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December 1989

Aicardi's Syndrome

Author Affiliations

Tucson, Ariz

Arch Ophthalmol. 1989;107(12):1724. doi:10.1001/archopht.1989.01070020806006

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To the Editor.  —In the June 1989 issue of the Archives, Gloor et al1 described a 19-month-old black female infant with a medical history of microcephaly, developmental delay, hypotonia, and seizures. The case was beautifully illustrated.Magnetic resonance imaging showed an arachnoidal cyst and partial agenesis of the corpus callosum. Funduscopic examination, according to the authors, revealed bilateral colobomatous discs. However, careful evaluation of the printed fundus photographs does not show optic disc colobomas but, rather, bilateral optic disc dysplasia and atypical peripapillary pigmentary changes. Moreover, the typical punched-out chorioretinal lesions were not present. These "punched-out" lesions are thought to be diagnostic of Aicardi's syndrome.2 Also, the electroencephalogram (EEG) in the case did not show a pure hypsarrhythmia, highly characteristic of the Aicardi syndrome,3,4 but a "pattern akin to it."Corpus callosum agenesis, arachnoidal cysts, and other neuroradiological midline anomalies have been described in patients with a

Gloor P, Pulido JS, Judisch GF.  Magnetic resonance imaging and fundus findings in a patient with Aicardi's syndrome . Arch Ophthalmol . 1989;107:922-923.Article
Aicardi J, Chevrie JJ, Rousselie F.  Le syndrome spasmes en flexion, agenesie calleuse, anomalies chorio-retiniennes . Arch Fr Pediatr . 1969;26:1103-1120.
3. Fariello RG, Chun RW, Doro JM, Buncic JR, Prichard JS.  EEG recognition of Aicardi's syndrome . Arch Neurol . 1977;34:563-566.Article
Kellaway P, Frost JD Jr, Hrachovy RA.  Infantile spasms . In: Morselli PL, Pippenger CE, Penry JK, eds. Antiepileptic Drug Therapy in Pediatrics . New York, NY: Raven Press; 1987:115-136.