Monocular temporal hemianopia associated with optic nerve hypoplasia.
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Biomedical subjects
Publications and source records attributed to Eric R Eggenberger.
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BACKGROUND: The neuroimaging evaluation of isolated pain in or around the eye has not been studied previously. We report the low diagnostic yield of neuroimaging in patients who have a normal ocular examination and unilateral eye pain or facial pain predominantly affecting the eye. METHODS: Retrospective review of patients referred to 3 neuro-ophthalmology practices for unexplained pain in or around the eye. Inclusion criteria were adults with isolated unilateral eye/facial pain, neuroimaging, and a normal eye exam. Ex-clusion criteria were symptoms typical of a defined pain syndrome (e.g., trigeminal neuralgia or giant cell arteritis), and exam findings that would account for the pain. RESULTS: One hundred and twenty-seven (127) of the 760 reviewed patients met study criteria, and underwent MRI (75) or CT (34) scans, or both (18). Imaging was normal in 106 (83%). Abnormalities (n = 21) on imaging (17%) included nonspecific T2-weighted hyperintensities (10), sinusitis (5), superior ophthalmic vein enlargement (1), pontine lacunar infarct (1), Chiari malformation (1), thalamic mass (1), old occipital stroke (1), and focal enlargement of the third cranial nerve (1). INTERPRETATION: Although imaging showed abnormalities in 17% of cases of isolated pain in or around the eye, only 2 abnormalities were believed to be possibly related to the pain and only 1 case was probably related. The diagnostic yield of neuroimaging in patients with a normal examination and isolated, unilateral eye/facial pain referred to a neuro-ophthalmologist is low.
Anisocoria, or a difference in pupil size, is a common condition. Its aetiology ranges from benign to life-threatening conditions. The clinical evaluation of anisocoria is discussed, emphasising the pharmacological aids (e.g., cocaine 10% eye drops, hydroxyamphetamine eye drops, pilocarpine 0.1% eye drops, pilocarpine 1% eye drops, apraclonidine) used in differentiating the different causes of anisocoria (e.g., physiological anisocoria, Horner syndrome, Adie pupil, pharmacological anisocoria, third nerve palsy).
PURPOSE: To report results of treatment with a monoclonal antibody (infliximab) directed against tumor necrosis factor alpha in seven patients with chronic and difficult-to-control idiopathic orbital inflammation (orbital myositis). DESIGN: Observational case series. METHODS: Retrospective data were collected from seven patients who had idiopathic orbital inflammation and who were evaluated at three medical centers. All patients were treated with infliximab after the failure of traditional therapy, which included corticosteroids, radiotherapy, or anti-inflammatory chemotherapeutic agents. RESULTS: All seven patients had a favorable response to treatment with infliximab. One patient with Behcet disease required supplemental oral corticosteroids. Pain, swelling, and need for concomitant corticosteroids were the primary measures of treatment success. Symptoms of comorbid disease in four patients also improved (Crohn disease in two, Behcet disease in one, and psoriasis in one). There were no untoward effects of treatment after a mean follow-up of 15.7 months (range, 4 to 31 months). CONCLUSIONS: Treatment with infliximab appears to offer another therapeutic option in cases of recalcitrant or recurrent idiopathic orbital inflammation in which conventional treatment fails.
PURPOSE: To determine if the monocular vertical prism dissociation test can differentiate between organic and nonorganic visual loss. DESIGN: A prospective, single-masked observational study. METHODS: Three institutional neuro-ophthalmology practices. Group 1 consisted of 30 normal controls. Group 2 included 30 patients with known organic visual loss. Group 3 contained 35 patients with suspected nonorganic monocular visual loss. Participants were asked to describe what they saw while viewing a single Snellen letter when a 4-prism diopter base-down prism was placed in front of their better eye. Outcome was measured by whether the participant sees one or two letters with the prism in place. RESULTS: Vision-appropriate results were given by all members of Group 1 (two images) and by all members of Group 2 (one image). Two images were seen by 31 of 35 members of Group 3, indicating nonorganic visual loss. The other 4 subjects in Group 3 saw one image; each was subsequently found to have occult pathology. CONCLUSION: The vertical prism test quickly differentiates organic from nonorganic monocular visual acuity loss.
Neurologic autoimmunity frequently occurs with thymoma, particularly myasthenia gravis and skeletal muscle-specific autoantibodies. Type 1 antineuronal nuclear antibody (ANNA-1/"anti-Hu"), which is recognized as an immunoglobulin G marker of small-cell lung carcinoma, has not been reported with thymoma. The authors identified four patients (three under age 40) with ANNA-1 and a paraneoplastic neurologic complication of thymoma. Retrospective testing of stored serum from 172 patients with thymoma revealed ANNA-1 in 3%. This report extends the oncologic implications of ANNA-1 seropositivity.
PURPOSE: To report a causal relationship between Chiari I malformation and its rare, but recognized manifestation of bilateral papilledema. DESIGN: Interventional case series. METHODS: Four adult female patients (mean age, 48, age range 25-59 years) with bilateral papilledema, signs and symptoms of increased intracranial pressure, and cranial magnetic resonance imaging (MRI) evidence of a Chiari I malformation ranging from 7 to 22 mm of tonsillar herniation underwent suboccipital decompression. RESULTS: In all four patients, suboccipital decompression was followed by resolution of bilateral papilledema and signs and symptoms of increased intracranial pressure. CONCLUSION: Patients with bilateral papilledema and presumed pseudotumor cerebri require a cranial MRI to determine if they have a Chiari I malformation, because patients with increased intracranial pressure and papilledema from a Chiari I malformation may benefit from suboccipital decompression.
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