Introduction:
Idiopathic Intracranial Hypertension (IIH) or Pseudotumor Cerebri (PTC) is a neurological disorder characterized by markedly elevated intracranial pressures in the absence of an intracranial lesion, inflammation, or obstruction. Secondary IgA nephropathy has been reported in patients with liver cirrhosis, celiac disease, rheumatoid arthritis, and ankylosing spondylitis. However neurological association with IgA nephropathy is rare. Here we present a young non obese patient initially diagnosed with IIH subsequently found to have proteinuric IgA nephropathy.
Methods:
31 year old female with a Body Mass Index (BMI) of 27kg/m2 presented with history of intermittent headache for 3 months with increasing severity over the preceding 7 days. She was not on any oral medications and blood pressure was 130/70. Fundus examination done showed grade 4 papilledema. CT Brain was unremarkable. MRI brain showed dilated pre optic nerve sheath and increased optic nerve diameter. Lumbar puncture subsequently done showed elevated opening pressure of 330 mm of Hg with cytology and biochemistry analysis of Cerebrospinal fluid (CSF) unremarkable. On routine investigation she was found to have 3+ protein on urine routine analysis with quantification of proteinuria as 1.2 gm/day. Kidney Function Test was within normal limits. Immunological workup including Anti Nuclear Antibody by Indirect Immunofluorescence, complement assays, Extractable Nuclear Antigen (ENA) profile was normal. She underwent an uncomplicated renal biopsy which showed IgA nephropathy with Oxford MEST scores M1E0S1T0C0. She was treated with intravenous mannitol and subsequently acetazolamide and also started on Telmisartan 40 mg once a day. She improved symptomatically and was discharged and on follow up after 1 month her proteinuria had decreased to 650 mg/day.
Results:
hile there are numerous conditions and medications which have been associated with pseudotumor cerebri, case reports of its occurrence in patients with kidney disease are limited. Till date there has been only one case report of IIH associated with IgA nephropathy. But the presentation was more severe with rapid progression to dialysis requiring End Stage Renal Disease. To the best of our knowledge this is the first case of proteinuric presentation of IgA nephropathy in association with IIH in a non-obese individual. The mechanism behind this association is unclear but hormonal and metabolic factors such as a hypothalamic leptin-resistance can play a role in this pathophysiological link.
Conclusions:
We reported a temporal coincidence of IIH and proteinuric IgA nephropathy. We recommend clinical awareness and further systematic research to obtain more information on this association. . Our findings come with therapeutic consequences as a reduction of NSAID prescription to treat IIH associated headache or use of acetazolamide or topiramate with caution in these patients since it can cause a subsequent reduction of Glomerular Filtration Rate (GFR) in these proteinuric patients.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.