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  Vol. 119 No. 9, September 2001 TABLE OF CONTENTS
  Archives
  •  Online Features
  Clinicopathologic Reports, Case Reports, and Small Case Series
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Visual Loss Due to Progressive Multifocal Leukoencephalopathy in a Congenital Immunodeficiency Disorder

Arch Ophthalmol. 2001;119:1376-1378.

Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

A 20-year-old man with Wiskott-Aldrich syndrome (WAS) initially developed a mild visual disturbance that progressed to blindness, increasing neurological deficits, and death within 4 months. Wiskott-Aldrich syndrome is an X-linked immunodeficiency disorder characterized by thrombocytopenia, eczema, and susceptibility to infection.1 This case illustrates the difficulties in reaching the final diagnosis of progressive multifocal leukoencephalopathy (PML) in this individual and its unusual histopathologic features.

Report of a Case

A 20-year-old white man with WAS had a 1-month history of decline in vision. Nine months previously, he had omitted 3 consecutive doses of immunoglobulin, which he had been receiving every 3 weeks since the age of 12 years. This was restarted when he developed lethargy and malaise. His mother had been diagnosed with multiple sclerosis at age 26 years.

An ophthalmological examination revealed a visual acuity of 20/17 OD and 20/20 OS with normal pupillary reactions. Color vision was abnormal, and visual field testing showed bilateral . . . [Full Text of this Article]


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Corresponding author: Susan M. Downes, MD, FRCOphth, Oxford Eye Hospital, Radcliffe Infirmary, Woodstock Road, Oxford OX2 6HE, England (e-mail: susan.downes@ophthalmology.oxford.ac.uk).







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