Serial Enzyme Studies of Serum and Cerebrospinal Fluid in Amaurotic Family Idiocy

The diagnostic utility of enzyme level analysis in biologic fluids has only recently been explored on a broad basis.^1,2,3 Particular emphasis has been placed upon the circulating glycolytic and transaminating enzymes. Based upon the supposition that for each determinable enzyme, there exists a specified pattern of concentration within individual tissues, it has been suggested and experimentally demonstrated that a rise in the serum content of any particular enzyme is a reflection of necrobiosis in such organs characterized by high concentration of that enzyme. It is further presumed that the enzyme elevation in serum represents a liberation from affected tissues rather than an elaboration. This is strengthened by the observation that the rise in circulating enzyme parallels the period of active destruction of the specified tissue and that such enzyme tests are of no avail as a measure of previous or quiescent degeneration. This concept, exemplified by the term "chemical