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September 1966

The Chronic Brain Syndrome of Infantile Hydrocephalus: A Follow-Up Study of 63 Spontaneously Arrested Cases

Author Affiliations

From the Department of Pediatric Neurology, University Hospital of Uppsala, Sweden.

Am J Dis Child. 1966;112(3):189-196. doi:10.1001/archpedi.1966.02090120057004

Developmental and constitutional deviations, neurological sequelae, and disturbances of mentality have been studied in 63 cases of spontaneously arrested hydrocephalus of different etiologies. The patients were 6 to 24 years old at the time of follow-up Most of them were late in passing the first developmental milestones. In general, floppiness and poor motor performance, particularly in the lower limbs, were characteristic signs in early life. Ataxia, the predominant neurological sign, was present in 41 patients (65%). It was combined with spastic diplegia in 11 subjects (17%), most of whom had other signs supporting a more advanced degree of brain damage. Tetraplegia predominated among the most severely disabled. Squint was noted in 38 cases and poor vision or blindness in 13. A dysplastic body build interpreted as due to hypothalamic dysfunction was found in 23 patients. In 23 subjects the IQ was above 90. Twenty-four subjects were educable but retarded, while 16 were imbeciles or idiots. Behavior deviations were common. A very characteristic behavior pattern called the "cocktail-party syndrome" is described. It was present in 25%.

It is concluded that untreated hydrocephalus apparent during early infancy may in a large percentage of survivors be followed by a very characteristic chronic brain syndrome. The special clinical signs of this syndrome are considered to be due mainly to the adverse influence of brain expansion in early life.

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