1979). Aust. Paediatr. J.. 278-280. the Aicardi syndrome in a 47, XXY male. The Aicardi syndrome is reported for the first time in a male child. His chromosomal analysis revealed a 47, XXY karyotype. This finding supports the suggestion that this condition is due to a newly mutated X-chromosomal dominant gene. The existence of a 47, XXY chromosomal constitution in this male allows heterozygous expressions of the gene as in the female.

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A review of dysembryoplastic neuroepithelial tumors (DNTs) in 14 patients over a 12-year period revealed four patients re-operated because of changes on magnetic resonance imaging (MRI) suggesting tumor recurrence or progression. In three of these, the histological features were identical to the initial DNT. In the fourth patient, persistent DNT was surrounded by WHO grade 2 oligoastrocytoma. In one of the other 10 patients, WHO grade 2 oligodendroglioma was present in white matter deep to and completely separate from a cortically based DNT. Fluorescence in situ hybridization showed codeletion of 1p and 19q in both the DNT and oligodendroglioma and oligoastrocytoma components. Deletions were not identified in any other tumor. Our findings corroborate other studies that 1p and 19q deletions are uncommon in DNT. These two unusual tumors also raise the possibility that rare DNTs may evolve into oligodendroglioma or oligoastrocytoma. DNTs with this altered biology can be identified by 1p and 19q deletion analysis.