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  • Title: Congenital hydrocephalus and clasped thumbs: two cases of brothers in a family.
    Author: Mochizuki Y, Suyehiro Y, Ihara Y, Tomimoto K, Saito A, Ito T.
    Journal: Brain Dev; 1981; 3(4):407-9. PubMed ID: 7316097.
    Abstract:
    Two cases of brothers with congenital hydrocephalus and clasped thumbs are reported. In consideration of the rare incidence of sporadic congenital hydrocephalus, hereditary origin of these cases based on X-linked recessive inheritance was suspected. Serial sectioning of the brain stem revealed stenosis and abnormal dorso-ventral elongation of the aqueduct. These findings support the postulation of Landrieu et al that the primary defect in X-linked hereditary hydrocephalus is a communicating hydrocephalus leading usually to aqueductal stenosis in consequence of lateral compression of the brain stem.
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