Document Type : Case Report
Biological-Molecular Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran
Department of Otorhinolaryngology, Faculty of Medicine, Baqiyatallah University of Medical Sciences, Tehran, Iran
Health Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran
Department of Rheumatology, Faculty of Medicine, Baqiyatallah University of Medical Sciences, Tehran, Iran
Introduction: Cranial nerve palsy in Wegener’s granulomatosis is a curious incident, particularly if it occurs without kidney or lung involvement. In a review of medical articles, only 1 case of Wegener’s granulomatosis with bilateral facial nerve palsy was found.
Case Presentation: The patient was a 16-year-old female who presented with pain and hearing loss in both ears and reduced muscle tone in the right side of her face. After myringotomy and ventilation tube embedding in both ears, she gradually developed infectious otorrhea in both ears. Despite antibiotic and antifungal therapy for 2 weeks after surgery, the patient developed paresis in the left facial nerve and gag reflex disorder. Both magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) tests were normal, but the C-anti-neutrophil cytoplasmic antibody (CANCA) test result was about forty times higher than normal, and a sinus biopsy also verified Wegener’s granulomatosis. The patient was then treated with pulse corticosteroid and cyclophosphamide therapy. Her gag reflex healed and the ear secretions stopped, but the facial nerve palsy continued.
Conclusion: Although cranial nerve involvement in Wegener’s granulomatosis is an unusual incidence, it may occur in some cases. In the current case, otitis media and cranial nerve paresis without kidney or lung disorders were the only signs of disease onset; of course, computerized tomography (CT) scan revealed a right mandibular sinus disorder without any clinical signs. Another important point in this case is that other identified Wegener’s patients were in their fourth or fifth decade of life; thus, Wegener’s was the last choice for a diagnosis in the current case.
- Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med. 2005;352(4):351-361. doi: 10.1056/NEJMoa041884.
- Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014;13(11):1121-1125. doi: 10.1016/j.autrev.2014.08.017.
- Bohm M, Gonzalez Fernandez MI, Ozen S, et al. Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis). Pediatr Rheumatol Online J. 2014;12:18. doi: 10.1186/1546-0096-12-18.
- Girard C, Charles P, Terrier B, et al. Tracheobronchial stenoses in granulomatosis with polyangiitis (Wegener’s): a report on 26 cases. Medicine (Baltimore). 2015;94(32):e1088. doi: 10.1097/MD.0000000000001088.
- Lutalo PM, D’Cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). J Autoimmun. 2014;48-49:94-98. doi: 10.1016/j.jaut.2014.01.028.
- Liu X, Cui Y, Li Y, Wang C, Zhao H, Han J. Using inpatient data to estimate the prevalence of Wegener’s granulomatosis in China. Intractable Rare Dis Res. 2016;5(1):31-35. doi: 10.5582/irdr.2015.01015.
- Tan LT, Davagnanam I, Isa H, et al. Clinical and imaging features predictive of orbital granulomatosis with polyangiitis and the risk of systemic involvement. Ophthalmology. 2014;121(6):1304-1309. doi: 10.1016/j.ophtha.2013.12.003.
- Guneyli S, Ceylan N, Bayraktaroglu S, et al. Imaging findings of pulmonary granulomatosis with polyangiitis (Wegener’s granulomatosis): lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy. Wien Klin Wochenschr. 2016;128(21-22):809-815. doi: 10.1007/s00508-015-0747-1.
- Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488- 498. doi: 10.7326/0003-4819-116-6-488.
- Nikolaou AC, Vlachtsis KC, Daniilidis MA, Petridis DG, Daniilidis IC. Wegener’s granulomatosis presenting with bilateral facial nerve palsy. Eur Arch Otorhinolaryngol. 2001;258(4):198-202. doi: 10.1007/s004050100327.