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• Vol.56 No.9
• CLINICA NEUROL, 56: 637−640, 2016
• Vol.56 No.9 contents

Brief Clinical Note

IgG4-related disease with cavernous sinus and intra-orbital lesions diagnosed by nasal mucosa biopsy

Ruka Nakata, M.D.¹⁾, Shunsuke Yoshimura, M.D.¹⁾, Masakatsu Motomura, M.D.¹⁾, Akira Tsujino, M.D.¹⁾, Tomayoshi Hayashi, M.D.²⁾ and Minoru Hara, M.D.³⁾

¹⁾Department of Clinical Neuroscience and Neurology, Nagasaki University Hospital
²⁾Department of Clinical Pathology, Nagasaki University Hospital
³⁾Department of Otolaryngology-Head and Neck Surgery, Nagasaki University Hospital

IgG4-related disease is a systemic disease characterized by lesions with IgG4 positive plasma cell infiltration in the involved organs and a raised serum IgG4 level. We report a patient of 70-year-old male presented orbital inflammation of IgG4-related disease. The patient developed right eye pain, double vision, and reduced eye sight. MRI image revealed mild right ocular proptosis and swelling of right carvenous sinus, bilateral intraorbital extraocular muscles and right optic nerve. Right optic nerve showed ring-like enhancement. IgG4-related disease was suspected with increased serum IgG4 level of 355 mg/dl, mediastinal lymphadenopathy and prostate enlargement. Transbronchial lung biopsy and prostate needle biopsy were administered with negative results. The eye related symptoms resolved with time, but serum IgG4 continuously increased. IgG4-related disease was diagnosed by nasal mucosa biopsy, which showed IgG4 positive plasma cells within the inflammatory infiltrate. This report emphasizes the usefulness of nasal mucosa biopsy for the diagnosis of IgG4 related disease with lesions difficult to approach.
Full Text of this Article in Japanese PDF (1276K)

(CLINICA NEUROL, 56: 637−640, 2016)
key words: IgG4-related disorder, optic neuritis, cavernous sinus syndrome, nasal mucosa biopsy

(Received: 29-Feb-16)