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Visual Loss Due to Progressive Multifocal Leukoencephalopathy in a Congenital Immunodeficiency Disorder
Arch Ophthalmol. 2001;119:1376-1378.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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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] Comment
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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