Aqueductal Stenosis in X‐Linked Hydrocephalus: a Secondary Phenomenon?

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Abstract
Two cases of X-linked hydrocephalus are reported. One underwent post mortem examination: serial sections of the brain stem failed to show stenosis of the aqueduct, the mean and minimal cross-sectional areas of which were normal. There were some morphological changes which were compatible with a mechanical compression of the brain stem. In the Bickers-Adams syndrome of X-linked hereditary hydrocephalus, the primary defect may be a communicating hydrocephalus leading usually, but not always, to aqueductal stenosis. The importance of electrophysiological investigations for the correct diagnosis of the characteristic clasped-thumb deformity is also indicated.