RT Journal Article
SR Electronic
T1 IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP 235
OP 239
DO 10.1136/practneurol-2015-001275
VO 16
IS 3
A1 Thomas Williams
A1 Monica Marta
A1 Gavin Giovannoni
YR 2016
UL http://pn.bmj.com/content/16/3/235.abstract
AB 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.