IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension

Published on Jun 1, 2016in Practical Neurology
· DOI :10.1136/practneurol-2015-001275
Thomas Williams2
Estimated H-index: 2
(QMUL: Queen Mary University of London),
Monica Marta18
Estimated H-index: 18
(QMUL: Queen Mary University of London),
Gavin Giovannoni72
Estimated H-index: 72
(QMUL: Queen Mary University of London)
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.
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