Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017:2017:9687383.
doi: 10.1155/2017/9687383. Epub 2017 Nov 30.

ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery

Shohei Harabuchi  1   2 , Nobuyuki Bandoh  1 , Rika Yasukawa  1   2 , Michihisa Kono  1 , Takashi Goto  1 , Yasuaki Harabuchi  2 , Hidetoshi Ikeda  3 , Hajime Kamada  3 , Hiroshi Nishihara  4
Affiliations
Case Reports

ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery

Shohei Harabuchi et al. Case Rep Otolaryngol. 2017.

Abstract

We report a rare case of granulomatosis with polyangiitis (GPA) presenting with hypertrophic cranial pachymeningitis (HCP), abducens nerve palsy, and stenosis of the internal carotid artery (ICA). A 59-year-old Japanese man presented with a year history of nasal obstruction and a 2-month history of slight headache. Histopathological examination of the granulomatous mucosa in the ethmoid sinuses resected by endoscopic sinus surgery revealed necrotizing vasculitis with multinucleated giant cells. The patient was diagnosed with the limited form of GPA as a result of the systemic examination. He declined immunosuppressive treatment. Eighteen months after the diagnosis of GPA, he presented with diplopia and severe headache. Though nasal findings indicating GPA were not observed in the nasal cavity, CT scan revealed a lesion of the right sphenoid sinus eroding the bone of the clivus. Gadolinium-enhanced MRI of the brain showed thickening of the dura mater around the right cavernous sinus and clivus. Magnetic resonance angiography and cerebral angiography revealed narrowing at the C5 portion of the ICA. Intravenous methylprednisolone pulse therapy followed by oral prednisolone and cyclophosphamide resolved headache and dramatically improved HCP and stenosis of the ICA.

PubMed Disclaimer

Figures

Figure 1
Figure 1
CT scan at first visit shows swelling of the mucosa in the bilateral ethmoid sinuses (a) along with a soft-tissue area occupying the right sphenoid sinus (b).
Figure 2
Figure 2
During endoscopic sinus surgery, granulomatous mucosa is apparent in the right (a) and left (b) ethmoid sinuses.
Figure 3
Figure 3
Histopathological examination of the granulomatous mucosa from the ethmoid sinus shows significant necrotizing vasculitis (a) and granulomatous inflammation with multinucleated giant cells (b) (HE staining, ×200). MT: middle turbinate.
Figure 4
Figure 4
CT scan 18 months after being diagnosed with GPA shows a lesion of the right sphenoid sinus eroding the bone of the clivus and thickening of the dura mater on axial (a) and sagittal (b) images (triangle).
Figure 5
Figure 5
Imaging studies before immunosuppressive treatment (a–d) and 2 years after the initiation of the treatment (e–g). Gadolinium-enhanced T1-weighted MRI of the brain reveals thickening of enhanced dura mater extending from the cavernous sinus to the clivus on axial (a) and coronal (b) images (triangle). Narrowing at the C5 portion of the internal carotid artery (ICA) is apparent on axial (a) and coronal (b) MRI, MRA (c), and lateral cerebral angiography of the ICA (d) (arrow). Thickening of the dura mater and stenosis of the ICA improved on axial (e) and coronal (f) MRI and MRA (g).
See this image and copyright information in PMC

References

    1. Reinhold-Keller E., de Groot K., Holl-Ulrich K., et al. Severe CNS manifestations as the clinical hallmark in generalized Wegener’s granulomatosis consistently negative for antineutrophil cytoplasmic antibodies (ANCA). A report of 3 cases and a review of the literature. Clinical and Experimental Rheumatology. 2001;19(5):541–549. - PubMed
    1. Higuera-Ortiz V., Reynoso A., Ruiz N., Delgado-Hernández R. D., Gómez-Garza G., Flores-Suárez L. F. Pachymeningitis in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review. Clinical Rheumatology. 2017;36(4):919–924. doi: 10.1007/s10067-016-3520-6. - DOI - PubMed
    1. Fam A. G., Lavine E., Lee L., Perez-Ordonez B., Goyal M. Cranial pachymeningitis: an unusual manifestation of Wegener’s granulomatosis. Journal of Rheumatology. 2003;30(9):2070–2074. - PubMed
    1. Yokoseki A., Saji E., Arakawa M., et al. Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody. Brain. 2014;137(2):520–536. doi: 10.1093/brain/awt314. - DOI - PubMed
    1. Sivakumar M. R., Chandrakantan A. A rare case of stroke in Wegener’s granulomatosis. Cerebrovascular Diseases. 2002;13(2):143–144. doi: 10.1159/000047765. - DOI - PubMed

Publication types

LinkOut - more resources