Sclerosteosis is a severe autosomal recessive disorder in which progressive bone overgrowth leads to gigantism, cranial nerve entrapment, and raised intracranial pressure. About 60 affected persons have been documented, the vast majority in the Afrikaner population of South Africa. In addition, sporadic cases or affected sibs have been reported from the USA, Switzerland, Japan, and Brazil.
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Peter Beighton (1988) studied this question.
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