Homonymous hemianopia and disc pallor after severe head injury

A woman with a history of severe right-sided head trauma at age four is seen at age 20 with temporal lobe seizures, decreased vision in her right eye without an afferent pupillary defect and with a total left homonymous hemianopia. In addition, she has bilateral optic atrophy, worse on the left side. Arguments are presented with respect to the cause of the decreased visual acuity and the asymmetric optic atrophy.     

Multicentric Glioblastoma Multiforme Mimicking Optic Neuritis

A 49-year-old previously healthy woman presented with acute painless visual loss in the right eye, a right relative afferent pupillary defect, and a normal fundus examination. She was diagnosed with retrobulbar “optic neuritis” and given a course of intravenous steroids. Despite treatment, however, she continued to lose vision and serial visual field testing confirmed a junctional scotoma in the fellow eye. Cranial magnetic resonance imaging (MRI) showed a mass at the junction between the right optic nerve and the anterior aspect of the chiasm and a right frontal lesion that proved to be multicentric glioblastoma multiforme. Clinicians should be aware of the possibility of aggressive neoplasm in the differential diagnosis of retrobulbar optic neuritis.     

Retinal Nerve Fiber Layer Thickness in Optic Tract Syndrome

Background Optic tract syndrome (OTS) is characterized by incongruous homonymous hemianopia and a perpendicular pattern of bilateral optic atrophy due to the optic tract lesion. However, loss of retinal nerve fiber layer thickness (RNFLT) associated with OTS has not been quantitatively assessed.Case A 20-year-old woman with blunt head trauma showed normal visual acuity, color vision, ocular motility, and intraocular pressure. Because of a relative afferent pupillary defect in her left eye and left-sided homonymous hemianopia, we suspected right-sided optic tract damage, although magnetic resonance imaging detected no intracranial lesion.Observations Using optical coherence tomography (OCT), the RNFLT of this case was measured at 31 months after the trauma and compared with age-matched normal controls (n = 41). Nasal, temporal, superior, and inferior quadrant RNFLT was reduced by 22%, 21%, 5%, and 46% in the right eye and 76%, 64%, 25%, and 27% in the left eye, respectively. The reduction was > 3 × the standard deviation of the normal mean values in the nasal and temporal quadrants of the left eye and in the inferior quadrant of the right eye.Conclusions OCT can determine the RNFLT reduction corresponding to the characteristic patterns of optic atrophy of OTS. Jpn J Ophthalmol 2005;49:294–296 © Japanese Ophthalmological Society 2005     

Shot in the dark

A 43-year-old woman presented with decreased vision in the right eye associated with painful eye movements 10 days after receiving her first dose of Pfizer-BioNTech coronavirus disease 2019 (COVID-19) vaccine (Pfizer Inc, New York, NY). Two days later she developed painful loss of vision in the left eye. Clinical presentation and magnetic resonance imaging findings were consistent with bilateral optic perineuritis transitioning to optic neuritis. Extensive evaluation including aquaporin-4 immunoglobin G (IgG), myelin oligodendrocyte glycoprotein IgG, and lumbar puncture was unrevealing. Visual acuity at nadir was counting fingers in both eyes, but after receiving intravenous steroids and plasma exchange vision eventually improved to 20/20 in each eye, although she was left with inferior visual field defects and bilateral optic disc pallor. This case highlights the diagnostic challenge in the evaluation of atypical optic neuritis with a review of post-COVID-19 vaccination-associated optic neuritis.     

Rapid, painless unilateral vision loss in a 37-year-old healthy woman

A 37-year-old woman experienced painless, progressive vision loss to no light perception in the left eye over the course of 3 days. The right eye was unaffected. On examination, the only other abnormal finding was a +4 left afferent pupillary defect. She was initially diagnosed with retrobulbar optic neuritis and admitted for treatment with intravenous methylprednisolone. Neuro-imaging revealed a large right anterior cerebral artery aneurysm that crossed the midline to compress the left optic nerve. The aneurysm was treated with coil embolization, which was technically successful but which did not lead to significant improvement in vision.     

Woman with atypical unilateral Leber's hereditary optic neuropathy with visual improvement

We describe a patient with Leber's hereditary optic neuropathy (LHON) who had a unilateral involvement and a gradual recovery of vision. A 50-year-old woman was referred to our clinic in December 2004 for the treatment of left optic neuritis. The visual acuity was 0.01 in her left eye and 1.5 in her right eye. The left eye had a central scotoma and a relative afferent pupillary defect. Ophthalmoscopy revealed a hyperaemic optic disc with indistinct margins in the left eye. Fluorescein angiography showed circumpapillary microangiopathy in both eyes and staining of the left optic disc. An nt 11778 mutation was identified and she was diagnosed with LHON. The central scotoma gradually improved, and the visual acuity had recovered to 0.3 in August 2007. LHON should still be considered even in older female patients presenting with unilateral acute visual loss when microangiopathy is seen. In such cases, molecular testing is effective in confirming a diagnosis of LHON.     

A Case of Decreased Visual Field after Uneventful Cataract Surgery: Nonarteritic Anterior Ischemic Optic Neuropathy

The purpose of this article is to report a case of nonarteritic anterior ischemic optic neuropathy (NAION) after uneventful cataract surgery. A 53-year-old Filipina underwent cataract surgery. She had a small optic disc with cup-to-disc ratio of 0.2 in the left eye and 0.3 in the right eye. On the first postoperative day, the uncorrected visual acuity (UCVA) was 20/20, with an intraocular pressure (IOP) of 20 mmHg in the left eye. At one week after operation, the UCVA was 20/20 and the IOP was 15 mmHg. Three weeks later, she underwent cataract surgery in the right eye. On the first postoperative day, her UCVA was 20/20 in both eyes, but she complained of a visual field decrease in the left eye. A relative afferent pupillary defect (RAPD) was noted and the optic disc was pallid and swollen diffusely. A red-free photo showed defect surrounding the optic disc. A visual field test showed tunnel vision sparing the central vision. In this report, the authors hypothesize an association between cataract extraction and delayed NAION. Since the risk of NAION in the fellow eye is 30-50%, visual acuity, visual field, fundus exam and RAPD should be routinely checked.     

Don't get off the track

A 43-year-old man noted decreased vision after head trauma, with normal neuroimaging acutely. He had a left homonymous hemianopia, confirmed with hemifield visual evoked potentials, from trauma to the right optic tract. Four months after trauma, a magnetic resonance imaging of the brain showed atrophy of the right optic tract, and funduscopy revealed optic disk pallor with decreased retinal nerve fiber layer measures consistent with an optic tract syndrome.     

Bilateral Homonymous Hemianopia with Sparing of the Vertical Meridian

A 19-year-old woman, who was 16 weeks post partum, collapsed with loss of consciousness. Following awakening she suffered abrupt loss of vision. Partial recovery occurred, although she has been left with bilateral homonymous hemianopia with sparing of vision just to the left of the vertical meridian. Magnetic resonance imaging demonstrated bilateral occipital infarcts, sparing the lips of the calcarine sulcus on the right. This is compatible with our expectation that the vertical meridian of the retinotopic map is represented at the superior and inferior lips of the calcarine sulcus.KEYWORDS: bilateral homonymous hemianopia, MRI, occipital infarction, vertical meridian sparing     

Leber's hereditary optic neuropathy masquerading as optic neuritis with spontaneous visual recovery

We report a case of Leber's hereditary optic neuropathy (LHON) masquerading as optic neuritis with late visual recovery. A 28‐year‐old man had gradual visual loss in both eyes for two weeks. Visual acuity was 0.4 in the right eye and 0.7 in the left. Fundus examination revealed hyperaemic discs in each eye. Fluorescein angiography revealed dye leakage at both optic discs in the late phase. Static perimetry (Humphrey 30‐2) revealed bilateral relative central scotomata. Magnetic resonance imaging of the optic nerves was normal and his lumbar puncture showed normal opening pressure. He received steroid pulse therapy for three days. Nevertheless, vision in his right eye deteriorated to 0.1 one month later and left vision worsened to 0.05 six months later. Fifteen months after onset, his vision began to improve. At 21 months, his vision recovered to 0.9 R and 1.0 L. Peripheral blood DNA sequencing revealed 14484 mutation of mitochondrial DNA (mtDNA). Visual recovery can occur in patients with Leber's hereditary optic neuropathy with mtDNA 14484 mutation. LHON could be misdiagnosed as optic neuritis in some cases. Molecular examination of mtDNA mutation can confirm the diagnosis of LHON in clinically controversial patients. We should keep in mind the diagnosis of LHON when optic neuritis shows poor response to pulse therapy.     

Optic neuritis with multiple sclerosis in a 10-year-old Asian girl

AIM: To report a case of unilateral optic neuritis with multiple sclerosis(MS) in an Asian child. · METHODS: A case report. · RESULTS: A 10-year-old Chinese girl presented with history of sudden loss of vision of the right eye for 3 days. It was associated with pain in eye movement. She gave history of fever associated with ataxic gait a year ago. She had been diagnosed with acute disseminated encephalomyelitis (ADEM). Visual acuity in the right eye was 'counting finger' with positive afferent pupillary defect. The optic disc was swollen and hyperemic. The colour vision was severely impaired. Visual field showed central scotoma and enlarged blind spot. Magnetic resonance imaging (MRI) of the brain revealed multiple intense lesions in the left occipital lobe, basal ganglia and periventricular regions suggesting MS. She was treated with intravenous methylprednisolone for 3 days, followed by oral prednisolone for 11 days. She had excellent recovery and her visual acuity improved to 6/9. She remained asymptomatic for 3 years. · CONCLUSION: The simultaneous occurrence of optic neuritis and MS is less common in children and seldom reported. We presented this case to highlight the possibility of this disease occurring in Asian population in a younger age group.     

亚洲女童视网膜炎合并多发性硬化1例

目的:报道亚洲儿童单侧视神经炎合并多发性硬化1例方法:病例报道结果:中国女孩10岁,右眼视力突然持续下降3d,伴有眼球运动痛。患者1a前曾有高热并伴有步态共济失调病史。当时被诊断为急性弥散性脑脊髓炎,右眼视力数指,伴有瞳孔传入阻滞,视盘充血水肿,色觉功能受损严重。视野检查显示中心暗点及盲点扩大。脑部磁共振MRI结果显示大脑左侧枕叶有较高密度影损害,神经中枢基部和脑室周围的区域提示多发性硬化。给予患者甲基强的松龙静脉滴注3d,以后改为口服强的松龙11d。患者出现明显好转,视力提高到6/9,并且维持3a没有复发结论:视神经炎合并多发性硬化在儿童中比较罕见且鲜有报道。我们报道这个病例就是为了强调这个疾病在亚洲青少年人群出现的可能性。     

A congruous superior quadrantanopsia following a junctional scotoma induced by asperogillosis

A 69-year old man presented to us with decreased vision in his right eye and a relative afferent pupillary defect. Under the presumption that he was suffering from retrobulbar optic neuritis or ischemic optic neuropathy, visual field tests were performed, revealing the presence of a junctional scotoma. Imaging studies revealed tumorous lesions extending from the sphenoid sinus at the right superior orbital fissure, with erosion of the right medial orbital wall and optic canal. Right optic nerve decompression was performed via an endoscopic sphenoidectomy, and histopathologic examination confirmed the presence of aspergillosis. The patient did not receive any postoperative antifungal treatment; however, his vision improved to 20 / 40, and his visual field developed a left congruous superior quadrantanopsia 18 months postoperatively. A junctional scotoma can be caused by aspergillosis, demonstrating the importance of examining the asymptomatic eye when a patient is experiencing a loss of vision in one eye. Furthermore, damage to the distal optic nerve adjacent to the proximal optic chiasm can induce unusual congruous superior quadrantanopsia.     

Bilateral optic neuritis in a patient with Beh?et’s disease who respond to therapeutic plasma exchange

Bilateral optic neuritis has been reported very rarely as a manifestation of neuro-Behçet’s disease. We present a 50 year old woman who had 20-year history of Behçet’s disease presented with acutely blurred vision associated with orbital pain in both eyes. Visual acuity was 0.4 in the right eye and light perception in the left eye; afferent pupillary defect was detected in the left eye. Bilateral swelling of the optic disk was found. The cerebrospinal fluid sample tests were within normal limits. Brain magnetic resonance imaging, magnetic resonance venography and fundus fluorescein angiography were normal. She was diagnosed with bilateral optic neuritis and treated with intravenous methyl prednisolone for 10 days. As there was no response to the treatment, therapeutic plasma exchange was started and the patient’s visual acuities improved moderately. We suggest that when high dose steroid is failed to treat ON in BD, treatment with TPE may be considered.     

Evolutionary history of neuro-ophthalmology

An acute painful unilateral loss of vision in a young adult with obvious signs and symptoms of optic neuropathy usually suggests a diagnosis of optic neuritis. However, a similar clinical picture can be caused by a compressive lesion in the anterior visual pathways. Although such lesions are rare, they can only be diagnosed through neuroimaging studies.We describe a 37-year-old Chinese female, who presented with mild pain and decreased vision in the left eye two weeks prior to consultation. A diagnosis of retrobulbar optic neuritis was made and she underwent pulsed intravenous therapy of 1 g methyl-prednisolone daily over three days. The magnetic resonance imaging done three days later showed a partially thrombosed paraseller aneurysm, abutting and displacing the optic chiasm laterally. She underwent a right temporal craniotomy and clipping of the aneurysm. This report highlights the importance of neuroimaging in unmasking compressive lesions in patients presenting with presumed optic neuritis.     

New Insight: Preperimetric Homonymous Hemianopia

A 29-year-old man was referred to our institution for a visual function evaluation 14 months after a traffic accident. His best-corrected visual acuity was 1.0 in both eyes. Brain magnetic resonance imaging revealed multifocal encephalomalacic changes in the left frontal and left temporal cortical and subcortical regions. Focal encephalomalacic changes in the left basal ganglia were observed in association with passive dilation of the left lateral ventricle, due to the presence of old haemorrhage. A non-specific scattered scotoma was found in the left eye. Spectral-domain optical coherence tomography (SD-OCT) was performed and peripapillary retinal nerve fibre layer (RNFL) thickness was reduced in the superotemporal and inferotemporal quadrants of the ipsilateral eye and the temporal and superonasal quadrants in the contralateral eye. SD-OCT revealed retinal ganglion cell degeneration extending from the nasal retina to the fovea in the right eye and from the temporal retina to the fovea in the left eye. In this patient, retrochiasmal defects resulted in RNFL defects corresponding to homonymous hemianopia without a visual field defect. Therefore, SD-OCT can be used to rule out a preperimetric homonymous hemianopia. This information would help to confirm the existence of an optic tract lesion before visual field defects.     

预后较好的视神经脊髓炎1例(英文)

患者,男性,马来人,26岁,以突发左眼视神经炎首诊,给予静脉甲基强的松龙治疗,3wk后完全缓解。1wk后,两次突发右眼视神经炎,Lhermitte征和Uthoff征呈阳性。第3d,患者双侧下身瘫痪,T8水平感觉丧失,症状进行性恶化,至第5d患者卧床不起。MRI检查显示双侧视神经增宽,胸髓至腰髓、脑干及少量大脑半球可见多节段性长的脱髓鞘斑块。迅速给予静脉甲基强的松龙初始大剂量治疗,继而口服强的松龙,并且进行密切的神经理疗。3mo后患者康复,可以借助辅助行走器行走。7mo后患者完全康复,能够重新开始其网站联系人的工作。在随后的1a随访期,病情未复发,患者最终视力提高,右眼6/9,左眼6/6,右眼视野表现永久性轻度中心盲点性暗点和普遍性视敏度降低。     

Optic Neuropathy As the Initial Presenting Sign of N-methyl-d-aspartate (NMDA) Encephalitis

A 52-year-old woman presented with painless vision loss for 3 months. She was in custody for allegedly robbing a bank and had recently been diagnosed with paranoid schizophrenia. She had 20/100 VA OD, a 2+RAPD, and optic atrophy. Extensive diagnostic workup including MRI, Fluorescein Angiography, Infectious Disease Panel, lumbar puncture, and leptomeningeal biopsy were unrevealing. Vision in her right eye declined to NLP and her left eye declined to 20/200 VA. Anti N-methyl-D-aspartate (NMDA) Autoimmune Encephalitis was diagnosed based on CSF serology and clinical suspicion. Her clinical course improved as she was treated with corticosteroids and rituximab.     

Optic tract compression from dolichoectatic basilar artery

PURPOSE: To report a case of optic tract compression caused by a dolichoectatic basilar artery. DESIGN: Observational case report. METHODS: A 74-year-old man with progressive loss of vision over 13 years and no other neurologic signs or symptoms was found to have bilateral optic nerve head pallor and a left homonymous hemianopia. RESULTS: Magnetic resonance imaging and angiography revealed a severe dolichoectatic basilar artery compressing the right optic tract. CONCLUSION: Basilar artery dolichoectasia may rarely cause compression of the optic tract and progressive visual loss.     

罕见梅毒性视神经周围炎1例(英文)

目的:报告1例罕见的由梅毒感染引起的视神经周围炎,经皮质类固醇治疗后严重视力缺损得到显著改善。方法:病例报告。结果:一名66岁马来裔女性患者来我院就诊,一眼突然出现视力缺损,动眼时有疼痛感。视力无光感,右眼出现相对性瞳孔传入缺陷(relative afferent pupillary defect,RAPD)。血清学和放射学检查证明为梅毒性视神经周围炎。肌肉注射苄星青霉素,静脉注射甲基醋酸泼尼松龙,然后长期口服类固醇,剂量逐渐降低。经过治疗,患者视力明显提高,恢复到6/18。随访4mo,未出现复发和类固醇相关的副作用。结论:本病诊断正确、治疗及时,能使患者视力得到显著改善。