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Focal Cerebral Atrophy in Incontinentia Pigmenti Achromians

Am J Dis Child. 1980;134(7):709-710. doi:10.1001/archpedi.1980.02130190075023.
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Incontinentia pigmenti achromians (IPA), also called hypomelanosis of Ito, is an uncommon cutaneous anomaly. Abnormalities in other organs, including the brain, have been found in about 50% of the patients so far reported.1.2 Focal, as opposed to generalized, neurological symptoms have rarely been described although previous reports have been limited to the dermatological literature and neurological investigations have not been adequate to establish the nature of the brain lesions. This is a description of a patient with IPA who had focal cerebral atrophy.

Report of a Case.—A 13-year-old black boy was referred for neurological evaluation because of the recent onset of seizures. He had been born at full term after an uneventful pregnancy and delivery. The neonatal period was complicated by jaundice and a blood transfusion. At 4 months of age, his mother noticed an irregular area of hypopigmentation on the right thigh, which extended during the next


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