ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies.
10.3346/jkms.2013.28.11.1690
- Author:
Sung Hee KIM
1
;
Jin PARK
;
Jung Ho BAE
;
Min Sun CHO
;
Kee Duk PARK
;
Jee Hyang JEONG
Author Information
1. Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, Korea. jjeong@ewha.ac.kr
- Publication Type:Case Reports ; Research Support, Non-U.S. Gov't
- Keywords:
Wegener Granulomatosis;
Cranial Nerve Diseases;
Refractory Sinusitis;
c-ANCA Negative
- MeSH:
Anti-Inflammatory Agents/therapeutic use;
Antibodies, Antineutrophil Cytoplasmic;
Antineoplastic Agents, Alkylating/therapeutic use;
Cranial Nerve Diseases/*diagnosis/drug therapy/radionuclide imaging;
Cyclophosphamide/therapeutic use;
Diagnosis, Differential;
Female;
Humans;
Middle Aged;
Prednisolone/therapeutic use;
Sinusitis/surgery;
Vasculitis;
Wegener Granulomatosis/*diagnosis/drug therapy/radionuclide imaging
- From:Journal of Korean Medical Science
2013;28(11):1690-1696
- CountryRepublic of Korea
- Language:English
-
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.
