Journal Article

Blepharoptosis and External Ophthalmoplegia Associated with Long-Term Antiretroviral Therapy

Warren L. Dinges, S. Robert Witherspoon, Kamel M. Itani, Abhimanyu Garg and Dolores M. Peterson

in Clinical Infectious Diseases

Published on behalf of Infectious Diseases Society of America

Volume 47, issue 6, pages 845-852
Published in print September 2008 | ISSN: 1058-4838
Published online September 2008 | e-ISSN: 1537-6591 | DOI: http://dx.doi.org/10.1086/591202
Blepharoptosis and External Ophthalmoplegia Associated with Long-Term Antiretroviral Therapy

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Background.Long-term antiretroviral therapy (ART) is associated with lipodystrophy, peripheral neuropathy, lactic acidosis, and myopathy. Blepharoptosis, without prior ART association, is usually caused by age-associated involutional ptosis, but it is also seen in mitochondrial myopathies with external ophthalmoplegia, cardiac conduction disturbances, and neurological impairments.

Methods.Patients presented over a 2-year period. Four patients underwent surgical blepharoptosis repair.

Results.Five human immunodeficiency virus type 1–infected patients (median age, 50 years; range, 46–53 years) who were receiving ART presented with severe blepharoptosis; 2 of these 5 also presented with external ophthalmoplegia. Findings included decreased palpebral fissure height (median, 6.5 mm; normal height, 9 mm), mildly impaired levator function (median, 10 mm; normal, >13 mm), and markedly decreased marginal reflex distance (median, 0.5 mm; normal, 4 mm). A greater advancement of the levator aponeurosis was required during surgical repair, a finding consistent more with myogenic than with involutional blepharoptosis. All patients had severe lipodystrophy, which preceded blepharoptosis by a median interval of 4.7 years (range, 2.8–5.7 years). Four patients also presented with peripheral neuropathy and metabolic abnormalities before the onset of blepharoptosis, and 3 had cardiac conduction disturbances. Patients received ART for a median of 7.8 years (range, 4.9–11.2 years), thymidine analogue–containing ART for a median of 7.1 years (range, 1.2–7.9 years), and protease inhibitor–containing ART for a median of 7.1 years (range, 4.9–8.9 years).

Conclusions.We report the novel findings of blepharoptosis and external ophthalmoplegia in patients who are receiving ART. Ptosis was preceded by lipodystrophy with long-term use of both thymidine-analogue– and protease inhibitor–containing ART. The findings are most consistent with myogenic ptosis in a generalized mitochondrial myopathy syndrome. Clinicians should also be watchful for other potential myopathic ptosis-associated complications, including proximal weakness, dysphagia, deafness, and cardiac conduction disturbances.

Journal Article.  4084 words.  Illustrated.

Subjects: Infectious Diseases ; Immunology ; Public Health and Epidemiology ; Microbiology

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