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PubMed1976

[A-scan-ultrasonography in unilateral optic nerve lesions (author's transl)].

With A-scan-ultrasonography it is possible to determine the time of transmission of ultrasound within the cross section of the distal optic nerve. In this way information regarding its thickness can be obtained. 156 patients with various unilateral optic nerve lesions were examined. Thickening of the affected nerve was found in the acute phase of neuritis nervi optici, trauma, optic nerve tumors and in papilloedema due to raised orbital or intracranial pressure. This investigation has proved a valuable aid in diagnosis in the following unilateral conditions: 1. Acute visual loss in the absence of significant fundus findings--an enlarged optic nerve ultrasonogram is an objective sign of a retrobulbar neuritis, or after injury, a haematoma. 2. Papilloedema--the optic nerve ultrasonogram gives information about the nerve. 3. Optic atrophy--an enlarged optic nerve ultrasonogram indicates an optic nerve or sheath tumor.

Adult↗
PubMed1991

Glaucomatous optic nerve atrophy: the circumlinear vessel revisited.

Baring of a circumlinear vessel, or gap sign, is a sign of glaucomatous optic neuropathy that can be rapidly assessed during direct ophthalmoscopy. We previously found a significant association between the presence of this sign and the diagnosis and status of visual field defects. In the current study we followed this same group of patients for a mean of 78 months. The rate of agreement between two independent observers for the presence or absence of a circumlinear vessel was 79%, and the intraobserver rate was 71%. The corresponding rates for the presence of a gap sign were 84% and 90%. The presence of baring was significantly associated with development of a visual field defect in the glaucoma suspect group (p = 0.0105). The development of a gap sign in photographs taken approximately 6 years later was significantly associated with clinical progression of the appearance of the disc or the visual field defect (p = 0.0142). We conclude that patients with baring of a circumlinear vessel should be closely monitored for the development or progression of the glaucomatous process.

Chronic Disease↗
PubMed1995

Focal narrowing of retinal arterioles in optic nerve atrophy.

PURPOSE: To investigate the occurrence of focal narrowing of retinal arterioles in optic neuropathies. PATIENTS AND METHODS: Color stereo optic disc photographs of patients with primary open-angle glaucoma (n = 114), pseudoexfoliative glaucoma (n = 41), normal-pressure glaucoma (n = 54), ocular hypertension (n = 45), nonarteritic anterior ischemic optic neuropathy (n = 20), descending optic nerve atrophy (n = 60), and control subjects (n = 100) were morphometrically examined in a masked fashion. Focal narrowing of the retinal arterioles was estimated on a scale ranging from 0 to 6. RESULTS: In the control subjects, focal vessel narrowing was significantly and positively correlated with age (P < 0.05). It occurred significantly more often and was more pronounced in eyes with optic neuropathies than in the normal eyes when matched for age. In the eyes with glaucoma, focal narrowing of the arterioles was significantly (P < 0.01) correlated with the degree of optic nerve damage. In addition to a tendency toward a more marked vessel narrowing in the eyes with normal-pressure glaucoma, it did not vary significantly between the various glaucoma types and other optic neuropathies. It was independent of an enlarged parapapillary chorioretinal atrophy. DISCUSSION: Similar to general constriction, focal narrowing of the retinal arterioles is found more frequently and is more marked in eyes with glaucomatous or nonglaucomatous optic nerve damage than in normal eyes. It is correlated with the degree of optic nerve damage, and is typical for optic nerve atrophy, but not pathognomonic for glaucoma.

Adolescent↗
PubMedDate not supplied

[Use of instenon in complex treatment of patients with optic nerve atrophy caused by craniocerebral trauma].

Instenon, a combined nootropic agent, was used on the step-type basis within a complex treatment of optic nerve atrophy caused by craniocerebral trauma. Our study revealed that instenon, when used within a complex treatment of a partial optic nerve atrophy caused by craniocerebral trauma, improved the visual acuity in 85.7% of patients and it expanded the total visual field in 76.2% of patients, whereas the routine therapy ensured the related improvements in 58.9% and 61.5% of patients, respectively. Besides, the drug provided for a better retinal electric sensitivity and for a better optic nerve conduction in 73.8% of cases versus 64.1% of cases with basic therapy. Increased velocity parameters in the ocular-artery blood flow were registered in 77.1%, of patients who received instenon, by duplex scanning.

Adolescent↗
PubMedDate not supplied

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 x 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.

Adult↗
PubMedDate not supplied

[Mechanisms of sensory function recovery in partial optic nerve atrophy before and after treatment with ampli-pulse phoresis].

Eighty-three patients (119 eyes) with partial optic nerve atrophy of different origin were treated by amplipulse phoresis and observed for 1 year. Based on the data of modern methods of examination of visual functions (changes in visual evoked potentials and achromatic static campimetry), the authors suggest that several mechanisms of visual function improvement underlie the therapeutic effect of amplipulse phoresis. These mechanisms are as follows: activation of the intact part of nerve fibers with restoration of myelin membranes, restoration of viability of nerve fibers in a state of parabiosis; a probable mechanism of visual acuity improvement is modification of the conduction of visual route on-channels, but this hypothesis needs further investigations.

Evoked Potentials, Visual↗
PubMed1983

Contrast sensitivity in evaluation of visual impairment due to macular degeneration and optic nerve lesions.

Spatial contrast sensitivity was investigated in a group of 17 patients with macular degeneration or optic atrophy. It has been reported earlier that patients with optic atrophy may have reduced contrast sensitivity at low spatial frequencies without decrease of visual acuity, a phenomenon called 'hidden visual loss'. The present study shows that also the opposite may be true: patients with macular degeneration or optic atrophy may have greatly decreased visual acuity and yet normal or nearly normal contrast sensitivity at low spatial frequencies and reasonably good sensitivity at intermediate spatial frequencies. This phenomenon could be called 'hidden vision'. In normal individuals and many visually impaired patients the spatial contrast sensitivity at high and intermediate spatial frequencies is equal when measured using grating fields of different size. In patients with central scotoma due to macular degeneration or optic atrophy contrast sensitivity is dependent on the size of the grating field: the maximal contrast sensitivity is higher when larger grating fields are used. The size of the smallest grating field that is needed for normal contrast sensitivity values at low spatial frequencies is one measure of visual impairment in this group of patients. Another measure of the function of the eccentric viewing area is the grating acuity which may also vary as a function of the grating field size.

Humans↗
PubMed2003

Optic nerve atrophy in propionic acidemia.

OBJECTIVE: Propionic acidemia is a rare metabolic disorder that is diagnosed in the early neonatal period. The disorder is characterized by life-threatening ketoacidosis, lethargy, failure to thrive, and developmental delay. Herein we report the ocular findings in a prospective series of six patients with propionic acidemia. DESIGN: Prospective case series. PARTICIPANTS: Six children (three male and three female) between the ages of 2 and 10 years with propionic acidemia who were examined at Children's Hospital Los Angeles. METHODS: A complete ophthalmic examination was performed on each of the six children. The examination included visual acuity testing, ocular motility, anterior segment examination, and funduscopic evaluation. Emphasis was placed on the function of the optic nerve and on the appearance of the optic disc, looking for possible atrophic changes. MAIN OUTCOME MEASURES: The clinical appearance of the optic disc and evidence of optic neuropathy. RESULTS: Optic nerve atrophy was present exclusively in all of the male patients in the series; none of the female patients demonstrated any detectable impairment of optic nerve function. The optic nerve atrophy was symmetric and age dependent and varied from moderate to severe. There were no other anterior or posterior segment abnormalities, other than one case of unilateral morning glory syndrome, diagnosed at birth. There was no correlation between metabolic control and the development and progression of optic nerve atrophy. CONCLUSIONS: Males with propionic acidemia have moderate to severe bilateral optic atrophy.

Amino Acid Metabolism, Inborn Errors↗
PubMed1997

Wolfram (DIDMOAD) syndrome.

Wolfram syndrome (MIM 222300) is the association of juvenile onset diabetes mellitus and optic atrophy, also known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness). Patients present with diabetes mellitus followed by optic atrophy in the first decade, cranial diabetes insipidus and sensorineural deafness in the second decade, dilated renal outflow tracts early in the third decade, and multiple neurological abnormalities early in the fourth decade. Other abnormalities include primary gonadal atrophy. Death occurs prematurely, often from respiratory failure associated with brainstem atrophy. Most patients eventually develop all complications of this progressive, neurodegenerative disorder. The pathogenesis is unknown, but the prevalence is 1 in 770000 in the UK and inheritance is autosomal recessive. A Wolfram gene has recently been mapped to chromosome 4p16.1, but there is evidence for locus heterogeneity, and it is still possible that a minority of patients may harbour a mitochondrial genome deletion. The best available diagnostic criteria are juvenile onset diabetes mellitus and optic atrophy, but there is a wide differential diagnosis which includes other causes of neurodegeneration.

Humans↗
PubMedDate not supplied

[Electrophysiological diagnosis in partial optic nerve atrophy syndrome].

Vision conduction routes were examined in 75 patients with the syndrome of optic nerve partial atrophy by recording the visual evoked potentials, threshold of electric sensitivity emergence, critical frequency of phosphene disappearance, electroretino- and electroneurograms. The findings of these examinations correlated best of all with the clinical picture when visual evoked potentials (latency increase, decrease of the amplitude with atrophy augmentation, interhemispheric asymmetry in chiasmal and retro-chiasmal involvement) or the critical frequency of phosphene disappearance (reliably reduced if a disease was developing) were recorded. Introduction of electrodes with therapeutic and diagnostic purpose permitted assessment of optic nerve function from the electroneurogram amplitude and time parameters.

Electrophysiology↗
PubMed2001

Mutation spectrum and splicing variants in the OPA1 gene.

Optic atrophy type 1 (OPA1, MIM 165500) is a dominantly inherited optic neuropathy that features low visual acuity leading in many cases to legal blindness. We have recently shown, with others, that mutations in the OPA1 gene encoding a dynamin-related mitochondrial protein, underlie the dominant form of optic atrophy. Here we report that OPA1 has eight mRNA isoforms as a result of the alternative splicing of exon 4 and two novel exons named 4b and 5b. In addition, we screened a cohort of 19 unrelated patients with dominant optic atrophy by direct sequencing of the 30 OPA1 exons (including exons 4b and 5b) and found mutations in 17 (89%) of them of which 8 were novel. A majority of these mutations were truncative (65%) and located in exons 8 to 28, but a number of them were amino acid changes predominantly found in the GTPase domain (exons 8 to 15). We hypothesize that at least two modifications of OPA1 may lead to dominant optic atrophy, that is alteration in GTPase activity and loss of the last seven C-terminal amino acids that putatively interact with other proteins.

Alternative Splicing↗
PubMed1982

X-recessive angiopathic opticopathy.

A familial optic atrophy with X-recessive heredity, distinct from Leber's optic atrophy (LOA), is described. The symptoms are: slight to moderate pallor of the papillomacular bundle at the disc possibly preceded by some hyperaemia of the disc, telangiectasia on the disc with normal retinal vessels, occurrence in the second decade of life, slow progression with often subclinical visual loss, a small relative central scotoma with an intact peripheral visual field, slight acquired tritanopia and deuteranopia, and vasomotor headaches. The disease may exhibit severe exacerbations with loss of vision to 1/60, provoked by vasoconstrictors and reacting favourably to vasodilators. This acute loss of vision is associated with ischaemia of the disc, a deep central scotoma with marked disturbance of colour vision in the form of an acquired deuteranopia, and sensoparalytic pupils. This is followed by increasing pallor of the disc, slow resolution of the central scotoma with a permanent reduction in the central light sensitivity, markedly disturbed Visual Evoked Potentials (VEP), acquired deuteranopia and normal ERG and EOG. In contrast to all hereditary opticopathies so far described, fluorescein angiography showed a disturbance of perfusion in the peripapillary choroid and the prelaminar part of the optic nerve. A similar disturbance of perfusion is described in anterior ischaemic optic neuropathy (AION) and low-tension glaucoma. To these acquired, non-hereditary recessive vascular opticopathies, which usually occur late in life, will have to be added the X-recessive vascular optic atrophy which we describe here, for which we propose the name: X-recessive angiopathic opticopathy. The differential diagnosis from some other hereditary, especially X-recessive, optic atrophies is discussed.

Adolescent↗
PubMed2003

[Vigabatrin-associated bilateral simple optic nerve atrophy with visual field constriction. A case report and a survey of the literature].

CASE REPORT: A 70-year-old male with optic disc pallor and visual field constriction up to 35 degrees bilaterally was previously treated with vigabatrin. The cumulative dosage was 3.7 kg. The b-wave was reduced in photopic and scotopic electroretinography and the patient had delayed visual evoked potentials. The retinal nerve fiber thickness was pathologically reduced in the Heidelberg retinal tomography (HRT) and the retinal nerve fiber layer analyzer (GDx). CONCLUSION: Vigabatrin is an antiepileptic drug and may increase the intraretinal GABA level due to an inhibition of the enzyme GABA-transaminase and may induce an ascending simple optic nerve atrophy. Vigabatrin-associated visual field defects may be irreversible. A visual field control is recommended before starting the treatment with vigabatrin and at regular 6-month intervals thereafter. In cases of visual field constriction, the therapy should be stopped. If the cumulative dosage is more than 3 kg of vigabatrin, the visual field controls should be performed more frequently because of the dosage-toxicity relationship.

Aged↗