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Disturbances in Bulk Growth: Megalencephaly, Micrencephaly, Atelencephaly and Others

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Developmental Neuropathology

Abstract

The term megalencephaly was introduced by Fletcher (1900) to designate a true hyperplasia of brain tissue. Its subsequent usage became rapidly diluted and the term was often used indiscriminately for a variety of lesions causing increased brain weight. In the older literature one finds it used for gliomatosis cerebri or for tuberous sclerosis. An increased bulk of the brain also results from metabolic diseases including gangliosidosis, spongy degeneration, or Alexander disease. In these diseases the increased brain weight is merely a symptom. The diagnostic usage of the term megalencephaly should be reserved for those conditions where there is a true hyperplasia of cerebral parenchyma. Megalocephaly or macrocephaly designate a large head from any of a great number of causes.

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Friede, R.L. (1989). Disturbances in Bulk Growth: Megalencephaly, Micrencephaly, Atelencephaly and Others. In: Developmental Neuropathology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-73697-1_25

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  • DOI: https://doi.org/10.1007/978-3-642-73697-1_25

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