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This article is part of the supplement: Abstracts from the Combined Northern and Yorkshire Deaneries Rheumatology Annual Conference 2012

Open Access Meeting abstract

Granulomatosis with polyangitis presenting with multiple cranial nerve palsies

Muhammad Kazmi*, Mohammed Akil and Rachael Kilding

  • * Corresponding author: Muhammad Kazmi

Author affiliations

Rheumatology Department, Royal Hallamshire Hospital, Sheffield, UK

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Citation and License

BMC Musculoskeletal Disorders 2013, 14(Suppl 1):A11  doi:10.1186/1471-2474-14-S1-A11


The electronic version of this article is the complete one and can be found online at: http://www.biomedcentral.com/1471-2474/14/S1/A11


Published:14 February 2013

© 2013 Kazmi et al; licensee BioMed Central Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Background

Wegener's granulomatosis/granulomatosis with polyangitis classically affects the upper and lower respiratory tracts as well as renal system and lungs, while cranial nerves and meningeal involvement is rare and present in only 2% to 4% of cases.

Case presentation

We report the case of localised granulomatosis with polyangitis in a 27 year old Caucasian gentleman who presented with multiple cranial neuropathies. He reported diplopia, hearing loss, dysphagia and dysarthria due to involvement of VI, XIII, IX, X and XII cranial nerves. A cerebral MRI demonstrated pachymeningitis along with mastoid changes and additional diagnostic workup showed elevated CRP and C-ANCA (PR-3) antibodies but did not show any pulmonary infiltrates. Induction treatment with prednisolone, cyclophosphamide and rituximab followed by maintenance MMF resulted in complete resolution of his clinical features within six months.

Conclusion

This case describes this extremely rare presentation of GPA with multiple cranial palsies as initial manifestation, as only less than 100 similar cases are reported in literature worldwide. This case was a diagnostic challenge as there was no eye/para-nasal sinus/chest or renal involvement.