Biomedical subjects
Publications and source records attributed to G A Chrousos.
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.
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.
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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.
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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.
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.
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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.
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.
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.
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.
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.
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.
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.
The eyes of 392 consecutive childhood cataract aspiration procedures were analysed for frequency of the development of chronic glaucoma and retinal detachment and the performance of secondary membrane surgery. Three different modifications of the aspiration procedure were employed: the standard needle and syringe procedure leaving the posterior capsule intact (304 eyes), rotoextraction with small opening of the posterior capsule (34 eyes) and Ocutome aspiration with wide excision of the posterior capsule (54 eyes). The mean postoperative follow-up was 5.5 years. Chronic glaucoma was found in 6.1% of the eyes. Coexisting ocular anomalies and retained lens cortex increased the risk for this complication. Retinal detachment was found in 1.5% of the cases. Coexisting ocular anomalies and uncontrolled vitreous disturbance increased the possibility of retinal detachment. Secondary membrane procedures were performed in 62% of the eyes in which the aspiration procedure left the posterior capsule intact, in nearly 12% of those in which the posterior capsule was minimally opened and in none of those which had the posterior capsule widely excised. The secondary membrane surgery itself appeared to increase the risk for both glaucoma and retinal detachment. The aspiration procedure with the lowest incidence for all three complications was the Ocutome aspiration with wide excision of the posterior capsule.
We performed complete ophthalmological examinations of 30 consecutive patients with Turner syndrome. Twenty-three had 45XO and 7 had 45XO/46XX karyotypes (mosaicism). Non-familial strabismus was the most prominent ocular abnormality and was present in 33% of the patients. Other eye findings included ptosis (16%), hypertelorism (10%), epicanthus (10%), and antimongoloid slants (10%). Red-green color deficiency was found in 10% of the patients. One patient had congenital periodic alternating nystagmus.
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