2 JAPANESE CASES WITH MICROCEPHALIC PRIMORDIAL DWARFISM - CLASSICAL SECKEL SYNDROME AND OSTEODYSPLASTIC PRIMORDIAL DWARFISM TYPE-II

A male infant with ''classical'' Seckel syndrome and a girl with osteo dysplastic primordial dwarfism type II are described. The boy with cla ssical Seckel syndrome had severe brain dysplasia, a finding hitherto unreported in patients with this syndrome. The patient with osteodyspl astic dwarfism type II had skeletal abnormalities including lumbar sco liosis, a small and high pelvis, metaphyseal flaring of the distal rad ii and ulnae, V-shaped metaphyseal flaring of the distal femorae, and short metacarpals and phalanges. The mother of this girl was short, mi crocephalic, and had disproportionately short forearms and legs. In vi ew of this, dominant inheritance of the disease was suggested.