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July 1980

Focal Cerebral Atrophy in Incontinentia Pigmenti Achromians

Author Affiliations

Departments of Neurology and Pediatrics; Department of Medicine; Department of Medicine Louisiana State University Medical Center PO Box 33932 Shreveport, LA 71103

Am J Dis Child. 1980;134(7):709-710. doi:10.1001/archpedi.1980.02130190075023

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