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Juvenile amaurotic family idiocy: Features suggestive of precocious senility

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Summary

A clinical and pathological study of juvenile amaurotic idiocy is presented with an illustrative case report. The case described had the classical criteria of retinitis pigmentosa, progressive mental deterioration and a convulsive component as evidenced by the electro-encephalogram.

Pathological and histochemical studies indicate pigmentary and neuronal changes similar to those associated with alterations of the senile brain. The neuronal deposits are of a lipochrome nature, similar to, if not identical with, lipofuscin. The concept of a precocious senility is advanced to stimulate comparative studies with the dementias of senility.

It is pointed out that, regardless of the etiology—either of lipoid disorder, congenital deficiency of the neuro-ectodermal germ layer, or arrested physiological maturity of the nerve cells—a final clinicopathological state of senility is reached in the nervous system of juvenile amaurotic family idiocy.

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  1. Clinical Laboratories, Kings Park State Hospital, Kings Park, N. Y.

    Reuben M. Cares M. D.

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  1. Reuben M. Cares M. D.
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Cares, R.M. Juvenile amaurotic family idiocy: Features suggestive of precocious senility. Psych Quar 25, 445–457 (1951). https://doi.org/10.1007/BF01584292

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