Severe localised granulomatosis with polyangiitis (Wegener's granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review
Author(s)
Peters, James E
Gupta, Vivek
Saeed, Ibtisam T
Offiah, Curtis
Jawad, Ali SM
Type
Journal Article
Abstract
BACKGROUND: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarely, pituitary infiltration. CASE PRESENTATION: We describe the case of a 32 year-old woman with limited but severe GPA manifesting as progressive cranial nerve palsies and pituitary dysfunction. Our patient initially presented with localised ENT involvement, but despite treatment with methotrexate, she deteriorated. Granulomatous inflammatory tissue around the skull base resulted in cavernous sinus syndrome, facial nerve palsy, palsies of cranial nerves IX-XII (Collet-Sicard syndrome), and the rare complication of cranial diabetes insipidus due to pituitary infiltration. The glossopharyngeal, vagus and accessory nerve palsies resulted in severe dysphagia and she required nasogastric tube feeding. Her neurological deficits substantially improved with treatment including high dose corticosteroid, cyclophosphamide and rituximab. CONCLUSIONS: This case emphasises that serious morbidity can arise from localised cranial Wegener's granulomatosis in the absence of systemic disease. In such cases intensive induction immunosuppression is required. Analysis of previously reported cases of pituitary involvement in GPA reveals that this rare complication predominantly affects female patients.
Date Issued
2018-05-01
Date Acceptance
2018-04-20
Citation
BMC Neurology, 2018, 18 (1)
ISSN
1471-2377
Publisher
BioMed Central
Journal / Book Title
BMC Neurology
Volume
18
Issue
1
Copyright Statement
© The Author(s). 2018. This article is distributed under the terms of the Creative Commons Attribution 4.0International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, andreproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link tothe Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Sponsor
United Kingdom Research and Innovation
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/29716529
PII: 10.1186/s12883-018-1058-8
Grant Number
MR/S004068/1
Subjects
ANCA
Cavernous sinus syndrome
Collet-Sicard syndrome
Cyclophosphamide
Diabetes insipidus
Granulomatosis with polyangiitis
Pituitary
Rituximab
Vasculitis
Wegener’s granulomatosis
Adult
Cranial Nerve Diseases
Cyclophosphamide
Diabetes Insipidus, Neurogenic
Female
Granulomatosis with Polyangiitis
Humans
Methotrexate
Rituximab
Publication Status
Published
Coverage Spatial
England
Article Number
ARTN 59