Bilateral Central Scotomas Research Articles

Myelinated retinal nerve fibres loss in Leber's hereditary optic neuropathy

Editor, A 40-year-old man presented with bilateral, rapid, painless visual loss. He had a history of right eye anisometropic amblyopia. No cigarette smoking or alcohol abuse was declared. He had two maternal uncles with similar histories of bilateral visual loss. No genetic counselling had been carried out previously. His best corrected visual acuity (VA) was counting fingers with − 8.75 D in the right eye, and 20/60 with − 1.5 D in the left eye. Fundus examination showed normal optic discs with myelinated retinal nerve fibres (MRNF) in the right eye (Fig. 1). Right fundus at first examination. Peripapillary myelinated retinal nerve fibres, particularly at the inferior temporal border. Humphrey 24–2 static visual field testing revealed bilateral central scotoma. Normal optic disc perfusion was found at fluorescein angiography. Blood biology, biochemistry and cerebral MRI were also normal. Polymerase chain reaction (PCR) studies of the patient's leucocytes revealed a homoplasmic G11-778-A mutation of mitochondrial DNA mtDNA (one of the most common mutations responsible for Leber's hereditary optic neuropathy [LHON]). A Ubiquinone® trial failed to stop bilateral visual deterioration and the scotoma size increased bilaterally. On examination 2 years later, VA was hand motion in both eyes, there was bilateral optic atrophy and the right eye MNRF had disappeared (Fig. 2). Fundi 2 years later. (A) The right fundus shows loss of myelinated retinal nerve fibres, partial loss of papillomacular bundle and optic disc atrophy. (B) The left fundus shows optic disc atrophy. (C) Red-free photograph of the right fundus shows atrophy of retinal nerve fibres and loss of myelin. Myelinated retinal nerve fibres is a benign, developmental abnormality found on routine examination in about 1% of ophthalmic patients. It can be associated with ipsilateral myopia, strabismus and amblyopia (Buys et al. 1993). It occurs when myelinization of the optic nerve (probably issued from heterotopic oligodendrocytes) continues beyond the lamina cribrosa to involve the retinal nerve fibres. It usually remains unchanged, although MNRF may disappear after damage of the retinal ganglion cells. Several causes have been reported, namely, retinal artery occlusion, diabetic retinopathy (Gentile et al. 2002), radiotherapy for choroidal melanoma (Mashayekhi et al. 2003), chronic glaucoma (Lopez Sanchez et al. 2002), optic neuritis (Sharpe & Sanders 1975), and compression of the anterior optic pathway from the inner layer (central or branch retinal artery occlusion) (Bachmann 1922; Teich 1987), to the lateral geniculate body (Gentile et al. 2002). As far as we know, loss of MNRF has not been previously reported in LHON. In this disease, the retinal ganglion cell damage is produced by intracellular mitochondrial respiratory insufficiency (Kerrison et al. 1995).

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 Behçet'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 Behçet's disease with marked visual recovery within 45days of onset.

Intérêt de la correction prismatique dans la réhabilitation visuelle de patients atteints de dégénérescence maculaire liée à l’âge

To evaluate visual improvement in an age-related macular degeneration (ARMD) population with bilateral central scotomas, after a 3- to 6-month prismatic treatment. Prisms prescribed for both eyes were oriented according to eccentric fixation. Criteria were visual acuity (VA), reading ability, contrast sensitivity subjective visual comfort, prism wearing frequency and autonomy level. Thirty-one patients (23 women, 8 men) 65-95 years old (mean, 78 years) were selected. Mean long distance VA remained stable (0.249 at inclusion and 0.261 after prismatic treatment); long distance VA was unchanged for 51.6% of patients, improved by at least one line for 35.5% and decreased for 12.9%. Reading ability was slightly improved (average of 1 line), as was short distance VA (P14 at inclusion, P12 after prismatic treatment); reading ability was unchanged for 45.2% of patients, improved by at least one line for 38.7%, and decreased for 16.1%. Contrast sensitivity was improved for all levels: to 98% of contrasts (mean at inclusion 0.248 and 0.280 after prismatic treatment), to 50% of contrasts (0.214 at inclusion and 0.238 after prismatic treatment) and to 25% of contrasts (0.157 at inclusion and 0.185 after prismatic treatment). Visual comfort improved immediately after prismatic treatment for 90.3% of patients; 80.6% of patients wore their prismatic correction regularly. Prismatic treatment in this population mainly improves contrast sensitivity and visual comfort. It provides patients with better initiation and stabilization of their eccentric viewing. Most of the time, prismatic treatment must be associated with low-vision rehabilitation.

Sprint car visual loss

A 16-year-old boy developed bilateral visual loss 24 hours after a sprint car accident. Bilateral central scotomas were demonstrated but were unexplained by a thorough eye examination and neuro-imaging. A diagnosis of commotio retinae was established by multi-focal ERG testing. This relatively new technique is discussed, including comparison to full-field ERG and clinical applications.

Filling-in phenomenon in patients with age-related macular degeneration: differences regarding uni- or bilaterality of central scotoma.

The purpose of this study was to explore the presence of the filling-in phenomenon in patients with uni- or bilateral central scotoma (CS) resulting from natural history or laser photocoagulation of choroidal neovascularization in age-related macular degeneration (AMD). Sixteen consecutive patients with unilateral CS and 14 patients with bilateral CS were assessed (44 eyes) with a scanning laser ophthalmoscope (SLO). Scotoma was delineated by scotometry with a point (1 degree x1 degree) moving radially from the periphery to the center of the lesion. In addition, patients underwent a line test, consisting of a horizontal line moving vertically and a vertical line moving horizontally, from the periphery to the center. The lines were longer than the macular lesion and were projected onto the retina. Patients were asked to indicate when the lines seemed interrupted. The perceptual filling-in phenomenon was considered to be present when limits of the perceived scotoma, determined by the line test, were smaller than those assessed by scotometry. In patients with bilateral CS, the results were analyzed to distinguish the less or more severely affected eye. In all eyes, the limits of the scotoma obtained with the scotometry test corresponded to the anatomic edges of the macular lesion. In patients with bilateral CS, the filling-in phenomenon was observed in 12 out of 14 (85%) less severely affected eyes, but only in one (7%) of their more severely affected eyes. In patients with unilateral CS, the phenomenon was observed in only one out of 16 (6%) eyes. These results suggest that the filling-in phenomenon mostly occurs in patients with bilateral central scotoma, and almost always in their less affected eye. Thus, it did usually not occur in an eye if the fellow eye was better.

A Novel Mutation of the OPA1 Gene in a Japanese Family with Optic Atrophy Type 1

Purpose: To report a novel mutation of the OPA1 gene in a Japanese family with optic atrophy type 1 (OPA1) and to describe the clinical features of this family. Methods: Standard ocular examinations were performed on the proband and his two affected sons. The DNA sequence of all exons and splice sites of the OPA1 gene was determined to detect mutations. Results: The proband and his sons had a heterozygous mutation of the OPA1 gene in the third nucleotide of intron 12 (IVS12+3A→T). Clinically, each patient had reduced visual acuity (onset within the first 6 years of life) and optic nerve pallor. The proband showed bilateral central scotomas and generalized dyschroatopsia. This is the first report of OPA1 gene mutation in Japanese patients with familial optic atrophy. Conclusions: A mutation of the OPA1 gene was detected in a Japanese family with OPA1, which follows the same pattern as reported in Western countries. It is suggested that mutations of the OPA1 gene contribute to the development of optic nerve atrophy regardless of ethnic groups. Screening for the OPA1 gene mutation will be useful for diagnosis of OPA1 in Japanese patients.

IMPROVEMENT OF VISUAL ACUITY OVER TIME IN PATIENTS WITH BILATERAL GEOGRAPHIC ATROPHY FROM AGE-RELATED MACULAR DEGENERATION

To study the improvement in visual acuity over time in patients with central scotomas. In a prospective natural history study of geographic atrophy (GA) from age-related macular degeneration (ARMD) with annual follow-up, 36 patients with bilateral GA completed 3 years of follow-up. Protocol visual acuity (VA) measurements were performed. Scanning laser ophthalmoscopy (SLO) was performed, and the areas of GA were measured from fundus photographs. Six eyes of six patients with VA ranging from 20/80 to 20/500 had a VA improvement of two or more lines (mean, 3.2 lines). This was found only in the worse-seeing eyes of the patients and was contemporaneous with the deterioration in VA of the better-seeing eyes. Four of six eyes that improved in acuity had an improvement in the ability to find and hold the fixation target in an area of seeing retina, as assessed by SLO at follow-up, and a fifth eye changed from one fixation site that had little functional retina to another site. Spontaneous improvement in VA in eyes with bilateral GA and central scotomas may occur. It appears to be related to deterioration in VA of the better-seeing fellow eye and is associated with improvement of fixation in the worse-seeing eye. The worse-seeing eye of a patient with bilateral ARMD may have the potential for better vision than measured VA indicates. This finding may have implications for the choice of patients in treatment trials, for interpretation of long-term results, and for planning and assessment of low vision intervention.

Clinical and immunocytochemical findings in a case of melanoma-associated retinopathy

Objective To describe an unusual case of melanoma-associated retinopathy (MAR). Design Retrospective, observational case report and experimental study. Participants A 61-year-old man with a history of cutaneous melanoma, acquired bilateral central scotomas, and night blindness. Intervention Serial full-field electroretinography (ERG) and Goldmann perimetry were performed. Serum was screened for cancer-associated retinopathy (CAR) antibodies by Western blotting. Sections of human and rat retina were examined by immunofluorescence microscopy to determine whether retinal cells were reactive with this patient’s serum. A metastatic workup was performed. Main outcome measures Electroretinography, Goldmann visual field testing, and immunocytochemistry were performed. Results The results were as follows: (1) The ERG showed a profound loss of the b-wave amplitude and a “negative” b-wave characteristic of congenital stationary night blindness; (2) a central scotoma and peripheral constriction were identified on Goldmann visual field tests; (3) as in other patients with MAR, bipolar cells in human and rat retinas were immunolabeled with this patient’s serum; and (4) a previously unsuspected focus of metastatic melanoma was discovered. Conclusions Recognition of this condition may help to identify an occult focus of metastatic melanoma.

Combined use of several preferred retinal loci in patients with macular disorders when reading single words.

To investigate the use of several preferred retinal loci (PRL) when attempting to read, two patients with bilateral central scotomas were asked to decipher single words, successively projected onto the retina using a scanning laser ophthalmoscope (SLO). Video-recordings of the fundus image, on which the projected targets were superimposed, were analyzed frame by frame. One patient used two PRL in association and the other used three, each PRL having a specific function. Single word reading made it easier than with full texts to correlate the images parts scrutinized and the retinal areas involved. Then, as patients were unable to describe their reading behavior, the examiner monitored refixation movements using the SLO and asked questions to help them to become aware of their reading behavior. Eventually, they could localize their PRL, describe their specific functions, and switch at will between them.

Paraneoplastische Retinopathie unter dem klinischen Bild einer Zapfendystrophie mit Achromatopsie

A 72-year-old woman developed recurrent blindness on exposure to bright light (sunlight). Examination revealed total achromatopsia; bilateral central scotomas, predominant suppression of the cone response by electroretinography, and narrowing of the retinal arteries on ophthalmoscopy. The general examination revealed a pelvic tumor that later proved to be a pleomorphic carcinoma of presumed uterine origin. The patient died of metastatic disease 9 months after the ocular symptoms developed. Histopathologic examination of the eyes revealed loss of the photoreceptors, most extensive in the macular regions, and selective loss of the cones from the rest of the retinas. No ocular metastases of inflammation were found. The changes described are interpreted as paraneoplastic retinopathy of autoimmune origin.

Ocular Manifestations of Patients With Circulating Antineutrophil Cytoplasmic Antibodies

Antineutrophil cytoplasmic antibodies are seen in patients with systemic vasculitides, especially Wegener's granulomatosis. Antineutrophil cytoplasmic antibodies are helpful laboratory markers for these disease. We report on the ocular findings of six patients with systemic vasculitis who had antineutrophil cytoplasmic antibodies. Four patients had systemic Wegener's granulomatosis, one had microscopic polyarteritis, and in one a specific histopathologic diagnosis could not be made. Two patients were first evaluated for systemic vasculitis because of their ocular manifestations. Ocular findings included ptosis, bilateral lacrimal gland masses, proptosis, choroidal folds, episcleritis, phlebitis, retinal and vitreous hemorrhage, keratitis sicca, and bilateral central scotomas. It was difficult to make a systemic diagnosis in all cases. If systemic vasculitis is in the differential diagnosis of a patient with suggestive ocular findings, antineutrophil cytoplasmic antibody testing should be considered. A prospective study of antineutrophil cytoplasmic antibody testing should be considered in patients with ocular findings that suggest the possibility of vasculitis.

Results of Primary Treatment with Bromocriptine of Prolactinomas with Extrasellar Extension

Nineteen patients, seven women and twelve men, with macroprolactinomas characterized by extrasellar extension and basal prolactin levels above 6 U/l were treated with 10-20 mg bromocriptine daily in four divided doses for a mean period of 3.4 years (range 1.5-5.5 years). Plasma prolactin levels fell dramatically in all patients and values in the low normal range were obtained in sixteen patients. Tumor size was reduced by more than 75% in seventeen patients and by 50-75% in two patients. Tumor reduction was associated with the development of a partial empty sella in fourteen cases. In seventeen cases the pituitary became visible. Diminished visual acuity (six patients), bitemporal hemianopia (nine patients), unilateral and bilateral central scotomas (three patients) and oculomotor palsy (two patients) improved or normalized in all cases. Hypogonadism (all patients), hypothyroidism (nine patients) and hypocorticism (four patients) improved or normalized in most cases. It is concluded that in the medical treatment of macroprolactinomas 10-20 mg bromocriptine in four divided doses effectively reduces both plasma prolactin level and tumor size. The good results in this study may be related to the continued use of a fixed dose regimen of bromocriptine regardless of the plasma prolactin lowering effect.

Reading with the rod visual system

Using the scanned text technique developed by Legge and his colleagues, the present study demonstrates that visually normal subjects can read with the isolated rod visual system. The subjects were able to read text (albeit slowly) at luminances near their absolute thresholds. The peak reading rates increase with luminance at about 40 words per minute for every tenfold increase in luminance. The authors suggest that the study of scotopic reading performance in normal subjects might contribute to an understanding of reading problems in patients with bilateral central scotoma.

Bromocriptine treatment of prolactin secreting macroadenomas: a radiological, ophthalmological and endocrinological study.

Twelve patients, six women and six men, with macroprolactinomas characterized by extrasellar extension and basal plasma prolactin levels greater than 6 U/l were treated with 10-20 mg bromocriptine daily in four divided doses for a mean period of 2.4 years (range 0.5-3.5 years). The following observations were made: Plasma prolactin levels fell dramatically in all patients and values in the low normal range were obtained in 10 patients. Tumour size was reduced by more than 75% in 11 patients and by 50-75% in one patient. Tumour-reduction was associated with the development of a partial empty sella in eight cases. In four cases the pituitary became visible. Diminished visual acuity (three patients), bitemporal hemianopia (three patients), unilateral or bilateral central scotomas (three patients) and oculomotor palsy (two patients) restored to normal. Hypogonadism (all patients), hypothyroidism (six patients) and hypocorticism (three patients) improved or normalized in most cases. It is concluded that in the medical treatment of macroprolactinomas 10-20 mg bromocriptine in four divided doses effectively reduces both plasma prolactin level and tumour size.

Acephalgic Migraine: Fifteen Years Experience

Sixty-one patients with acephalgic migraine have been seen and thoroughly evaluated at the USAF School of Aerospace Medicine over the past 15 years. Patient age ranged in years from 21 to 61, with the number of spells varying between 1 and 100 and lasting from 15 seconds to 3 hours. These phenomena were present for 2 weeks to 25 years. Ocular involvement was represented by scintillation, transient hemianopia, bilateral central scotomata, classic amaurosis fugax, diplopia, altitudinal field loss, tunnel vision; temporal crescent involvement, and several patients who developed a transient central scotoma in one eye with alterations in color perception. Other neurologic involvement occurred in 29%. Permanent neurologic deficit occurred in only one patient. Positive family history for migraine was present in only 24%. Headache is not a necessary feature of migraine attacks. One should consider migraine in any acute episodic neurologic disorder.

STRACHAN'S SYNDROME 30 YEARS AFTER ONSET

While a prisoner-of-war in Malaya from 1942-1945, a 29-year-old man developed a painful sensorimotor neuropathy, bilateral central scotomata and sensorineural deafness. Examination 34 years later, after a long period of adequate nutrition, revealed considerable residual deficit. Nerve conduction studies suggested axonal degeneration with prominent collateral reinnervation. This case of Strachan's syndrome is reported to draw attention to the limited functional recovery and to focus attention on this condition at a time when famine conditions are rife in Southeast Asia.

Familial subacute necrotizing encephalomyelopathy of the adult form (adult Leigh syndrome)

A family--mother and 2 sons--with a heredodegenerative neurological disease is described. The disease started with bilateral optic atrophy, central scotoma, and color blindness during the second decade. This was followed by a quiescent period until additional neurological symptoms appeared, around the age of 50 years in the case of the mother and 40 and 30 years, respectively, in the sons. The additional symptoms were ataxia, spastic paresis, clonic jerks, grand mal seizures, psychia lability, and slight dementia. The disease was progressive, resulting in permanent hospitalization within a few years. The mother died at the age of 63 years and the sons at 46 and 43 years of age. Neuropathological examination revealed lesions histopathologically characteristic of subacute necrotizing encephalomyelopathy (SNE, Leigh disease), and their distribution in the brain and brainstem also conformed to this disease. On the basis of the clinical course and neuropathological findings, we consider that these 3 patients represent the first reported familial cases of the adult form of SNE.

Central scotomas due to digoxin toxicity.

Summary An old lady of 82 years presented with bilateral central scotomas. Digoxin was assayed at a toxic level in the serum. On withdrawal of the drug the scotomas improved. Diagnosis and management are discussed.

Central scotomata due to chlorpropamide (Diabenese). Case report.

Bilateral central scotomata may occur as an unusual toxic reaction related to the use of chlorpropamide (Diabenese *) as an oral hypoglycemic agent. The patient in this report developed blurred vision two days after beginning to take chlorpropamide. Givner¹has reported a patient who developed bilateral central scotomata after six months of chlorpropamide therapy. One must be constantly aware of the toxic aspect of any medication. Givner points out that one might be inclined to ascribe visual loss only to retinopathy in the diabetic patient. <h3>Report of Case</h3> A 53-year-old railroad worker was seen by his family physician on March 3, 1962, in a routine examination for fatigue and weakness. Examination was normal except for glycosuria 3+. Fasting blood sugar was 343 mg%. Complete blood count was normal. On March 10, 1962, the patient began to take chlorpropamide 250 mg each morning. Diabetic diet was advised. Two days later, the

Bilateral central scotomas of hysterical origin.

Since publication by Lincoff and Ennis, 1 in 1956, of a case of bilateral central scotomas in a hysterical child, two similar cases have been treated by the eye service at New York University-Bellevue Medical Center. A study of these cases has led to the conclusion that the syndrome of bilateral central scotoma of acute onset in the absence of visible ocular pathology, toxic symptoms, or a family history of hereditary blindness is probably hysterical in origin. The diagnosis of bilateral retrobulbar neuritis has always seemed unsound, for it is most unlikely that the discrete lesions of demyelinating disease will select simultaneously the axial portions of the separate optic nerves. While a single lesion in the chiasm can do this, a lesion so placed is distinguished by temporal hemianopic features. It is sometimes argued that the defect is only bilateral and simultaneous in its recognition, that the lesion in one