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Neuropediatrics 1981; 12(3): 279-286
DOI: 10.1055/s-2008-1059658
© Georg Thieme Verlag KG Stuttgart · New York

AlCARDI's SYNDROME - REPORT OF AN AUTOPSY CASE

H.  Ohtsuki1 , H.  Haebara1 , K.  Takahashi1 , O.  Midorikawa1 , E.  Tomoyoshi2 , S.  Torii2 , K.  Miura3
  • 1Department of Pathology, Kyoto City Hospital, Mibu Higashi-Takada-cho, Nakagyo-ku, Kyoto 604, Japan
  • 2Section of Pediatrics, The Japan Baptist Hospital, Kita-shirakawa Yamanomoto-cho, Sakyo-ku, Kyoto 606, Japan
  • 3Section of Ophthalmology, The Kyoto Teishin Hospital, Shin-machi Rokkaku, Nakagyo-ku, Kyoto 604, Japan
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

A Japanese girl aged 19 months had generalized convulsions, agenesis of the corpus callosum, peculiar chorioretinopathy, scoliosis and marked retardation of psychomotor development. Pathological investigation revealed polymicrogyria, cortical heterotopias, disorganization of the cerebellar cortex. In ocular histopathology, lacunae were composed of hypo- or depigmented zones of the retinal pigment epithelium accompanied with dispersion of pigments into the rod and cone layer. Coloboma of the optic nerve was also evident. There was no evidence of inflammation or pathogenic organisms.

Key words

Aicardi's syndrome - convulsions - agenesis of the corpus callosum - chorioretinopathy

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