ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery
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...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Otolaryngology |
| Online Access: | http://dx.doi.org/10.1155/2017/9687383 |
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| author | Shohei Harabuchi Nobuyuki Bandoh Rika Yasukawa Michihisa Kono Takashi Goto Yasuaki Harabuchi Hidetoshi Ikeda Hajime Kamada Hiroshi Nishihara |
| author_facet | Shohei Harabuchi Nobuyuki Bandoh Rika Yasukawa Michihisa Kono Takashi Goto Yasuaki Harabuchi Hidetoshi Ikeda Hajime Kamada Hiroshi Nishihara |
| author_sort | Shohei Harabuchi |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-371983bc31d54e40abe96dc3b481cfd3 |
| institution | OA Journals |
| issn | 2090-6765 2090-6773 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Otolaryngology |
| spelling | doaj-art-371983bc31d54e40abe96dc3b481cfd32025-08-20T02:07:45ZengWileyCase Reports in Otolaryngology2090-67652090-67732017-01-01201710.1155/2017/96873839687383ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid ArteryShohei Harabuchi0Nobuyuki Bandoh1Rika Yasukawa2Michihisa Kono3Takashi Goto4Yasuaki Harabuchi5Hidetoshi Ikeda6Hajime Kamada7Hiroshi Nishihara8Department of Otolaryngology-Head and Neck Surgery, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanDepartment of Otolaryngology-Head and Neck Surgery, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanDepartment of Otolaryngology-Head and Neck Surgery, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanDepartment of Otolaryngology-Head and Neck Surgery, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanDepartment of Otolaryngology-Head and Neck Surgery, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanDepartment of Otolaryngology-Head and Neck Surgery, Asahikawa Medical University, Midorigaoka-Higashi 2-1-1-1, Asahikawa 078-8510, JapanDepartment of Neurosurgery, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanDepartment of Neurosurgery, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanDepartment of Biology and Genetics, Laboratory of Cancer Medical Science, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, JapanWe 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.http://dx.doi.org/10.1155/2017/9687383 |
| spellingShingle | Shohei Harabuchi Nobuyuki Bandoh Rika Yasukawa Michihisa Kono Takashi Goto Yasuaki Harabuchi Hidetoshi Ikeda Hajime Kamada Hiroshi Nishihara ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery Case Reports in Otolaryngology |
| title | ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery |
| title_full | ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery |
| title_fullStr | ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery |
| title_full_unstemmed | ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery |
| title_short | ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery |
| title_sort | anca negative granulomatosis with polyangiitis presenting with hypertrophic cranial pachymeningitis abducens nerve palsy and stenosis of the internal carotid artery |
| url | http://dx.doi.org/10.1155/2017/9687383 |
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