J Korean Med Sci.  2013 Nov;28(11):1690-1696. 10.3346/jkms.2013.28.11.1690.

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

• Kim SH
• Park J
• Bae JH
• Cho MS
• Park KD
• Jeong JH

• KMID: 1777672
• DOI: http://doi.org/10.3346/jkms.2013.28.11.1690

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.

Keyword

Wegener Granulomatosis; Cranial Nerve Diseases; Refractory Sinusitis; c-ANCA Negative

MeSH Terms

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
Anti-Inflammatory Agents
Antibodies, Antineutrophil Cytoplasmic
Antineoplastic Agents, Alkylating
Cyclophosphamide
Prednisolone

Figure

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  Fig. 1 Brain MRI. (A to D) The first MRI. Initial outside brain MRI was not remarkable, except bilateral mastoiditis and extensive sinusitis. (E to H) The second MRI. Repeated brain MRI after 5 months shows new meningeal thickening with enhancement around the tentorium cerebelli, inferior aspect of frontotemporal lobe adjacent to sinuses and nasopharynx. Also diffuse sinusitis in bilateral frontal, ethmoid, sphenoid, and maxillary sinuses were still existed.

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  Fig. 2 Neck CT. (A to C) Quite extensive sinusitis expanding to orbit and retromaxillary fat pad is observed. Note that massive swollen lesions of nasopharynx with positive enhancement penetrated the skull base.

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  Fig. 3 18F-FDG PET. (A to C) Prominent hypermetabolism in bilateral tonsil area of nasopharynx and multiple lymph nodes of neck level II and III were shown. (D-F) Multiple sinusitis were also demonstrated as hypermetabolic lesions. 18F-FDG PET: 18F-fluorodeoxyglucose positron-emission tomography.

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  Fig. 4 Nasopharygenal biopsy. (A) The nasopharyngeal tissue shows dense polymorphous inflammatory cell infiltrates composed of mature lymphocytes, plasma cells, histiocytes, eosinophils and neutrophils. There are few multinucleated giant cells which represent granulomatous component of the disease. (B) Granulomatous inflammation involving vessel wall and lumen (vasculitis) with necrosis is evident.

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