Study Reports Rare Case of Idiopathic Intracranial Hypertension with Onset of Lupus in Teenage Girl

Japanese researchers reported a rare case of idiopathic intracranial hypertension (IIH) accompanying the onset of systemic lupus erythematosus (SLE) in a patient. The study, “Bilateral Abducens Nerve Palsy due to Idiopathic Intracranial Hypertension as an Initial Manifestation of Systemic Lupus Erythematosus,” was published in Internal Medicine.

IIH is a syndrome characterized by elevated intracranial pressure and cerebrospinal fluid pressure, which induces chronic headaches, vomiting, and ophthalmologic problems. IIH mainly emerges in young and overweight women, but can also result from infection, medication, or medical conditions like vitamin deficiency, endocrinological disease, and autoimmune inflammatory diseases like SLE.

In the case, a 14-year-old girl was admitted to a hospital for elevated fever lasting several days. The patient underwent screening for possible viral infection, ophthalmological abnormalities, and other diseases.

Initially, the hypothesis of IIH was ruled out, because the patient was not receiving any corticosteroids, antibiotics, or supplements like vitamin A, all known to be risk factors for IIH. Furthermore, she did not complain of headaches or nausea, symptoms present in almost all IIH cases. She also was not obese, and did not have cranial neuropathy, motor or sensory neuron disorders, pathological reflexes, or evidence of active viral infection — all of which would raise the odds of IIH. Brain MRI scans were negative for ischemia, thrombosis or vasculitis.

Cerebrospinal fluid (CSF) analysis, however, revealed extremely elevated opening pressure, indicating that the patient suffered from IIH, causing bilateral incomplete abducens nerve palsy with papilledema — or, optic disc swelling caused by increased intracranial pressure.

A definitive SLE diagnosis was performed according to the American College of Rheumatology (ACR) SLE classification criteria, and confirmed through the slightly increased levels of the inflammatory proteins and the anti-inflammatory antibodies found in her CSF. The patient was then successfully treated using intravenously and orally administrated corticosteroids.

“We experienced a case of SLE with bilateral abducens nerve palsies due to IIH. The presence of IIH in SLE patients is rare, but this potential occurrence should not be overlooked,” the researchers concluded. “Therefore, the present case had to be carefully distinguished from cranial nerve palsy in the differential diagnosis.”