Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Harabuchi, Shohei, Bandoh, Nobuyuki, Yasukawa, Rika, Kono, Michihisa, Goto, Takashi, Harabuchi, Yasuaki, Ikeda, Hidetoshi, Kamada, Hajime, Nishihara, Hiroshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5733126/
https://www.ncbi.nlm.nih.gov/pubmed/29527371
http://dx.doi.org/10.1155/2017/9687383
_version_ 1783286844955295744
author Harabuchi, Shohei
Bandoh, Nobuyuki
Yasukawa, Rika
Kono, Michihisa
Goto, Takashi
Harabuchi, Yasuaki
Ikeda, Hidetoshi
Kamada, Hajime
Nishihara, Hiroshi
author_facet Harabuchi, Shohei
Bandoh, Nobuyuki
Yasukawa, Rika
Kono, Michihisa
Goto, Takashi
Harabuchi, Yasuaki
Ikeda, Hidetoshi
Kamada, Hajime
Nishihara, Hiroshi
author_sort Harabuchi, Shohei
collection PubMed
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 Online
Article
Text
id pubmed-5733126
institution National Center for Biotechnology Information
language English
publishDate 2017
publisher Hindawi
record_format MEDLINE/PubMed
spelling pubmed-57331262018-03-11 ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery Harabuchi, Shohei Bandoh, Nobuyuki Yasukawa, Rika Kono, Michihisa Goto, Takashi Harabuchi, Yasuaki Ikeda, Hidetoshi Kamada, Hajime Nishihara, Hiroshi Case Rep Otolaryngol Case Report 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. Hindawi 2017 2017-11-30 /pmc/articles/PMC5733126/ /pubmed/29527371 http://dx.doi.org/10.1155/2017/9687383 Text en Copyright © 2017 Shohei Harabuchi et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Harabuchi, Shohei
Bandoh, Nobuyuki
Yasukawa, Rika
Kono, Michihisa
Goto, Takashi
Harabuchi, Yasuaki
Ikeda, Hidetoshi
Kamada, Hajime
Nishihara, Hiroshi
ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery
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
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5733126/
https://www.ncbi.nlm.nih.gov/pubmed/29527371
http://dx.doi.org/10.1155/2017/9687383
work_keys_str_mv AT harabuchishohei ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT bandohnobuyuki ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT yasukawarika ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT konomichihisa ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT gototakashi ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT harabuchiyasuaki ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT ikedahidetoshi ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT kamadahajime ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery
AT nishiharahiroshi ancanegativegranulomatosiswithpolyangiitispresentingwithhypertrophiccranialpachymeningitisabducensnervepalsyandstenosisoftheinternalcarotidartery