A Case of Chronic Relapsing Inflammatory Optic Neuropathy

Steven Toh ◽  
Chean Chung Shen

Chronic relapsing inflammatory optic neuropathy (CRION) is a recently described form of recurrent isolated subacute optic neuropathy, with accumulating evidence that it is a nosological distinct entity. The condition is highly responsive to systemic steroid treatment and prone to relapse on steroid withdrawal. Diagnosis and management of this condition is often challenging. This 33-year-old lady with family history of multiple sclerosis (MS), with uniocular visual loss of her right eye since 2 years old without apparent cause, presented with reduction of vision and loss of colour vision in the left eye, associated with painful eye movement. There was internuclear ophthalmoplegia but slit lamp examination were unremarkable. She had no other related sensory or motor symptoms. Magnetic resonance imaging (MRI) did not reveal any features of MS. Aquaporin-4 antibody, anti-MOG and gene testing for Leber’s hereditary optic neuropathy were all negative. Metabolic, infective, and other autoimmune causes were also excluded. Visual evoked potential studies of left eye showed a mild reduction in amplitude with no prolongation of latency. Her multiple optic neuritis recurrences were treated with intravenous steroids followed by tapering regime of oral prednisolone with good effect. Knowledge of this rare condition as part of the differential diagnoses of possible aetiologies of optic neuropathy is important among Ophthalmologists, as prompt diagnosis and steroid treatment helped reduce the associated risk of blindness. Multiple relapses after initial successful treatment of inflammatory optic neuropathy should raise the suspicion of CRION.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S19

2011 ◽  
Vol 17 (7) ◽  
pp. 885-887 ◽  
Koji Shinoda ◽  
Takuya Matsushita ◽  
Konosuke Furuta ◽  
Noriko Isobe ◽  
Tomomi Yonekawa ◽  

This report describes, for the first time, an occurrence of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) in a 19-year-old female with neuromyelitis optica (NMO) spectrum disorder, who had anti-aquaporin-4 (AQP4) antibody. A high signal intensity lesion on T2-weighted MRI was detected in the midbrain tegmentum adjacent to the aqueduct, and presumably involved the medial longitudinal fasciculus bilaterally at the caudal levels. Plasma exchange resolved both WEBINO syndrome and the midbrain lesion. Although WEBINO syndrome is occasionally reported in multiple sclerosis patients, diagnosis of NMO should not be excluded in patients with WEBINO syndrome, because AQP4 is expressed abundantly around the periaqueductal region.

2007 ◽  
Vol 17 (4) ◽  
pp. 683-684 ◽  
F. Sáenz-Francés ◽  
C. Calvo-González ◽  
M. Jiménez-Santos ◽  
C. Méndez-Hernández ◽  
A.M. Fernandez-Vidal ◽  

Purpose To report a case of herpetic optic neuritis associated with herpetic keratitis. Methods A 65 year old woman presented with oedema in the nasal sector of his right papilla. Blood biochemistry, a haemogram, erythrocyte sedimentation rate and C-reactive protein were all normal. The patient was diagnosed as having a non-arteritic anterior ischaemic optic neuropathy. One week later slit lamp examination showed diffuse stromal corneal oedema and a dendritic lesion in the nasal zone of the corneal epithelium. Results Serology for varicela-zoster virus was positive. Treatment was started with valacyclovir given orally and topical acyclovir ointment. A week later, the optic disc swelling and corneal lesions had resolved. Conclusions The precise mechanism through which the papilla and cornea were successively affected in our patient is unclear but the sensitive innervation of both these structures is provided by the nasal branch of the nasociliary nerve and the spread of herpes via this nerve could affect both sites.

2013 ◽  
Vol 19 (12) ◽  
pp. 1618-1626 ◽  
Laurence Jasse ◽  
Sandra Vukusic ◽  
Françoise Durand-Dubief ◽  
Cristina Vartin ◽  
Carolina Piras ◽  

Objective: The objective of this article is to evaluate in multiple sclerosis (MS) patients the prevalence of persistent complaints of visual disturbances and the mechanisms and resulting functional disability of persistent visual complaints (PVCs). Methods: Firstly, the prevalence of PVCs was calculated in 303 MS patients. MS-related data of patients with or without PVCs were compared. Secondly, 70 patients with PVCs performed an extensive neuro-ophthalmologic assessment and a vision-related quality of life questionnaire, the National Eye Institute Visual Functionary Questionnaire (NEI-VFQ-25). Results: PVCs were reported in 105 MS patients (34.6%). Patients with PVCs had more frequently primary progressive MS (30.5% vs 13.6%) and more neuro-ophthalmologic relapses (1.97 vs 1.36) than patients without PVCs. In the mechanisms/disability study, an afferent visual and an ocular-motor pathways dysfunction were respectively diagnosed in 41 and 59 patients, mostly related to bilateral optic neuropathy and bilateral internuclear ophthalmoplegia. The NEI-VFQ 25 score was poor and significantly correlated with the number of impaired neuro-ophthalmologic tests. Conclusion: Our study emphasizes the high prevalence of PVC in MS patients. Regarding the nature of neuro-ophthalmologic deficit, our results suggest that persistent optic neuropathy, as part of the progressive evolution of the disease, is not rare. We also demonstrate that isolated ocular motor dysfunctions induce visual disability in daily life.

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Masaomi Kubota ◽  
Nobumasa Tamura ◽  
Takaaki Hayashi ◽  
Euido Nishijima ◽  
Haruhiko Yanagisawa ◽  

Treatments for paraneoplastic optic neuropathy (PON), a tumor-related autoimmune disease, include immunosuppression, plasma exchange, and immunoglobulin therapies, as well as treatment of the underlying disease. Herein, we describe the clinical course of an older adult patient with PON whose loss of vision improved after switching between epidermal growth factor receptor-tyrosine kinase inhibitor (EGFR-TKI) treatments for cancer. A 76-year-old woman, who had been treated with gefitinib for lung adenocarcinoma for two years, presented with acute bilateral visual disturbances. Her decimal best-corrected visual acuity (BCVA) was 0.3 in the right eye (RE) and 0.7 in the left eye (LE). Slit-lamp examination and funduscopy showed no abnormal findings. Two weeks later, her BCVA decreased to 0.2 in the RE and 0.01 in the LE. Goldman’s perimetry showed a defect in the lower nasal RE and extensive visual-field loss in the LE. Single-flash electroretinograms showed normal amplitudes. Magnetic resonance imaging revealed left optic neuritis and showed neither metastatic cancer nor multiple sclerosis. Pattern-reversal visual evoked potentials showed decreased P100 amplitudes in both eyes (BE). Based on a diagnosis of PON from clinical findings, methylprednisolone pulse treatment was administered. However, her BCVA became no light perception in BE two months after the first visit. Because the tumor tissue was found to be positive for the EGFR T790M resistance mutation by bronchoscopy, the EGFR-TKI treatment was changed to osimertinib, decreasing the size of the lung cancer lesions. Her BCVA improved to hand motion in BE. Her final BCVA was 0.01 in the RE, counting fingers 10 cm in the LE. She died at the age of 79 years. To our knowledge, no reports have shown improvement in BCVA in patients with PON after changing EGFR-TKI treatments. This report indicates that some patients may develop severe visual dysfunction without early treatment for the primary tumor.

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Sasitorn Siritho ◽  
Weerachai Tantinikorn ◽  
Paithoon Wichiwaniwate ◽  
Krit Pongpirul

A 59-year-old male who presented with a nonspecific headache at the vertex, resembling retrobulbar optic neuritis, was treated as such but did not show any improvement. Although optic nerve compression from sphenoid mucocele was finally discovered, the delayed diagnosis and improper treatment led to a permanent visual loss. Optic neuritis could be caused by a common problem, “mucocele/sinusitis,” but might be easily overlooked in general practice. Rhinogenic optic neuropathy should, therefore, be considered in every case of optic neuritis whenever atypical presentation occurs or is unresponsive to high-dose steroid treatment.

2018 ◽  
Vol 103 (6) ◽  
pp. 797-801 ◽  
Quangang Xu ◽  
Wenjuan Du ◽  
Huanfen Zhou ◽  
Xin Zhang ◽  
Hongjuan Liu ◽  

ObjectiveParaneoplastic optic neuropathy (PON) is relatively uncommon, and the visual outcomes and prognosis of this disease have not been well documented. The aim of this study was to investigate the clinical features and prognosis of antibody-mediated PON.MethodsClinical data were retrospectively collected from hospitalised patients diagnosed with PON at the Neuro-Ophthalmology Department at the Chinese People’s Liberation Army General Hospital from January 2015 to June 2017.ResultsA total of seven patients (four females and three males, 13 involved eyes) were included with a mean age of 56.28±11.32 years (36–70 years). Simultaneous or early sequential bilateral eye involvement (5/7, 71.4%) was common in the patients with PON. Severe vision loss (≤0.1) was seen in 76.9% (10/13) of the eyes. There were 13 eyes in the acute phase of the disease, and six eyes presented with optic disc oedema. All patients had definite evidence of paraneoplastic-associated antibodies (three with serum positive for antiamphilphysin, one for anti-PNMA2 (Ma2/Ta), one for anti-Yo, one for anti-Ma2 and one for anti-CV2). All of the serum samples were negative for myelin oligodendrocyte glycoprotein antibody and two patients companied with seropositive for the aquaporin-4 antibody. Five patients had history of primary malignancy, including thyroid cancer, type B thymoma, testicular seminoma, cervical cancer and lung carcinoma. Two patients had positive paraneoplastic syndrome antibodies (anti-Yo and antiamphiphysin), but the solid tumour had not been found through a PET scan. Visual acuity in 9/13 (69.2%) eyes was below 0.1, and all of the patients survived to the follow-up with no metastatic lesions.ConclusionsPON is relative rare, with a predominance of bilateral involvement and more with a poor visual prognosis. Paraneoplastic antibody testing can contribute to the diagnosis of PON, distinct from other types of optic neuropathies, which can help doctors to find the primary cancer earlier to guide further treatment.

2008 ◽  
Vol 17 (04) ◽  
pp. 193-196 ◽  
Robert D Steigerwalt ◽  
M Rosaria Cesarone ◽  
Gianni Belcaro ◽  
Antonella Pascarella ◽  
Mauro De Angelis ◽  

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