Amiodarone optic neuropathy without disc edema.

A 48-year-old man presented with bilateral blurred vision and visual field changes while prescribed amiodarone. Improvement of vision and visual field defects was documented within 3 weeks after discontinuation of the medication, and complete resolution occurred at 3 months. A unique feature of this amiodarone-associated optic neuropathy is the absence of any optic nerve edema.

Pathologic findings in a steroid-responsive optic nerve infarct in giant-cell arteritis.

OBJECTIVE: To investigate the pathophysiologic mechanism of optic nerve infarction in giant-cell arteritis (GCA). BACKGROUND: Previous pathologic reports of optic nerve infarction in GCA involved patients who were blind at the time of death. The optic nerve infarcts were primarily retrolaminar in localization. Simultaneous short ciliary and ophthalmic artery vasculitis was found in all patients. METHODS: Clinical neurologic and ophthalmologic examination, temporal artery biopsy, and neuroimaging tests were performed in a patient with an anterior ischemic optic neuropathy secondary to GCA. Pathologic examination of the viscera, eye, and brain were performed at autopsy 1 month later. RESULTS: A prelaminar/retrolaminar infarct was found in this patient. Subsiding vasculitis was limited to the short ciliary arteries, sparing the central retinal, pial, and ophthalmic arteries. CONCLUSIONS: The authors believe that the visual improvement observed in this patient was the result of preserved, anterior optic nerve collateral circulation, as well as the neuroprotective and anti-inflammatory effect of the corticosteroids.

Anterior ischemic optic neuropathy in Churg-Strauss syndrome.

A patient with a history of chronic rhinitis, sinusitis, and bronchial asthma developed a unilateral anterior ischemic optic neuropathy and a mild sensory motor neuropathy as the initial manifestation of systemic vasculitis. We made a probable diagnosis of Churg-Strauss syndrome. Fluorescein angiography showed delayed choroidal perfusion in both eyes. Marked eosinophilia, increased sedimentation rate, and elevated perinuclear anti-neutrophil cytoplasmic antibody and anti-myeloperoxidase antibody titers were the main laboratory abnormalities that supported this diagnosis. Therapy with high-dose methylprednisolone and cyclophosphamide may have preserved vision in the better seeing eye, improved its choroidal circulation, and reversed the laboratory abnormalities except for a mild persistent eosinophilia. The neuropathy is gradually improving.

Side effects of glucocorticoid treatment. Experience of the Optic Neuritis Treatment Trial.

OBJECTIVE: To determine the incidence of side effects from short-term glucocorticoid therapy prescribed for treatment of optic neuritis in the Optic Neuritis Treatment Trial. DESIGN: Randomized, placebo-controlled, multicenter clinical trial. SETTING: Fifteen university- or hospital-based centers throughout the United States. PATIENTS: A total of 457 patients between the ages of 18 and 46 years with acute demyelinative optic neuritis were studied. INTERVENTIONS: (1) Intravenous methylprednisolone (250 mg every 6 hours) for 3 days while hospitalized followed by oral prednisone (1 mg/kg per day) for 11 days; (2) oral prednisone (1 mg/kg per day) for 14 days; and (3) oral placebo for 14 days. Each regimen was followed by a short taper. RESULTS: Only two patients experienced major side effects, psychotic depression in one and acute pancreatitis in the other. Both of these patients were from the intravenous methylprednisolone group and both of the side effects resolved without sequelae. Patients in both groups receiving active drugs more often reported sleep disturbances, mood change, stomach upset, and facial flushing and gained more weight during the treatment period than patients in the placebo group (P < .001 for each comparison). CONCLUSIONS: Although minor side effects are common, short-term glucocorticoid therapy in young, healthy adults is relatively safe. Because of the infrequency of serious side effects, outpatient administration of high-dose intravenous glucocorticoids may be feasible.

Papillophlebitis and arteriolar occlusion in a pregnant woman.

Among the pathologic findings affecting the eye during pregnancy, microvascular abnormalities affecting both choroidal and retinal circulation have been reported in cases of complicated pregnancy. We report a case of papillophlebitis and arteriolar occlusion in a pregnant woman without any complications throughout her pregnancy. The patient was placed on glucocorticoids and her vision, visual fields and funduscopic appearance improved almost to normal. Despite the improvement with treatment, the contributing role of glucocorticoids in this case could not be fully determined. Papillophlebitis and arteriolar occlusion should be included among gestational-related vasculopathies.

Chorioretinal lacuna in the amniotic band syndrome.

The malformations in the amniotic band syndrome (ABS) are due to entrapment of fetal parts by fibrous band in the amniotic sac. Limbs are most commonly affected followed by craniofacial defects in one third of patients. Ocular defects include corneal leukomas and lid colobomas often contiguous with facial clefts, strabismus, hypertelorism, and microphthalmos. Unilateral chorioretinal defects or lacunae are rare findings in the ABS. We report a female infant with such a lacunar defect along with central nervous abnormalities, and discuss the differential diagnosis and the embryopathic implications.

Neuroretinitis in cat scratch disease.

We report a patient with neuroretinitis, whose associated lymphadenopathy, exposure to cats, and strongly positive cat scratch antigen skin tests suggested the diagnosis of cat scratch disease. Cat scratch disease should be added to the list of infectious agents believed to produce the clinical picture of neuroretinitis.

Eye movement disorders in men with isolated hypogonadotropic hypogonadism.

The eye movement abnormalities in two men with isolated hypogonadotropic hypogonadism were studied clinically and electro-oculographically. Both demonstrated striking saccadic dysmetria. Subsequent neuroradiologic investigation confirmed atrophy of the cerebellar vermis in one of the patients. This is in concert with other midline structural abnormalities described in patients with isolated hypogonadotropic hypogonadism and suggests that this syndrome may arise from a genetically linked developmental abnormality of midline central nervous system structures.

Accommodation deficiency in healthy young individuals.

Ten patients, ages 10 to 19, with accommodation insufficiency are reported. All patients were in good health and asymptomatic, except for asthenopia during near visual activities. Each patient's amplitude of accommodation was measured and found to be considerably below the minimal normal for their respective ages (an average of 6 diopters). Only three patients had associated convergence insufficiency. No etiology for the diminished accommodation was suggested by history or could be identified by careful examination. All patients were successfully managed optically with bifocals or reading glasses, although three required the addition of base-in prisms for the near exodeviation. Near vision testing and determination of the near point of accommodation should be part of the pediatric ophthalmologic examination in all patients with complaints referable to their reading and visual performance at near.

Ocular findings in partial trisomy 3q. A case report and review of the literature.

The authors report the ocular findings in a patient with partial trisomy 3 [46, XY-15, der(15), t(3;15) (q26;128) mat] and review the literature on this chromosomal anomaly. The patient's main ocular finding was infantile glaucoma. The patient's mother was phenotypically normal and carried a balanced chromosome translocation. Awareness of the ocular abnormalities associated with partial trisomy syndromes is essential since more children with these syndromes survive today than in the past.

Glucose utilization of visual cortex following extra-occipital interruptions of the visual pathways by tumor. A positron emission tomography study.

To assess the effect of extra-occipital lesions on the local cerebral glucose utilization of the primary and associative visual cortex, 29 patients were studied in the unstimulated state by positron emission tomography and [18F]2-deoxyglucose. Quantitative Goldmann perimetry was done in each patient at the time of the positron emission tomographic study. Nine patients showed homonymous defects, either hemianopsia or quadrantanopsia, whereas nine patients had heteronymous defects. Eleven control subjects, free of any neurological symptoms and with normal visual fields, were also studied with [18F]2-deoxyglucose positron emission tomography. In the normal control subjects and in patients with a heteronymous defect, left-to-right differences in the local cerebral metabolic rate for glucose of the visual cortex varied less than 10%. In patients with hemianopic defects, differences ranged from 8 to 38%, with the hypometabolic cortex always contralateral to the field defect. In patients with quadrantanopic defects, the visual cortex contralateral to the field defect demonstrated differences from 14 to 24% above and below the calcarine fissure, the cortex that received greater input from the affected field being hypometabolic.

Spasm of the near reflex associated with organic disease.

Spasm of the near reflex is characterized by transient attacks of convergence, accommodation, and miosis. It is usually observed in young individuals and considered functional. We studied seven patients with spasm of the near reflex who had associated neurologic disorders or head trauma. Two patients had posterior fossa abnormalities (cerebellar tumor, Arnold-Chiari malformation), two patients had pituitary tumors, one patient had a vestibulopathy, and two patients had a history of antecedent head trauma.

Two cases of downbeat nystagmus and oscillopsia associated with carbamazepine.

Downbeat nystagmus is often associated with structural lesions at the craniocervical junction, but has occasionally been reported as a manifestation of metabolic imbalance or drug intoxication. We recorded the eye movements of two patients with reversible downbeat nystagmus related to carbamazepine therapy. The nystagmus of both patients resolved after reduction of the serum carbamazepine levels. Neuroradiologic investigations including magnetic resonance imaging scans in both patients showed no evidence of intracranial abnormality. In patients with downbeat nystagmus who are taking anticonvulsant medications, consideration should be given to reduction in dose before further investigation is undertaken.

Prevention of ocular toxicity of carmustine (BCNU) with supraophthalmic intracarotid infusion.

Intracarotid administration of carmustine (BCNU) is an increasingly accepted therapeutic modality for primary brain tumors. Its beneficial effect on prolonging survival, however, is countered by its frequent delayed complication of retinal toxicity and severe visual loss, which reportedly occurs in approximately 70% of the patients. Since this complication probably results from increased flow of the drug into the ophthalmic artery, the authors have attempted to infuse BCNU via a small intracarotid catheter advanced beyond the origin of the ophthalmic artery. In a series of nine patients (average follow-ups, 10.7 months), the authors were successful in treating six patients with exclusively supraophthalmic infusions. None of these patients developed any ocular toxicity. From the remaining three patients, two patients received one infraophthalmic infusion with no retinal toxicity. The third patient, however, who received three infraophthalmic infusions, developed ipsilateral retinopathy. These results suggest that retinopathic complications of intracarotid BCNU can be avoided if the infusion catheter is advanced beyond the origin of the ophthalmic artery.

Near-evoked nystagmus in spasmus nutans.

Near-evoked nystagmus was evident in two children with spasmus nutans by clinical observation and electro-oculographic recording. In one child the nystagmus appeared to be evoked by fusional convergence and in the other by convergence-accommodation. These cases represent an atypical form of spasmus nutans in which the nystagmus is modulated by centers controlling visuomotor changes with near viewing.

Weill-Marchesani syndrome with bilateral angle-closure glaucoma.

To our knowledge this is the first reported case of cyclopentolate induced bilateral acute angle-closure glaucoma associated with the Weill-Marchesani syndrome without lens subluxation. The diagnosis of Weill-Marchesani syndrome was not initially suspected, and Pilocarpine was used in an attempt to break the acute attack. Treatment with Pilocarpine-induced myosis only worsened the glaucoma by causing a severe pupillary block. This case is important because it demonstrates that mid-dilatation and cycloplegia can induce acute glaucoma in patients with the Weill-Marchesani syndrome and points out the importance for considering the diagnosis of Weill-Marchesani syndrome in any case of acute glaucoma in childhood.

Habitual head turning in spasmus nutans: an oculographic study.

Head turning in patients with spasmus nutans seems to transiently dampen the nystagmus. This was evident in two children by clinical observation and electrooculographic recording. The suppression of the nystagmus may have been mediated by the vestibular system.