Abstract
There can be little doubt that in the group of so-called familial mental deficiencies numerous types are at present included which actually differ in etiology, clinical manifestations and pathology. Any attempt at singling out clinicopathologic syndromes from this heterogeneous group appears, therefore, of interest. The purpose of this presentation is to describe an apparently new syndrome characterized by severe mental deficiency, peculiar physical signs and conspicuous pathologic changes. The relation of this condition to juvenile amaurotic idiocy and gargoylism will be briefly discussed, and the causation and pathogenesis of the morbid process will be indicated. REPORT OF CASES Six cases of this type of mental defect were observed in siblings of one family. The family history included four generations of Italian ancestry on both the paternal and the maternal side. On the paternal line, the grandparents were living and in good health: They had 9 children, 1 of whom died

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