Case series of idiopathic intracranial hypertension in three patients with immune-complex glomerulonephritis

BMC Neurol. 2021 Jul 13;21(1):278. doi: 10.1186/s12883-021-02297-3.

Abstract

Background: Idiopathic intracranial hypertension (IIH) is defined by an increased cerebrospinal fluid pressure in the absence of inflammation, structural obstructions, or mass lesions. Although the underlying pathogenesis of IIH is not fully understood, associations with specific risk factors as obesity, obstruction of cerebral venous sinuses, medications, endocrine or systemic conditions and chronic kidney disease have been described. Immune-complex glomerulonephritis as IgA-nephropathy is a frequent cause of chronic kidney failure, which was reported previously in one IIH patient. To date, there is no knowledge about the variable relation of immune-complex nephritis, kidney function and the course of IIH.

Case presentation: We report three cases (two females) of concurrent diagnosis of IIH and immune-complex glomerulonephritis. All patients presented with typical IIH symptoms of headache and visual disturbances. Two patients had been diagnosed with IgA-nephropathy only few weeks prior to IIH diagnosis. The third patient had been diagnosed earlier with terminal kidney failure due to a cryoglobulin glomerulonephritis.

Conclusion: We propose a possible link between renal deposition of immune-complexes and increased cerebrospinal fluid pressure. Pathophysiological hypotheses and clinical implications are discussed. We recommend clinical awareness and further systematic research to obtain more information on the association of IIH and immune-complex glomerulonephritis.

Keywords: Case report; Idiopathic intracranial hypertension; IgA nephropathy; Immune-complex glomerulonephritis; Nephropathy; Pseudotumor cerebri.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cranial Sinuses / diagnostic imaging
  • Female
  • Glomerulonephritis / complications*
  • Glomerulonephritis / diagnostic imaging*
  • Headache / diagnostic imaging
  • Headache / etiology
  • Humans
  • Immune Complex Diseases / complications*
  • Immune Complex Diseases / diagnostic imaging*
  • Male
  • Obesity / complications
  • Obesity / diagnostic imaging
  • Pseudotumor Cerebri / complications*
  • Pseudotumor Cerebri / diagnostic imaging*
  • Risk Factors
  • Vision Disorders / diagnostic imaging
  • Vision Disorders / etiology
  • Young Adult