Five cases of MNBCCS caused by a dominant gene in three generations of one family are described. The history suggests that one other member of the family is affected. Another a 6 months old child, has some suggestive features—Skin lesions and/or dental cysts were major clinical signs which caused the three older patients to present for treatment. The disease was unsuspected in two younger patients 7 and 5 years old.— Intracranial calcifications and rib malformations were present in all the patients in whom the disease was proved, and all showed generalised bone changes.
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