Article Text
PDFNeurological rarities
IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension
Abstract
Hypertrophic pachymeningitis secondary to IgG4-related disease is a rare but sometimes devastating cause of intracranial hypertension. It has the potential for an excellent response to corticosteroids or rituximab. We discuss the clinical presentation, imaging, histology (with its difficult distinction from lymphoma), management and follow-up of a case, including relapse and re-treatment following an initial response to rituximab.
- IgG4-Related Disease
- Hypertrophic Pachymeningitis
- Intracranial Hypertension
- Rituximab
Statistics from Altmetric.com
Linked Articles
- Editors' commentary
Read the full text or download the PDF:
Other content recommended for you
- A nationwide survey of hypertrophic pachymeningitis in Japan
- Rituximab for corticosteroid-resistant relapsing IgG4-related intracranial pachymeningitis: report of two cases
- Advances in the diagnosis and management of IgG4 related disease
- IgG4-related disease presenting with multiorgan involvement
- Pathological findings of hypertrophic pachymeningitis associated with eosinophilic granulomatosis with polyangiitis
- Clinicopathological case: headache and systemic inflammatory illness—Occam vs Hickam
- The enigmatic immunoglobulin G4-related disease and its varied cardiovascular manifestations
- Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations
- Hypertrophic pachymeningoencephalitis associated with temporal giant cell arteritis
- A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase