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February 1937

TWO CASES OF CONGENITAL ANOMALIES OF THE BRAIN

Author Affiliations

AMSTERDAM, NETHERLANDS
From the Pediatric Department (Professor Cornelia de Lange) and the Neurologic Laboratory (Professor B. Brouwer) of the University of Amsterdam.

Am J Dis Child. 1937;53(2):429-444. doi:10.1001/archpedi.1937.04140090002001
Abstract

Some years ago this journal published a report of my observations on three children presenting congenital hypertrophy of the muscles, extrapyramidal motor disturbances and mental deficiency.1 The report also cited a fourth case, found in the literature. After the death of the first child examination of the brain was possible. Underdevelopment or inhibition of the striatum, especially the neostriatum, was observed. Moreover, there was a widespread porencephalic process, regarded as secondary. In my opinion the four children presented in their symptoms a clinical entity, though it is not yet possible to explain the relationship between the malformation of the striate body and the muscular hypertrophy.

Since publication of the article, there have been sent to the Children's Clinic of the University of Amsterdam two infants considered by the attending physicians as having the same triad of symptoms. Though the diagnoses are not accepted as entirely correct, the two cases

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