Aicardi syndrome
- Department of Ophthalmology, Harrogate District Hospital, Lancaster Park Road, Harrogate HG2 7SX
- Department of Neurology, Harrogate District Hospital, Lancaster Park Road, Harrogate HG2 7SX
- Department of Ophthalmology, Clarendon Wing, The General Infirmary at Leeds, Belmont Grove, Leeds LS2 9NS
- Accepted 6 November 1997
- Department of Ophthalmology, Harrogate District Hospital, Lancaster Park Road, Harrogate HG2 7SX
- Department of Neurology, Harrogate District Hospital, Lancaster Park Road, Harrogate HG2 7SX
- Department of Ophthalmology, Clarendon Wing, The General Infirmary at Leeds, Belmont Grove, Leeds LS2 9NS
- Accepted 6 November 1997
- Department of Ophthalmology, Harrogate District Hospital, Lancaster Park Road, Harrogate HG2 7SX
- Department of Neurology, Harrogate District Hospital, Lancaster Park Road, Harrogate HG2 7SX
- Department of Ophthalmology, Clarendon Wing, The General Infirmary at Leeds, Belmont Grove, Leeds LS2 9NS
- Dr A M King, Department of Ophthalmology, Harrogate District Hospital, Lancaster Park Road, Harrogate HG2 7SX
- Accepted 6 November 1997
Editor,—Aicardi syndrome1 is a congenital disorder characterised by severe epilepsy, agenesis of the corpus callosum, typical chorioretinal lacunae, and learning disabilities. Normally it carries a poor prognosis. We present a case in which these symptoms have been found in a 49 year old woman, suggesting a “forme fruste” of the disorder.
CASE REPORT
A 49 year old woman previously thought to have cerebral and retinal toxoplasmosis was referred because of visual deterioration, poor balance, and obvious visual field restriction.
On examination her corrected visual acuities were right 0.2 (6/9.5) and left 0.8 (6/38) (Bailey-Lovie chart). The fundi of both eyes showed large chorioretinal defects adjacent to the discs and smaller rounded pale yellow “punched out” chorioretinal lacunae scattered around the posterior pole, as is typical in Aicardi syndrome. Her discs were hypoplastic with marginal pigmentation …
