World Neurosurg
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Case Reports
Cranial reconstruction for treatment of intracranial hypertension from sclerosteosis: case-based update.
Sclerosteosis (OMIM 269500) is a progressive, autosomal recessive, sclerosing bone disorder with a well defined phenotype. This phenotype is correlated with a deficiency in the protein product sclerostin, leading to bony overgrowth from the loss of inhibition of osteocyte function. Calvarial overgrowth can lead to cranial nerve palsies, visual impairment, and compression of the medulla at the foramen magnum. There is a presumption that calvarial thickening may lead to elevated intracranial pressure in these patients, although pressure measurements have not been published. ⋯ After surgery, the patient had resolution of the headaches and radiographic near-resolution of the syrinx. The authors review their experience and the relevant literature with this rare case.
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Historical Article
Models of functional cerebral localization at the dawning of modern neurosurgery.
The concept of a functional cerebral localization gave the needed support for the development of neurosurgery as a specialty. It should be noted though that the presence of functions on discrete areas of the cortex was a very controversial topic at that time. The objective of this paper is to review models of cortical organization at the end of the 19th century, highlighting beliefs, theories, and controversies behind them. A better understanding of this historical moment is essential to appreciate the debate between holists and localizers that stirred neuroscientists worldwide in the first half of the 20th century.