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Brain, Vol. 110, No. 1, 237-260, 1987
© 1987 Oxford University Press

research-article

ARHINENCEPHALY

THE SPECTRUM OF ASSOCIATED MALFORMATIONS

JOYCE A. KOBORI, MAIE KAARSOO HERRICK and HENRY URICH

From the The Division of Pediatric Neurology, Stanford University School of Medicine, the Department of Pathology (Neuropathology), Santa Clara Valley Medical Center and Stanford University School of Medicine Stanford, California, USA

Correspondence to: Correspondence to: Dr M. K. Herrick, Santa Clara Valley Medical Center, 751 South Bascom Avenue, San Jose, California 95128, USA.

Eight cases are presented of arhinencephaly and its associated malformations, which included 2 examples of holoprosencephaly and 3 of agenesis of the corpus callosum. Additional features included cortical malformations, anomalies of the long tracts and of the optic pathway, cerebellar hypoplasia and dentato-olivary dysplasia. Each of these components covered a wide spectrum ranging in severity from extreme to minimal. Craniofacial dysmorphism, and cardiac, renal and endocrine disorders were present in some cases. Only 2 cases were associated with chromosomal abnormalities, 1 with trisomy 13, the other with PART Ial trisomy 7(7q+). Of possible environmental factors, maternal diabetes was recorded in 1 case. While all cases can be classified into broad categories, the individual variations render each case apparently unique.

Received January 3, 1986. Revised April 24, 1986. Accepted May 6, 1986.


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