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ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies

Title
ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies
Authors
Kim, Sung-HeePark, JinBae, Jung HoCho, Min-SunPark, Kee DukJeong, Jee Hyang
Ewha Authors
박기덕조민선배정호정지향
SCOPUS Author ID
박기덕scopus; 조민선scopus; 배정호scopus; 정지향scopus
Issue Date
2013
Journal Title
JOURNAL OF KOREAN MEDICAL SCIENCE
ISSN
1011-8934JCR Link
Citation
vol. 28, no. 11, pp. 1690 - 1696
Keywords
Wegener GranulomatosisCranial Nerve DiseasesRefractory Sinusitisc-ANCA Negative
Publisher
KOREAN ACAD MEDICAL SCIENCES
Indexed
SCI; SCIE; SCOPUS; KCI WOS scopus
Abstract
Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.
DOI
10.3346/jkms.2013.28.11.1690
Appears in Collections:
의학전문대학원 > 의학과 > Journal papers
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