Ischaemic Optic Neuropathy in Lyme Disease

A 57-year-old man was referred for a painful acute inferior visual field defect in his right eye. Fundus examination of the right eye revealed diffuse optic disc oedema compatible with a papillitis. Cerebrospinal fluid (CSF) findings were consistent with lymphocytic meningitis, and serologic tests for Lyme disease were positive in both serum and CSF. After treatment with ceftriaxone and bolus of methylprednisolone, right eye inferior altitudinal visual field defect persisted despite resolution of papillitis, and fundus examination disclosed a superior optic atrophy in the right eye. To our knowledge, it is the first reported case of a unilateral Lyme optic neuritis occurring simultaneously to neuroborreliosis and further complicated by non-arteritic anterior ischaemic optic neuropathy.     

Postoperative visual improvement in spheno-orbital meningioma: a case report

A 59-year-old woman complained of right eye proptosis and visual impairment (20/100) lasting 2 months. CT and MRI scans disclosed a right spheno-orbital meningioma ranging over the optic foramen and cavernous sinus. Histopathology revealed transitional meningioma. Visual acuity (20/25), visual field, and proptosis were improved after surgery at the time of the ophthalmologic examination 6 months later. We present an additional case and review the available literature concerning postoperative visual outcome.     

Visual field defects in patients taking vigabatrin

PURPOSE: To investigate visual field loss in patients on long-term treatment with the antiepileptic drug vigabatrin, recently reported to cause visual disturbances. METHODS: Eighteen patients taking vigabatrin for 0.5-9.5 years were examined with automated perimetry up to 60 degrees from fixation using the Humphrey Field Analyser. Five patients with epilepsy receiving other medications served as controls. Patients found to have a visual field defect underwent ophthalmologic examination. RESULTS: Among the 18 patients in the vigabatrin group, visual field defects categorised as mild were revealed in 6 right eyes (33%) and 8 left eyes (44.4%), while defects categorised as severe were found in 9 right eyes (50%) and 8 left eyes (44.4%). The majority of the defects (66.7% in the right eye) were peripheral constriction with nasal predominance. The location of the defects was confirmed in 8 patients also tested with Kowa AP340 perimetry. CONCLUSION: According to our results, visual field defects among the patients on vigabatrin therapy may occur more frequently than previously recognised.     

Eccentricity compensated dark-on-bright stimuli in screening for neuro-ophthalmic visual field loss

One hundred and sixty-nine (87 right, 82 left) eyes of 98 consecutive patients aged 9-78 years (mean 42 yrs) were examined with both dark-on-bright and standard Goldmann bright-on-dark stimuli in a Neuro-Ophthalmology clinic. Single intensity suprathreshold static dark-on-bright stimuli were successively presented on a high resolution monitor under computer control at pre-selected coordinates in the central visual field, and the stimulus awareness was recorded by the patient pressing a button. Conventional luminous stimuli revealed a visual field abnormality in 110 eyes (57 right, 53 left) from 75 patients, and the dark stimuli correctly detected the presence of scotomas in 90% of eyes, with good or fair topographical correlation in 82% of eyes and poor correlation in 18% of eyes. Among the 59 eyes (30 right and 29 left, 36 patients) with no visual field defect, the dark stimuli produced a normal result in 86.5% of eyes. Dark-on-bright stimuli allow accurate diagnostic visual field examination in neuro-ophthalmic practice.     

Beidseitige Stauungspapillen,einseitiger Visusverlust und Abduzensparese

A 35-year-old woman complained of headache, reduced visual acuity, restricted visual field in the right eye and blindness in the left eye. The examination of the retina showed papilledema and peripapillary hemorrhages in both eyes. Magnetic resonance imaging (MRI) revealed a sinus thrombosis. Despite modern imaging technologies sinus thrombosis is an often overlooked, life-threatening disease and needs immediate treatment in order to avoid long-term consequences. An ophthalmological examination can be pioneering as it leads to further imaging.     

Optic Neuropathy Mimicking Normal Tension Glaucoma Associated with Internal Carotid Artery Hypoplasia

Enlargement (excavation) of the right optic disc was discovered in a 41-year-old woman during an examination to fit her for contact lenses. She was initially diagnosed with normal tension glaucoma (NTG) in that eye and subsequently treated with topical medication. However, Goldmann perimetry results indicated there were changes in the temporal visual field that were not typical for glaucoma. In addition, magnetic resonance angiography (MRA), three-dimensional computed tomographic angiography (3D-CT) and orbital color Doppler imaging (CDI) results revealed the presence of segmental hypoplasia and stenosis of the right cervical internal carotid artery (ICA). The patient therefore had ipsilateral coexisting ICA hypoplasia and optic neuropathy. We postulate that the visual field change in this patient was due to an ischaemic event in the optic nerve that was related to a congenital anomaly of the right cervical ICA.     

Functional and clinical findings in 3 female siblings with crystalline retinopathy

Purpose To present functional and clinical findings in three female siblings with crystalline retinopathy. Methods Functional and clinical examinations, including full-field and multi-focal electroretinograms (ERG), visual field, dark adaptation and fundus fluorescein angiography (FFA) were performed in three female siblings of a nonconsanguineous Chinese family, who exhibited characteristic retinal crystalline flecks of Bietti crystalline retinopathy. Results Ophthalmological examination revealed similar findings in the first and second daughter of the family. Best-corrected visual acuity was hand movement and finger counting respectively and funduscopic examination showed RPE clumping and relatively fewer yellow-white deposits in the posterior pole and mid-peripheral retina. FFA revealed massive RPE and choriocapillaris destruction. Ganzfeld ERG was undetectable or reduced markedly and multifocal ERG showed all responses were markedly depressed. Ophthalmological examination showed relative preservation of retinal function in the third daughter of the family (the proband). Best-corrected visual acuity was 0.5 in the right eye and 0.4 in the left eye. Funduscopic examination showed numerous crystal deposits scattered throughout the posterior pole. Dark adaptation revealed rod thresholds elevated dramatically and visual field examination demonstrated paracentral scotomas in both eyes. Full-field ERG was decreased slightly and multifocal ERG showed the central responses were markedly depressed. Conclusion The present study describes typical crystalline retinopathy affecting three female siblings in a family.     

Photophobia associated with a demyelinating lesion of the retrochiasmal visual pathway

PURPOSE: To present two patients in whom photophobia was a predominant presenting symptom of retrochiasmal demyelination. DESIGN: Observational case report. METHODS: Two women who experienced an acute onset of photophobia underwent neuroophthalmic examination and investigations at a single institution. RESULTS: Examination revealed a homonymous visual field defect in both patients. Magnetic resonance imaging (MRI) demonstrated a hyperintense lesion in the area of the right posterior thalamus in one patient and enhancing lesions in the deep white matter of the temporal lobes bilaterally with contrast enhancement of the right optic tract in the second patient. Additional investigations, including lesion biopsy in the first patient, revealed demyelination as the most likely etiology. Improvement of photophobia, visual field defect, and radiographic abnormalities was documented over the next three to four months without accompanying treatment in both patients. CONCLUSIONS: Central pathology, including demyelination of the retrochiasmal visual pathway, should be considered in patients who experience acute photophobia.     

Congenital retinal macrovessel: atypical presentation using optical coherence tomography

To describe a congenital retinal macrovessel with macular thickening. This case was investigated using fundus photography, fluorescein angiography, Spectralis optical coherence tomography and a 10–2 visual field test. A 23-year-old man was referred to our clinic with decreased vision in the right eye. Fundus examination of the right eye revealed a congenital retinal macrovessel that originated inferior to the superotemporal branch of the central retinal vein. Using fluorescein angiography, early filling and delayed emptying of the aberrant vein were observed. Spectralis optical coherence tomography demonstrated macular thickening and was supported by a 10–2 visual field test that revealed a relative scotoma corresponding to the same location. At the 18-month follow-up, visual acuity remained stable. Although rare, this case demonstrated that macular thickening can cause decreased visual acuity in the presence of a congenital retinal macrovessel.     

Myelinated retinal nerve fibers (MRNF) – Dilemmas related to their influence on visual function

Myelinated nerve fibers (MNF) occur in less than 1% of the population, however, they might be responsible for diagnostic dilemmas in cases with visual loss. The case report of an aged pseudophakic patient with visual deterioration in the right eye and MNF in both eyes is presented. The documentation provided by the patient proved recent several examinations of both fundi, and all of them were described as normal. Physical examination revealed the posterior capsule opacification in the right eye, white lesions on the retina of the right eye around the optic disk, and in the left eye – the peripheral, which could correspond to the myelinated fibers. Although visual field changes and OCTs corresponded to the NMF, it turned out, however, that visual acuity loss was in fact caused by PCO and was reversed by the YAG capsulotomy procedure. This case shows some problems related to MNF diagnosis and evaluation of their influence on visual function.     

Central retinal artery occlusion or branch retinal artery occlusion in the young associated with high lipoprotein (a) levels

INTRODUCTION: High plasma lipoprotein (a) concentration is an independent risk factor for atherosclerosis and thrombosis. PATIENTS: We present the cases of two young women, one with central retinal artery occlusion and the other with branch retinal artery occlusion. Case 1: A 32-year-old woman was seen in our clinic complaining of a sudden decrease in visual acuity to 1/50 with a central visual field defect in the right eye. The fundus examination revealed an occlusion of the central retinal artery, which was confirmed by fluorescein fundus angiography. Thorough systemic evaluation showed no signs of cardiovascular disease, no coagulation abnormalities, and no clinical or biological evidence of a systemic disorder. On the other hand, hypercholesterolemia and elevated levels of lipoprotein (a) were detected. Case 2: A 35-year-old woman consulted complaining of a visual field loss in the left eye without reduction of visual acuity. Fundus examination and angiography showed the occlusion of the temporal-superior branch artery. A detailed systemic work-up revealed a pseudo-bicuspid aortic valve and high lipoprotein (a) levels. CONCLUSION: These two cases emphasize the importance of a thorough systemic evaluation of young adults with a retinal artery occlusion. High plasma lipoprotein (a) concentration is a risk factor which should not be neglected.     

Suspected simultaneous bilateral anterior ischemic optic neuropathy in a patient with Behçet's disease

AIM: To report a 47-year-old Japanese woman with a one-year history of Beh?et's disease who complained of sudden bilateral visual loss with concurrent anterior ischemic optic neuropathy (AION). CASE REPORT: The patient's Snellen visual acuity was 0.1 (OD) and 0.3 (OS) of onset. There was bilateral mild anterior chamber inflammation. Bilateral optic disc pale swelling was observed without retinal exudates and edema. Fluorescein angiography demonstrated bilateral hypofluorescence of the optic disc in early frames but with no distinct retinal vasculitis. Visual field showed bilateral relative central scotoma and right altitudinal hemianopsia. Laboratory examination revealed an ESR of 26 mm in the first hour with a C-reactive protein level of < 0.3 mg/dl. Periocular injection of triamcinolone acetonide in both eyes without systemic corticosteroid administration improved her visual acuity to 0.7 (OD) and 1.2 (OS) within 45 days of onset. Bilateral optic disc swelling gradually resolved. In the early stages, fluorescein angiography demonstrated normal optic disc filling in both eyes. There was a residual right central scotoma on visual field. CONCLUSION: We observed an extremely rare case of simultaneous bilateral AION with Beh?et's disease with marked visual recovery within 45 days of onset.     

Endoscopic and trans-fornix removal of a giant orbital-ethmoidal osteoma

A 39-year old female was referred with a 2 year history of slowly progressive headache, exophthalmos, diplopia and restricted eye movements with exotropia of the right eye. Orthoptic examination revealed restricted elevation and mildly restricted adduction of the right eye. CT and MRI demonstrated a large (35 x 20 x 23 mm) calcified infraorbital lesion extending into the ethmoidal sinus. Because the visual field defects were progressive and the acuity OD dropped to 20/80 surgical intervention was necessary. The osteoma was successfully removed using an inferior and medial orbitotomy with swinging eyelid combined with an endoscopic approach. In a second procedure the orbital floor was reconstructed with a porous polyethylene (Medpor) implant. A final procedure consisted of a 3 mm recession of the left superior rectus muscle and infundibulotomy by the sinus surgeon to open the blocked maxillary sinus. After 6 months visual acuity OD had returned to 20/20. Orthoptic examination showed normal binocular function.     

The Pulfrich phenomenon and its alleviation with a neutral density filter.

A case is described in which a presumed vascular accident resulted in long-standing visual difficulties in both reading and the analysis of vectors of moving objects. Clinical examination revealed minimal right optic atrophy with a relative superior altitudinal visual field defect associated with a positive Pulfrich effect. A partial head turn to the right in association with paresis of saccades and pursuit eye movements to the right was also evident. Spectacles for distance which incorporated a neutral density filter before the left eye were prescribed. These practically eliminated the Pulfrich effect and alleviated the problems of vector analysis. A near correction was provided which incorporated prisms with bases to the right. This eliminated the reading difficulties. The visual problems experienced by patients suffering from a positive Pulfrich effect are described and discussed.     

Optic nerve neuropathy following a lightning accident--electrophysiologic and computerized tomography findings

A case is described in which the patient had been struck by lightning, with involvement of one eye and the visual pathways. Ophthalmological examination revealed a posterior capsule cataract in the right eye and peripheral constriction of the visual field of the left eye, although central vision in the left eye was not affected. The peak latency of the pattern reversal-evoked potentials in the left eye was longer and their amplitude lower. The skin ERG was normal. The CT scan revealed a thickening of the left optic nerve. The importance of electrophysiological and CT scan examination in the diagnosis and etiology of abnormalities caused in the eye by lightning is emphasized.     

Lhermitte-Duclos Disease and Cerebellar Gangliocytoma—An Incidental Finding in a Patient with Gradual Vision Loss

A 50-year-old male patient presented to the neuro-ophthalmology clinic with chief complaints of gradual decrease in vision in both eyes, more in the left eye, for 6 years. On general examination, the patient had a hemiplegic gait. His presenting acuity was 20/50 in the right eye and 20/320 in the left eye, not improving further. He had dense posterior subcapsular cataracts in both eyes, and fundus examination revealed pale discs. Humphrey visual field tests 30-2 revealed a vertical nasal midline defect in the right eye and grossly depressed fields in the left eye. Keeping in mind the above findings, the authors requested for a magnetic resonance imaging (MRI) of the brain. The brain MRI shows a large infarct in the right parieto-occipital lobe and a small circumscribed lesion in the left cerebellum. The radiologist opined that it could possibly be a gangliocytoma of the cerebellum, and a possible diagnosis of Lhermitte-Duclos syndrome was made.     

Bilateral simultaneous anterior ischemic optic neuropathy associated with sildenafil

We report a case of simultaneous bilateral nonarteritic anterior ischemic optic neuropathy (NAION) due to sildenafil use. A 55-year-old man with an unremarkable medical history presented simultaneous bilateral NAION 8 months after continuous use of sildenafil 4-5 times a month. At presentation, visual acuity (VA) was 0.7 in the right eye (RE) and 0.9 in the left eye (LE). The visual field showed an inferior altitudinal defect in both eyes and a fundus examination revealed prominent optic disc edema in the RE and a crowded optic disc in the LE. The patient was counseled to discontinue sildenafil, and 3 weeks later VA was 1.0 in both eyes and the optic disc edema in the RE was resolved. However, a visual field defect remained in the RE. Three months later, visual fields were unchanged. To the best of our knowledge, this is the first reported case of simultaneous bilateral NAION due to sildenafil use.     

Optic nerve cyst associated with optic disk pits

Purpose To report a case with two optic disk pits which were associated with an optic nerve cyst in the same eye.Methods Observational case report.Results A 47-year-old patient noted visual impairment in the right eye. On examination the best corrected visual acuity in the right eye was 20/80 and in the left eye was 20/20. Biomicroscopy revealed, in the right eye, a very pale optic disk with two optic disk pits without macular elevation. Magnetic resonance imaging (MRI) revealed a well circumscribed 6×6-mm² round cystic lesion within the right optic nerve sheath adjacent to the temporal aspect of the right optic nerve at its retrobulbar segment, which compressed and displaced the nerve.Conclusions In the case of an extremely pale optic disk with congenital pits and visual impairment without macular detachment, radiological examination is indicated in order to exclude the possibility of coexisting optic nerve anomalies.     

OCT and muti-focal ERG findings in spontaneous closure of bilateral traumatic macular holes

Purpose To report the spontaneous closure of bilateral traumatic macular holes in a young patient, followed up with optical coherence tomography (OCT), muti-focal electroretinogram (mfERG), and Humphrey visual field examination. Methods A 25-year-old male who suffered from bilateral blunt trauma to the eyes and developed traumatic macular holes was followed with ophthalmic examination, OCT, mfERG, and Humphrey visual field examination. Results The OCT results revealed spontaneous closure in both eyes 2 weeks after trauma, and the macular holes remained closed in the 7 months of follow-up. Visual acuity improved to 0.7 in right and 0.9 in left eye finally. Visual field examination and mfERG results improved slightly compared with the initial tests. However, there still were central scotomas in both eyes, especially in right eye at the end of follow-up, and the peaks of the retinal response density had not recovered in the macular area of the mfERG topography. Conclusions Spontaneous closure of unilateral traumatic macular hole is not uncommon, but there is no report of spontaneous closure of bilateral traumatic macular holes as yet. Since the traumatic macular holes may close spontaneously, traumatic macular holes may be observed for a period of follow-up.     

Early unilateral macular sensitivity changes in microperimetry in a case of pituitary adenoma

The value of the MP-1 microperimeter in early diagnosis of pituitary tumours by detection of changes in macular sensitivity is described. A 21-year-old female presented with blurred vision in the right eye. Ophthalmic examination was unremarkable. Static perimetry of the central visual field (30°) was performed by use of a Humphrey automatic perimeter, and the retinal sensitivity of the 12 central degrees was measured by use of the MP-1. Static perimetry revealed a peripheral visual field defect without involvement of the macular region. The MP-1 revealed an important loss of sensitivity (3.4 ± 4.8 dB) in the central 12°. Magnetic resonance imaging revealed a pituitary adenoma with sellar and suprasellar extension. Three months after surgical removal, use of the MP-1 revealed complete recovery of the sensitivity (19.28 ± 2.5 dB), fixation location, and fixation stability of the right eye. In pituitary tumours, the presence of a sub-clinical form of macular involvement can be demonstrated and followed-up accurately by use of the MP-1.