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Results for “Optic Atrophy”

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PubMed1979

Variation of phenotype in Charcot-Marie-Tooth disease.

A two year old from a family with typical Charcot-Marie-Tooth disease has extensive ocular and central abnormalities, severe generalized neuropathy, and amyotrophy. Many abnormalities previously described in association with Charcot-Marie-Tooth disease are combined in this patient, including chorioretinal degeneration, optic atrophy, facial weakness, oligophrenia, and generalized amyotrophy. Documentation of the diagnosis in the child's ancestors and the precedent for each of his abnormalities suggest that these features represent a virulent expression of the inherited defect.

Amyotrophic Lateral Sclerosis↗
PubMed1999

Causes of visual impairment among students at the Alabama School for the Blind.

BACKGROUND: There are relatively few studies on causes of childhood visual impairment. This study determines causes of visual impairment and resultant visual acuity among students enrolled at the Alabama School for the Blind (ASB). METHODS: All students enrolled at ASB during the 1996-1997 school year (N = 123) were examined between April 1994 and March 1997. A retrospective record review was performed to determine the primary cause of visual impairment and the visual acuity with best correction. RESULTS: The most frequent causes of visual impairment involved the optic nerve (30.9%): optic atrophy, 13%; Leber's optic atrophy, 4.1%; optic nerve hypoplasia, 5.7%; and glaucoma, 8.1%. Other common causes included congenital malformations other than those categorized separately (primarily aniridia), 12.2%; cataracts/aphakia, 13.8%; albinism, 13.0%; and retinitis pigmentosa, 8.1%. Nystagmus, chorioretinitis, and other conditions accounted for the remaining 22% of diagnoses. Best-corrected visual acuity in the better eye ranged from 20/20 to no light perception (NLP), with 44.3% having acuity better than 20/200; 26.2% having 20/200 to less than 400; 13.1% having measurable acuity of 20/400 or worse; 9% having light perception; and 7.4% having NLP. CONCLUSIONS: Optic nerve disorders are the most common cause of visual impairment at ASB. Relatively few students (16.4%) had no measurable visual acuity.

Adolescent↗
PubMed1981

The latent period of the pupil light reflex in lesions of the optic nerve.

The latent period of the pupil light reflex was investigated by means of infrared pupillography in patients suffering from optic neuritis (retrobulbar neuritis, papillitis), papilledema, optic atrophy or anterior ischemic optic neuropathy. The inflammatory diseases of the optic nerve and the optic atrophy cause significant prolongation of the latent period of the pupil light reflex. Conversely, in papilledema no prolonged latent period could be demonstrated. The vascular diseases of the optic nerve affect the pupil latent period also, however, to a lower degree.

Computers↗
PubMedDate not supplied

[Computerized tomography of the orbit and optic nerve canals in optic nerve atrophy of diverse etiology].

Computerized tomography has been used to examine 10 patients with cranial injuries aftereffects presenting as optic nerve atrophies (Group 1), 24 patients with nontraumatic optic nerve atrophies (Group 2), and 89 subjects without optic nerve abnormalities. Density asymmetry in the projection of the optic nerve canal cranial openings has been the computerized tomography sign that indicated the involvement of the optic nerve in Group 1 patients. Increased pneumatization of the main bone sinus has been detected 2 times more frequently in Group 2 patients.

Adolescent↗
PubMed2005

Progressive encephalopathy with edema, hypsarrhythmia, and optic nerve atrophy (PEHO)-like syndrome: what diagnostic characteristics are defining?

Progressive encephalopathy with edema, hypsarrhythmia, and optic nerve atrophy (PEHO) syndrome is a rare, apparently autosomal recessive condition in which characteristic dysmorphic features are associated with subcutaneous edema, visual deficit, early arrest of psychomotor development, seizures, and cerebellar atrophy. A condition similar to PEHO syndrome, but without the neuroradiologic or ophthalmologic signs, is known as PEHO-like syndrome. We present the case of a child with PEHO-like syndrome and underline the need for a careful follow-up of these patients to identify signs and symptoms that can have a later onset, such as optic atrophy.

Brain Edema↗
PubMedDate not supplied

[Visual electrically evoked potentials in healthy subjects and patients with optic nerve atrophy].

Visual electrically evoked potentials (VEEP) were measured by the method developed by the author in 14 healthy subjects (14 eyes) and 20 patients with optic atrophy (22 eyes). The normative values were established. VEEP with slightly decreased amplitude, otherwise unchanged, were found in the patients in all the cases with visual acuity of at least 0.03. Comparative study of VEEP and flash VEP has lead the author to a hypothesis that retinal ganglion cells and their axons are a source of VEEP (provided suprathreshold electrical stimuli are employed). Comparison of VEEP, flash and pattern VEPs has not revealed significant correlations between them, this indicating the possible presence of a specific 'channel' in the visual system, responsible for VEEP generation.

Adolescent↗
PubMed2006

[Optic nerve atrophy after vitrectomy for diabetic retinopathy: its systemic and local risk factors].

PURPOSE: To evaluate the incidence and risk factors of optic nerve atrophy after vitrectomy for diabetic retinopathy. SUBJECTS AND METHODS: Clinical charts of 48 eyes of 40 patients who underwent vitrectomy for diabetic retinopathy were retrospectively reviewed. The relationship between postoperative optic nerve atrophy and patients' physical condition was statistically analyzed. RESULT: Postoperative optic nerve atrophy, distinct from glaucomatous optic nerve atrophy, occurred in 5 eyes (10.4%). Its incidence was correlated with higher plasma creatinine concentration (p=0.001), proliferative diabetic retinopathy (p= 0.046), and retinal white vessel formation (p= 0.007). Maintenance of the best postoperative visual acuity was difficult in the patients with optic nerve atrophy. CONCLUSION: Patients with proliferative diabetic retinopathy accompanied by renal dysfunction were at high risk of optic nerve atrophy after vitrectomy.

Adult↗
PubMed1978

[Diabetes insipidus, diabetes mellitus, optic nerve atrophy, and deafness--an autosomal recessive syndrome (didmoad-syndrome) (author's transl)].

By reporting a further case attention is drawn to the autosomal recessive inherited DIDMOAD-syndrome. While diabetes mellitus and optic atrophy are easy to recognize, one often has specifically to look for deafness, diabetes insipidus and the frequently associated dilatation of the urinary tract. Awareness of this condition is important for genetic counselling and vocational guidance, and allows to avoid invasive neuroradiological investigations.

Child↗
PubMed1997

Visual failure caused by raised intracranial pressure in craniosynostosis.

Craniosynostosis, the premature fusion of one or multiple cranial sutures, can be complicated by visual failure resulting from raised intracranial pressure (ICP). Of the 290 children operated on at the Birmingham Children's Hospital between 1978 and 1995 for craniosynostosis, 9 were found to have defective visual acuity attributable to raised ICP. Mean age at presentation was 3.11 years (range: 1-6 years) and mean follow-up, 7.33 years (range: 1.5-16 years). All these patients presented significantly later than usual, and 5 of them developed recurrent craniosynostosis. At the initial examination bilateral papilloedema was seen in 4 patients, unilateral disc oedema in 1 patient, bilateral optic atrophy in 3 patients and unilateral optic atrophy in 1 patient. Following decompressive craniofacial surgery, the visual outcome was good in 4 out of 5 patients with papilloedema and poor in all patients with optic atrophy. visual failure resulting from raised ICP in craniosynostosis is a devastating complication, which appears to be associated with late presentation and recurrent craniosynostosis.

Blindness↗
PubMed2003

Retinal and optic disc atrophy associated with a CACNA1F mutation in a Japanese family.

OBJECTIVE: To describe retinal and optic disc atrophy and a progressive decrease of visual function in 2 Japanese brothers. Both had a mutation in the CACNA1F gene, the causative gene of incomplete congenital stationary night blindness (CSNB). METHODS: We studied observational case reports and performed comprehensive ophthalmologic examinations including best-corrected visual acuity, biomicroscopy, ophthalmoscopy, fundus photography, and electroretinography. Genomic DNA was extracted from the peripheral blood, and all 48 exons of the CACNA1F gene were directly sequenced. RESULTS: The 2 brothers had retinal and optic disc atrophy and a progressive reduction of visual acuity with increasing age. Although these clinical features are not typical of previous patients with incomplete CSNB, both patients had an in-frame mutation with deletion and insertion in exon 4 of the CACNA1F gene. In both patients, the bright-flash, mixed rod-cone electroretinogram had a negative configuration, a characteristic of incomplete CSNB. However, the full-field scotopic and photopic electroretinograms were nonrecordable, indicating severe, diffuse retinal malfunction, which is not typical in incomplete CSNB. CONCLUSION: These findings indicate that a mutation of the CACNA1F gene may be associated with retinal and optic disc atrophy with a progressive decline of visual function. Clinical Relevance In patients with retinal and optic disc atrophy associated with negative-type electroretinograms, a CACNA1F gene mutation should be considered.

Amino Acid Sequence↗
PubMed1983

Parainfectious optic neuritis. Report and review following varicella.

Severe loss of vision and bilateral optic disc edema occurred in a 10-year-old girl 38 days after the rash of varicella first appeared and subsequent encephalitis developed. Recovery of visual function was nearly complete, but optic atrophy persisted. Parainfectious optic neuritis following other viral infections, with the exception of herpes zoster, has a similar clinical profile: delayed onset, severe visual loss, optic disc edema, bilaterality, good recovery, and residual optic atrophy. Conduction delays in our patient's patterned visual evoked potentials implicated demyelination within the optic nerves. The often favorable recovery, frequency of delayed onset, and bilateral involvement of the optic nerves support an autoimmune process in the pathogenesis of parainfectious optic neuritis.

Chickenpox↗
PubMed1990

[Visual evoked potentials to a light flash in patients with optic nerve atrophy].

Informativeness of different methods used for registration of optic generated potentials in response to light flash (FOP) has been studied in 27 healthy persons and 21 patients (32 eyes) with partial atrophy of the optic nerve. It is shown that the most informative one for diagnosing optic nerve atrophy and determining visual acuity in such patients is a method of investigation of the critical incidence of rythm reproduction of FOP light flickerings. Considering the complexity and difficulty of the mentioned method for its practical usage, a simplified method is proposed; a registration of FOP of a stable state with the incidence of stimulations of 33 Hz, that allows to detect cases of pathology with visual acuity not less than 0.2.

Evoked Potentials, Visual↗
PubMed2005

Registered blindness and low vision in Kuwait.

AIM: To estimate the incidence rates and major causes of registered blindness and low vision in Kuwait. METHODS: Data on age, gender and cause of blindness and low vision were collected from the Visual Disability Committee while evaluating Kuwaiti citizens applying for a blindness allowance from January 2000 to December 2004. Criteria for legal blindness in Kuwait are visual acuity 6/60 or less in the better eye with best possible correction or a visual field less than 20 degrees around the central fixation point. Incidence rates per 100,000 person years of observation were calculated for both genders in four age subgroups and four severity categories. The causes of registered blindness were classified according to the International Classification of Diseases, 10th edition. RESULTS: 412 persons were registered as blind, 272 males (66.01%) and 140 females (33.98%), mean age 28.7 +/-25.2 years, 39.32% below 20 years of age, 31.79% 21-40 years, 18.68% 41-60 years, and 9.95% 61 years and over . Male gender was prevalent for all age subgroups. The overall incidence rate was 9.97 per 100,000 person years of observation, 13.33 for the male and 6.69 for the female patients. The incidence rates rose from 7.35 for those 20 years and younger to 14.80 for the age subgroup 41-60 and 23.16 for those 61 years and above. The rates of severe visual impairment classified in categories 4 and 5 were higher than the rates for categories 2 and 3. Retinitis pigmentosa was the leading cause of blindness, followed by congenital anomalies and optic atrophy. In the subgroup below 20 the rate of optic atrophy was highest, followed by congenital malformations, retinitis pigmentosa and retinopathy of prematurity. In the next age subgroup (21-40 years), the rate of retinitis pigmentosa was three times as high as in the younger subgroup, followed by optic atrophy, congenital malformations and albinism. In the subgroup 41-60 the incidence rate for phthisis bulbi was twice as high as the rates for retinitis pigmentosa and optic atrophy. For those 61 years and older, the incidence rate of phthisis bulbi was almost five times as high as that for optic atrophy. The incidence rates for the male patients were higher for the major causes of disability in all age subgroups. CONCLUSIONS: The overall incidence rate of registered blindness for Kuwait is less than in many other national registries. The marked prevalence of the male gender in all age subgroups is specific for Kuwait. The rates of the leading causes of registered blindness reflect the prevalence of the younger subgroups in our registry. Additional data on co-morbidity and dedicated efforts to reveal unrecognized and unregistered blindness, particularly among females, will overcome the limitations of the registry, and will serve to outline the tendencies in avoidable vision loss and monitor the efficacy of the prevention programs in the future.

Adolescent↗
PubMed1985

Pattern ERGs and visual evoked potentials in maculopathies and optic nerve diseases.

Normative data were obtained in 52 volunteers to both transient and steady-state checkerboard pattern reversal. Two sizes of checks subtending 15' and 31' of visual angle were used. The simultaneous recording of pattern ERGs (P-ERGs) and visual evoked potentials (VEPs) permitted the determination of the retinocortical time. That is, the transit time expressed in milliseconds between the activation of retinal structures and the arrival of the signal in the visual cortex. Three groups of patients with maculopathies, optic atrophy, and suspected optic nerve demyelination were also studied. Early maculopathies were characterized by delayed P-ERG b-waves and delayed VEPs but normal retinocortical time. Optic nerve demyelination was characterized by normal P-ERGs, delayed VEPs, and prolonged retinocortical time. Optic atrophy and established maculopathies were characterized by abnormal P-ERGs and VEPs. The implications of these findings were discussed.

Adult↗
PubMed1982

[Transsphenoidal Microneurosurgery. Part 4: Neuroophthalmological cure in pituitary adenoma].

Fifty-nine pituitary adenomas treated with transsphenoidal approach were analyzed in the light of visual field defects and the postoperative cure rates. Twenty-six cases had visual field defects. The majority of the defects were temporal 1/2 or superior 1/4, although atypical deficits such as incongruous homonymous hemianopsia, nasal 1/2 defect, superior 1/2 defect, peripheral constrict were seen. The visual field defects were correlated with the size of the tumor in Hardy and Vezina classification of the suprasellar extension. The visual field defects appeared in 100% of Type C, 89% of Type B, 50% of Type A, 0% of Type O. In 24 out of 26 patients with preoperative visual field defect, the visual field improved either to normal or wider fields postoperatively. There were 9 patients with optic atrophy, which also showed the correlation with the tumor size. Only the tumor of Type B or C revealed optic atrophy but still 45.4% patients in this group had no optic atrophy. The visual field defect in 85% of the patients without optic atrophy improved to normal post-operatively, while 66% with mild and only 20% with severe optic atrophy showed complete recovery. In conclusions, authors emphasize followings. 1) Improvement rate in the visual field defect of pituitary adenomas by transsphenoidal approach was superior to the previously reported rates by transcranial operations. 2) The factors affecting the functional prognosis in the visual field defect are the natures of the tumor, surgical approach and the factors affecting the plasticity of the optic nerves. The existence and degree of optic atrophy could be one of the references for the plasticity of the optic nerves. 3) There is a close relation between the morphological potential of the tumor and optic atrophy/visual field defect. From these standpoints of view, the early diagnosis and treatment should be emphasized.

Adenoma↗
PubMedDate not supplied

[Operative treatment of anterior cranial base meningiomas].

BACKGROUND AND PURPOSE: The aim of the study is to assess outcome of surgical treatment of anterior cranial base meningiomas with special emphasis on the evaluation of visual skills after surgery. MATERIALS AND METHODS: A series of 52 consecutive patients operated on for anterior cranial base meningioma in the last ten years is reported. 18 patients had decreased visual acuity on admission, 6 experienced blindness in one eye, and 4 were totally blind; defects of visual fields were found in 17 patients. Primary optic atrophy and secondary optic atrophy were noted in fundcoscopy in 20 and 2 patients, respectively. Papilloedema was found in 3 patients. RESULTS: Meningiomas were resected radically (Simpson I surgery) in 13 patients and in 34 patients Simpson II surgery with coagulation of the dural attachment was made; meningiomas were partially removed in 5 patients (Simpson IV surgery). In one patient dense hemiparesis occurred after the surgery, and 3 others presented adynamic syndrome, one of whom made a good recovery in follow-up examination. Two patients died: the first one due to a large brain oedema and the second one due to myocardial infarction after uncomplicated postoperative course. The postoperative course in the other 46 patients was uneventful with good outcome. Visual acuity improved in 15 cases and did not change in 10 patients; visual acuity further decreased in 3 patients. CONCLUSIONS: Visual recovery is significantly related to preoperative visual acuity values of no less than 0.3 D and to the presence of normal optic discs on fundoscopic examination additionally tumour size less than 3.5 cm favourably affects visual prognosis in meningiomas of tuberculum sellae.

Adult↗