Chiasmal enlargement and optic nerve enhancement on magnetic resonance imaging in leber hereditary optic neuropathy.
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Biomedical subjects
Publications and source records attributed to Andrew G Lee.
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PURPOSE: To determine the feasibility of screening for depression, dementia, and functional impairment in an ophthalmology outpatient clinic. DESIGN: Prospective pilot survey study. PARTICIPANTS: Fifty consecutive ophthalmology clinic outpatients. METHODS: Consecutive patients more than 64 years of age with visits to the comprehensive eye clinic and the neuroophthalmology clinic at the University of Iowa were assessed. Suitable subjects were asked to complete a short questionnaire on instrumental activities of daily living, to answer a single question on depression, and to complete a clock drawing task in the office waiting room or examination room. RESULTS: The 50 questionnaires reviewed showed that most respondents were functional (94%) in instrumental activities of daily living, had normal clock drawing results (80%), and were not depressed (80%). A significant minority, however, were identified by the screening to have functional impairment (6%), abnormal clock drawing results (20%), or depression (20%), and these patients were offered geriatric assessment. The tests were rapid (fewer than 5 minutes to perform) and easy to administer. CONCLUSIONS: Screening for geriatric syndromes in the eye clinic was rapid, easy to perform, and detected a significant number of patients with functional disability, depression, and possible dementia.
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A 63-year-old man who underwent uneventful trans-sphenoidal resection of a pituitary adenoma with fat packing complained postoperatively of progressive binocular visual acuity loss. Neuroimaging showed a suprasellar pneumatocele compressing the optic chiasm and a communication between the sphenoid sinus and the sella. After a second trans-sphenoidal procedure to remove the air and fully pack the sphenoid sinus, visual acuity recovered dramatically. A rare complication of trans-sphenoidal surgery for pituitary adenoma, suprasellar pneumatocele probably forms through a ball-valve mechanism that results from incomplete packing of the sellar floor. This case highlights the need for effective sphenoid sinus packing and for ophthalmic monitoring after trans-sphenoidal surgery.
BACKGROUND: Within the past 10 years, several immunomodulatory agents (IMAs) have become available for the treatment of relapsing multiple sclerosis (MS), making therapeutic decisions more complex. We performed a systematic review of the literature to assess the efficacy and safety of these agents on physical, inflammatory, and cognitive measures of disease activity. METHODS: We identified relevant studies by searching electronic databases (MEDLINE and Current Contents) from January 1, 1993, through August 31, 2001. We included English-language reports of data from phase 3 trials of interferon beta-1b (Betaseron), 2 preparations of interferon beta-1a (Avonex and Rebif), or glatiramer acetate (Copaxone) for the treatment of relapsing MS. RESULTS: Twenty-one studies met explicit inclusion criteria. Comparison of study results indicated no differences among IMAs regarding their efficacy on relapse-related measures. Interferon beta-1a significantly reduced disability progression, whereas no significant effect of glatiramer acetate or interferon beta-1b on disability progression was seen. On inflammatory measures, all of the IMAs showed reductions in the burden of disease (T2-weighted lesions) to varying degrees. Interferon beta and glatiramer acetate reduced new lesion activity; however, interferon beta had a more profound effect. One interferon beta-1a preparation (Avonex) appeared to reduce brain atrophy, whereas glatiramer acetate showed an effect in 1 of 2 studies. Only Avonex demonstrated efficacy in slowing progression of cognitive dysfunction. CONCLUSIONS: Data show that the IMAs have similar effects on several physical and inflammatory measures. In addition, Avonex has demonstrated efficacy in slowing cognitive progression in relapsing MS. One disadvantage of interferon beta is the possibility of immunogenicity, which may occur more often with subcutaneous administration. The IMAs have similar safety and tolerability profiles.
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Chronic progressive external ophthalmoplegia (CPEO) is a descriptive term for a heterogenous group of disorders characterized by chronic, progressive, bilateral, and usually symmetric ocular motility deficit and ptosis. Significant pain, proptosis, or pupil involvement are not features of CPEO and should prompt evaluation for alternative etiologies. Mitochondrial DNA mutations are increasingly being recognized as the etiology for CPEO syndromes. Clinicians should recognize the specific syndromes associated with CPEO, characterized by variable systemic, neurologic, or other findings. Treatment is limited, but newer therapies are being investigated.
PURPOSE: To describe diplopia as the initial manifestation of the Burkitt lymphoma. DESIGN: Observational case report. METHODS: Retrospective chart review. RESULTS: A 53-year-old human immunodeficiency virus (HIV)-positive man presented with a severe headache associated with binocular diplopia. Flow cytometric analysis of the cerebrospinal fluid demonstrated a monoclonal B-lymphoid cell population consistent with the Burkitt lymphoma. CONCLUSION: Ophthalmologists should be aware that diplopia may be the presenting manifestation of Burkitt lymphoma in an immunocompromised patient.
PURPOSE: We report a case of Leber hereditary optic neuropathy in a patient who was using ephedra alkaloids at the time of onset of his optic neuropathy. DESIGN: Observational case report. METHODS: Bilateral, painless, progressive loss of vision developed in a 30-year-old man. He reported a one pack-per day, 10-year history of tobacco use and drinking 18 to 24 cans of beer per day as well as the use of a dietary supplement containing ephedra and caffeine before the onset of vision loss. RESULTS: We report the bilateral, progressive, painless loss of vision in a patient with bilateral cecocentral scotomas and optic nerve pallor. Mitochondrial DNA testing confirmed a Leber hereditary optic neuropathy mutation site at loci 11778. CONCLUSIONS: We propose that our patient's use of a dietary supplement containing ephedra alkaloids played an additive role to his tobacco and alcohol use in causing stress to the already abnormal mitochondrial function, and thereby contributed to the onset of his optic neuropathy. We recommend that patients and families with known Leber hereditary optic neuropathy should be asked about the use of over-the-counter drugs or supplements, and it may be prudent to advise patients against using such dietary supplements.