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Progressive Pigmentation of Chorioretinal Lesions in Aicardi Syndrome
Luis H. Ospina, MD;
Harish Nayak, MD;
Andrew Q. McCormick, MD, CM
Vancouver, British Columbia
Arch Ophthalmol. 2004;122:790.
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A 3-month-old female infant was referred to the ophthalmology unit as part of the investigation for infantile spasms. Ocular examination showed normal visual attentiveness and pupillary function. The optic nerves were vertically oval and there were multiple chorioretinal lacunae adjacent to the discs (Figure 1). Neuroimages revealed absence of the corpus callosum. Aicardi syndrome was diagnosed. By the age of 6 years devastating neurological deterioration had occurred. Reexamination of the retinas at this time revealed increased pigmentation of the retinal lesions, even within the lacunae (Figure 2).
Figure appears in full text version.
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Figure 1. Vertically oval optic nerve and multiple chorioretinal lacunae adjacent to the disc.
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Figure appears in full text version.
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Figure 2. Increased pigmentation of the retinal lesions.
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COMMENT
In 1965 Aicardi and associates1 described a syndrome consisting of callosal agenesis, chorioretinal lacunae, and infantile spasms. Since then many cases of this X-linked recessive . . . [Full Text of this Article]
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