).
Background:
IIH is characterized by elevated intracranial pressure, papilledema, and sixth cranial nerve palsy, without hydrocephalus or lesions on imaging, and with a normal CSF composition. It is one of the least common neuropsychiatric manifestations of SLE with very few cases reported and with an estimated prevalence of 0.7%.
Results:
A 28-year-old male with a history of immune thrombocytopenia and splenectomy at age 12 presented with four days of diplopia while abroad. He denied having fever, headache or photophobia. Evaluation revealed bilateral papilledema, but a CT scan showed no structural changes, and no other neurological deficits were noted. Upon returning to his home country, he was diagnosed with right-sided sixth nerve palsy. Patient again denied other symptoms like headache or nausea. MRA ruled out cerebral vein thrombosis, and lumbar puncture showed an elevated opening pressure (540 mmH2O) with unremarkable CSF analysis. Inflammatory markers in serum were positive for D-dimer (1830 IU) and interleukin 6. Antibodies were positive for ANA (1:320), anti-dsDNA (1:80) and hypocomplementemia. Active sediment, 2.6 grams of proteinuria in 24-hour urine and preserved renal function were detected. A renal biopsy showed diffuse proliferative glomerulonephritis class IV. He was diagnosed with SLE and idiopathic intracranial hypertension. Repeated lumbar puncture after 4 weeks on acetazolamide showed a marked decrease in intracranial hypertension (320 mmH2O) and fundoscopy showed partial resolution of papilledema.
Conclusions:
IHH should be considered in patients presenting with diplopia and bilateral papilledema even in the absence of common symptoms such as headache. A thorough neurological examination is crucial. Additionally, SLE must be considered as a potential cause of IIH, particularly in patients lacking typical risk factors, ensuring a more accurate diagnosis and effective management.
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