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PubMed2001

Juvenile nasopharyngeal angiofibroma presenting as Foster Kennedy Syndrome.

Juvenile nasopharyngral angiofibroma (JNA) is a rare benign tumor of the nasopharynx that occurs in adolescent boys with epistaxis and nasal obstruction. It may grow into the cranium causing elevated intra-cranial pressure and compression on the optic nerve. A histological-proven case of JNA in an 18 year-old Ethiopian boys is presented. He became blind due to optic atrophy in the right eye, but salvaged a useful vision in the left eye radiotherapy. The possibility of Foster-Kennedy Syndrome, a presentation of one atrophic and one papilloedematous optic nerve head of bilateral asymmetric optic atrophy, is discussed. Controversies about its histological appearance, natural history, diagnostic methods and management modalities are reviewed. JNA should be considered in adolescent boys who present with optic atrophy and/or nasal mass. Early detection and initial surgical treatment with adjunct radiotherapy could have prevented visual loss on this boy.

Adolescent↗
PubMed1998

[Wolfram syndrome. Three case reports].

Wolfram syndrome is a rare disorder defined by the occurrence of diabete mellitus, diabete insipidus, optic atrophy and deafness; DIDMOAD is a commonly accepted acronym. We report 3 further cases: two girls and one boy. Their age ranged from 12 to 17 years. The diagnosis was based on the presence of juvenile diabete mellitus, bilateral optic atrophy, urologic signs, with urinary tract dilation; and deafness in two cases. The ophthalmic signs of Wolfram syndrome are progressive decrease in visual acuity, constriction of the peripheral visual field with or without central scotoma, color vision disturbances and bilateral optic disc atrophy. Diabetic retinopathy is a rare complication. The other clinical features are discussed so as to differentiate between Wolfram syndrome and other optic atrophies associated with diabete mellitus. We discuss of the pathogenic hypothesis including the mitochondrial dysfunction.

Adolescent↗
PubMed1995

[Wolfram syndrome. Personal experience].

We report on a young patient who suffered from diabetes mellitus and neurosensorial deafness from the age of two. One year later she was noted to have deteriorated vision and the diagnosis of optic atrophy was made, her visual acuity decreased progressively. At the age of six she was admitted to our hospital because of thiamine responsive megaloblastic anemia, a rare clinical feature of Wolfram's syndrome (only 13 cases have been reported to date). Thiamine (75 mg/day) was commenced at a single oral dose with a rapid increase of Hb level after a few days of therapy. The insulin requirement didn't decrease during thiamine therapy, the C-peptide level after glucagon remained almost indosable. No improvement was observed in the deafness and in the optic atrophy. These findings suggest that diabetes mellitus and optic atrophy, in Wolfram's syndrome are not related to thiamine metabolism.

Autoantibodies↗
PubMed1997

Optic neuropathy in familial dysautonomia.

Optic atrophy, which is indicative of a CNS disorder, is a rarely described manifestation of familial dysautonomia (Riley-Day syndrome). As these patients are now living longer, the prevalence of optic neuropathy also may be increasing. We present a man with familial dysautonomia and visual loss resulting from optic atrophy and visual field defect suggestive of chiasmal pathology.

Dysautonomia, Familial↗
PubMed2001

The PEHO syndrome.

The progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome is a pediatric disorder of unknown origin, characterized by a combination of postnatally progressive encephalopathy, hypsarrhythmia, and optic atrophy. The pathological findings are early progressive atrophy of the cerebellum, brainstem, and optic nerves. Nitric acid (NO) has recently been implicated in the mechanisms of seizure activity and neurodegeneration, which are both very active in the PEHO syndrome. However, recent studies have provided evidence that insulin-like growth factor 1 (IGF-1) may prevent the NO-mediated neuronal damage and is essential for the survival of the cerebellar granule cells. These cells will degenerate in the PEHO syndrome. In this study, we set out to test the hypothesis that NO production is activated in the PEHO syndrome and that NO production may be correlated with the reduced production of IGF-1 in the brain. Cerebrospinal fluid IGF-1 was determined with an RIA kit and NO metabolites by the Griess calorimetric method. In patients with the PEHO syndrome, as compared with controls, the levels of IGF-1 were reduced and the levels of nitrite/nitrate were markedly elevated. Defective production of IGF-1 probably reflects the underlying neurodegeneration and the increase in NO production probably reflects the seizure activity and/or neurodegeneration. These are the first biochemical abnormalities found in the PEHO syndrome and their study may lead to a better understanding of this devasting disease.

Brain Diseases↗
PubMed1992

C wave of electroretinogram and visual evoked response in optic neuritis due to demyelinating diseases.

In 21 consecutive cases with unilateral optic neuritis due to demyelinating disease, ERG, C wave of ERG and VER were recorded and studied in both eyes. Also 8 cases suffering from optic atrophy due to trauma or glaucoma were included. The analysis of data shows that the C-wave is significantly diminished in both eyes, namely the affected and the sound, in the cases of optic neuritis. Also in comparison to VER, C wave has a comparative probability in diagnosing optic neuritis due to demyelinating disease. The C wave of ERG is normal in the cases of optic atrophy. The mechanism of C wave involvement during the course of demyelinating disease may be related with an abnormality of the ionic homeostasis of the subretinal space.

Adolescent↗
PubMed2007

CD4(+), CD8(+), immunoglobulin status and ocular lesions among some onchocerciasis-infected rural Nigerians.

CD4(+), CD8(+), immunoglobulin status and ocular lesions of some onchocerciasis-infected rural Nigerians as manifested by the presence of microfilariae in their skin snips and some chronic symptoms of the parasitic infestation were evaluated. The highest mean CD4(+) and mean microfilarial (mff) load of 560 +/- 20.46 unit cells/microl and 20 mff/mg were recorded among the individuals within the second decade of life, while the least mean CD4+ and mff load of 307.20 +/- 11.23 unit cell/microl and 6.5 mff/mg occurred among volunteers after 60 years of age. The highest mean CD8(+) of 388.00 +/- 23.71 unit cells/microl occurred at the third decade of life. The individuals above 60 years had the least mean CD8(+) of 350.25 +/- 11.90 unit cells/microl. The volunteers had mean CD4(+) of 372.45 +/- 109.02 unit cells/microl and mean CD8(+) of 359.42 +/- with an overall CD4(+):CD8(+) ratio of 1.04. The mean CD4(+) and mean CD8(+) had positive correlation with the mean microfilarial load (r = 0.52 and r = 0.40), respectively. The mean IgE, IgG, lgA, IgM, and IgD were 2,074.82 +/- 823.09, 19.36 +/- 2.49, 3.88 +/- 0.26, 3.59 +/- 0.38, and 0.29 +/- 0.19 mg/dl, and these immunoglobulins negatively correlated with the mean microfilarial load at r = -0.02, r = -0.15, r = -0.82, r = -0.37, and r = 0.26, respectively. Among these immunoglobulins evaluated, only mean IgE (2,074.82 +/- 823 ng/ml) was statistically different from the control subjects (0 mg/dl) at t = 3.39, P < 0.05. In all, the prevalence of the visual impairment and lesions were low. Among the six visual lesions, namely, cataract, choroidoretinitis, iridocyclitis, glaucoma, sclerosing keratitis, and optic atrophy encountered in Egoro-Eguare, only optic atrophy and sclerosing keratitis was reported among the children. The female adults had the highest prevalence of reduced vision 20(43.5%). Also, the prevalence of choroidoretinitis (2.2%) and iridocyclitis (2.2%) were the least prevalent ocular lesions reported among these female adults. The depletion of the CD4(+) contributed to the low prevalence of visual impairment and lesions in this locality. The depleted CD4(+), CD8(+), and the lower values of IgA, IgM, and IgG contributed in the maintenance of chronicity of onchocerciasis in Egoro-Eguare, Nigeria.

Adolescent↗
PubMed1979

[Axial computerised tomography - answered and unanswered questions on the ophthalmologist (author's transl)].

The results of 67 examinations with computerized axial tomography are presented with reference to the indications. 15 examinations were performed in exophthalmos, 11 in unknown visual field defects, 9 in optic atrophy of unknown origin, 8 each in optic neuritis, papilledema and unclear visual disturbance. 8 furthur examinations were performed in different cases. The axial computerized tomography proved to be an efficient complementary examination technique if it is limited to well defined indications. The best results are obtained examining exophthalmos and visual field defects.

Brain Neoplasms↗
PubMed2004

Hereditary optic neuropathies.

AIMS: To provide a clinical update on the hereditary optic neuropathies. METHODS: Review of the literature. RESULTS: The hereditary optic neuropathies comprise a group of disorders in which the cause of optic nerve dysfunction appears to be hereditable, based on familial expression or genetic analysis. In some hereditary optic neuropathies, optic nerve dysfunction is typically the only manifestation of the disease. In others, various neurologic and systemic abnormalities are regularly observed. CONCLUSION: The most common hereditary optic neuropathies are autosomal dominant optic atrophy (Kjer's disease) and maternally inherited Leber's hereditary optic neuropathy. We review the clinical phenotypes of these and other inherited disorders with optic nerve involvement.

Dysautonomia, Familial↗
PubMed1975

[Vascular pseudopapillitis (author's transl)].

Besides the true ischaemias of the optic nerve, which explain the post-haemorrhagic amaurosis, the low tension glaucoma and the open angle glaucoma, there are two varieties of vascular pseudopapillitis. First the variety due to arteriosclerosis, which is characterized by a palid oedema of the disc, followed by optic atrophy, a diminution of the vision field, as well as by signs of arteriosclerosis at the biopsy of the temporal artery. The second variety is the temporal arteritis, which is also characterized by a palid oedema of the disc, followed by optic atrophy, a visual loss, which is often complete, a marked rise of the erythrocyte sedimentation rate and a typical granulomatous arteritis at the biopsy of the temporal artery. These two varieties are due to an involvement either of the short posterior ciliary arteries or of the central vascular or pial system of the optic nerve.

Arteriosclerosis↗
PubMed2002

[Wolfram syndrome. A new case report].

The WOLFRAM syndrome (SW) is a rare hereditary disorder described for the first time in 1938 as the coexistence of a diabetes mellitus and an optic atrophy to which join frequently a diabetes insipidus and a bilateral deafness. Several genetic studies are current to determine transmission mechanisms, physiopathology of the disorder to update a curative therapy. On the occasion of a new case report we remind the main characteristics of this syndrome. The SW is a rare neurodegenerative disorder characterized by the coexistence of diabetes mellitus, diabetes insipidus, optic atrophy and deafness. Several other manifestations are frequently associated: neurological, urologic and endocrinous abnormalities as well as growth troubles. Only diabetes mellitus and optic atrophy are compulsory for the diagnosis. This Syndrome is transmitted to the autosomic recessive mode. Mitochondrial heredity is in the course of study. The systematic practice of a cerebral IRM during a SW allows finding several malformative abnormalities of which the most typical is the agenesis of the post-pituitary gland. The prognosis of the affection is essentially conditioned by the evoluting complications of the uropathy and the diabetes mellitus. The treatment is symptomatic. Genetic therapy is in.

Adult↗
PubMedDate not supplied

Acute blindness and putaminal necrosis in methanol intoxication.

PURPOSE: To review the neuro-ophthalmological and radiological findings of acute methyl alcohol intoxication. METHOD: 8 acute methyl alcohol intoxication cases were evaluated. RESULTS: All patients were male and their ages varied between 21 and 55. At the initial examination, 6 to 12 days after methanol intake, visual acuity ranged from no light perception to counting fingers at 2 meters with no color perception. Bilateral dense central scotomas were detected in patients whose vision was slightly preserved. Pupillary light reactions were either absent or sluggish. In 4 cases, edema of the optic disk and the peripapillary nerve fiber layer was observed. Three months later, optic atrophy had developed. Five patients underwent magnetic resonance imaging. Bilateral putaminal hyperintense lesions on T2 weighted images were observed in 3 cases. Two patients died and autopsy permission could not be obtained. Follow-up examination 12 months later revealed optic atrophy in the other six cases, with no improvement in vision. CONCLUSION: Methanol intoxication is detrimental to health, possibly resulting in blindness and occasionally death. In association with ocular signs and the other systemic and laboratory features, the ophthalmologist should be alert to the diagnosis of methanol intoxication in which visual loss may be the only symptom.

Acute Disease↗