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November 1983

Neurology of Hereditary Metabolic Diseases of Children

Arch Neurol. 1983;40(12):776. doi:10.1001/archneur.1983.04050110094029

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In this recently published text, Adams and Lyon cut a wide swath through the jungle of childhood metabolic disorders to provide the reader with a clear, safe path to follow in formulating a clinical diagnostic approach to children suspected of having a hereditary metabolic disease. The book restricts its content "to diseases that are hereditary, progressive, and neurologic in their major expression" and that have "a demonstrable biochemical disorder." The authors have limited the scope of information to emphasize the clinical aspects of each disease and to avoid undue length. They have consciously omitted detailed discussions of metabolic pathways, histopathology, and genetics.

The principal objective for the authors in writing the text was to "recapitulate the steps whereby the clinician categorizes a given problem and proceeds in the selection and utilization of laboratory steps preliminary to undertaking therapeutic measures." They clearly achieve this objective, and, in the process, present a

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