Some Problems in the Pathology of Mental Deficiency with Microcephaly

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Abstract
Nine cases of micro-cephaly are described. Microcephalia vera (4 cases) is a disseminated dysgenetic process resulting in a miniature brain which resembles the normal externally except in size but is the seat of complex defects in neuron differentiation. Schizencephalic microcephaly (1 case) is a focal dysgenetic process resulting in focal cerebral and cerebellar mantle defects and defects in the neuraxis. Encephaloclastic microcephaly (4 cases) is the result of regressive changes occurring in fetal or perinatal life. There is usually a complex mixture of tissue destruction and growth disturbances occassioned by the retrograde processes. All types of microcephaly were associated with severe mental deficiency. The skull is moulded to the cranial contents and is not primarily affected when there is reduction of head size. In contrast, 4 mentally deficient patients with unrelieved cranio-synostosis showed only anatomic changes due to pressure, except coincident mongolism in one. This emphasizes the importance of early operation in craniosynostosis.

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