Cerbral gigantism (Sotos' syndrome)

The metacarpophalangeal pattern (MCPP) profile in 17 out of 22 patients with Sotos' syndrome was studied throughout the growth period, from 2 weeks to 19 years of age. Two typical MCPP profiles will be described. The first type of profile was found in 13 patients. Although the form of the profile is the same throughout the years, the lengthening of the fingers is most pronounced around 2 years of age. Before and after that period there is a flattening of the curve. The metacarpals are relatively longer below the age of 3 years, above 3 years the lengthening of the proximal phalanges is more pronounced in the profile. The second type of profile was found in 4 patients. This curve is almost a mirror image of the first profile. The fingers are not as long as in the first type and the profile is less pronounced. In one of the 17 patients the transition from the first type to the second type could be demonstrated. One patient had a normal profile. (orig.)