Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Optic neuropathy from connected intra- and extraorbital lesions in IgG4-related disease

Tsuneaki Yoshinaga, M.D., Ph.D.1)2), Toru Kurokawa, M.D., Ph.D.3), Takeshi Uehara, M.D., Ph.D.4), Junpei Nitta, M.D., Ph.D.5), Tetsuyoshi Horiuchi, M.D., Ph.D.6) and Yoshiki Sekijima, M.D., Ph.D.1)2)

Corresponding author: Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine[3-1-1 Asahi, Matsumoto 390-8621, Japan]
1)Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
2)Department of NeuroHealth Innovation, Institute for Biomedical Sciences, Shinshu University
3)Department of Ophthalmology, Shinshu University Hospital
4)Department of Biomedical Laboratory Medicine, Shinshu University Hospital
5)Department of Neurosurgery, Kobayashi Neurosurgical Hospital
6)Department of Neurosurgery, Shinshu University Hospital

We present the case of a 74-year-old woman complaining of blurred vision in the left eye who was found to have a unilateral, continuous lesion of the optic nerve and nerve sheath accompanied by an intracranial mass next to the cavernous sinus and meninges. Surgical decompression of the left optic nerve in the optic canal and partial resection of the mass followed by prednisolone administration were successful. Immunohistochemical analysis disclosed abundant infiltration of IgG4-positive plasma cells at >10 cells/high power field. These findings indicated a new pattern of compressive optic neuropathy with confirmed IgG4 histopathological findings. Such an extensive lesion may produce visual disturbance.
Full Text of this Article in Japanese PDF (3599K)

(CLINICA NEUROL, 59: 746|751, 2019)
key words: IgG4-related disease, IgG4-opthalmic disease, IgG4-related hypertrophic pachymeningitis, optic neuropathy

(Received: 9-Jul-19)