Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient
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CASE REPORT
Open Access
Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient Chaoyi Feng1, Qian Chen1, Wei Liu1, Yingwen Bi2, Jiang Qian1, Min Wang1, Xinghuai Sun1,3 and Guohong Tian1,3*
Abstract Background: Neurosarcoidosis is a rare systemic disorder that can affect the eye and other organs, including the central nervous system. Neurosarcoidosis infiltrating the optic nerve presenting as central retinal vein occlusion combined with artery ischaemia has not been reported in the literature previously. We describe a Chinese patient presenting with acute monocular vision loss, in whom an optic nerve biopsy confirmed the diagnosis of neurosarcoidosis. Case presentation: A 47-year-old woman complained of acute decreased vision in her left eye over the course of 1 month. She reported that her vision deteriorated quickly within first 3 days of consulting an ophthalmologist at a local hospital. She was diagnosed with central retinal vein occlusion after funduscopic examination and fundus fluorescein angiography, and the vision in her left eye further deteriorated to no light perception. An orbital magnetic resonance imaging showed an abnormal T1-weighted image of the optic nerve after contrast enhancement. She was referred to a neuro-ophthalmologist for further evaluation. After routine blood tests ruled out infectious and metastatic diseases, she was prescribed 500 mg/d methylprednisolone for 5 days, but her vision did not improve. As she could still not perceive light, an optic nerve biopsy was performed, and the histopathology revealed non-necrotising granuloma that was consistent with neurosarcoidosis. Conclusions: Isolated optic nerve infiltration by neurosarcoidosis without the involvement of the central nervous system or other systemic organs is challenging to diagnose. Biopsy of the optic nerve sheath is crucial for the final diagnosis of neurosarcoidosis. Therefore, a comprehensive ophthalmologic and systemic examination and work-up for inflammation of the eye, chest, and central nervous system should be conducted for atypical cases. Keywords: Optic neuropathy, Neurosarcoidosis, Retinal vein occlusion, Optic nerve biopsy
Background Neurosarcoidosis is very rare, but the most common neuro-ophthalmic manifestation is optic neuropathy [1– 3]. Diagnosing neurosarcoidosis without the involvement of other organs is very challenging. Due to its unspecific clinical manifestation and laboratory findings, * Correspondence: [email protected] 1 Department of Ophthalmology, Eye Ear Nose and Throat Hospital, Fudan University, 83 Fenyang Road, Shanghai 200031, China 3 NHC Key Laboratory of Myopia (Fudan University), Key Laboratory of Visual Impairment and Restoration, Shanghai, China Full list of author information is available at the end of the article
neurosarcoidosis can masquerade as optic neuritis, optic nerve sheath meningioma, a metastatic tumour, or a variety of other issues [4–6]. We describe a Chinese middle-aged woman who presented with monocular central retin
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