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Mongoloid Idiocy Mongolism

athyreosis, normal, congenital, phalanx, times and usually

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MONGOLOID IDIOCY; MONGOLISM There is often confusion between mongoloid idiocy and congenital athyreosis (myxidiocy) owing to their great similarity in the first year of life. For this reason mongoloid idiocy, which is marked by external stigmata, will be discussed here, although it has nothing to do with affections of the thyroid. At the same time, there may occasionally be thyroid disorders in these patients, aside from other malformations and physical anomalies.

The mongoloid form of congenital idiocy is, in the first place, marked by oblique position of the eyes, converging inward and downward, and also brachycephaly. There is besides in nearly all cases epicanthus and conjunctivitis whieh may be very pronounced and obstinate. These patients are of normal or even excessive growth with considerable obesity in the first year of life in consequence of excessive food and insufficient physical exercise (Figs. 133, 134, and 137).

The face is ugly, owing to broad, prominent cheek-bones or temples, small, broad, and clumsy saddle nose, coarsely modelled ears, which are usually different in shape and nipped in the external margins on one or both sides. Figs. 134 and 137 illustrate this peculiarity. The mouth is usually open owing to adenoid proliferations in the naso pharyngeal space, and the en larged and swollen tongue protrudes from almost immovable lips. The neck is very fat ; so are the trunk and extremities. The abdomen is distended, often asso ciated with diastasis of the recti and umbilical hernia in conse quence of habitual constipation.

There is a surprising relaxa tion of all the articulations, which adinit of almost incredible, pain less twisting of the extremities (Fig. 134).

The bones present either no anomalies at all, or there is a mix ture of premature osseous nuclei— for instance, of the digital pha langes—and retardation of others. The bones of the hand in the second or third year are never as backward as is the case in athy reosis Figs. 135 and 136).

Again, while in mongoloid idiocy all affections injuring the bone marrow, or excessive feeding, quite generally lead to rachitis, this is not the case in athyreosis.

Where the diagnosis presents any difficulties, the radiogram of the hand will be of help. In athyreosis there is persistence of the condition found in the new-born, as late as the fifth or even tenth year; in mongo loid idiocy there is premature, nortnal or very slightly retarded growth of the epiphyses and osseous nuclei at the root of the hand. Dentition is usually delayed, but is often complete and occasionally even early. The change of teeth is not notably disturbed, but leads to a degenerated second set.

The cranium, in direct contrast to athyreosis, is always very short and small in all diameters. The fontanel is either closed at the normal period or remains open till the third to fifth year. Roundish cavities in the sutures are frequent, and persist for a long time (Kassowitz). The saddle nose is one of the typical peculiarities, likewise the narrow roof of the mouth. There is often a pronounced barrel chest, whether rachitis is present or not. A shortened second phalanx of the little finger is a characteristic sign. In these cases the end phalanx deviates toward the inner side and does not, reach the articulation of the end phalanx of the third finger, which is the case in normal children.

Other malformations often consist in congenital affections of the heart tGarrod, Gossage, Guthrie, Neumann, Sutherland, Bourneville, Kassowitz, Desgeorges, Fennel, and others). Neumann observed them three times in thirteen cases, Muir-Sutherland five times in twenty five, Kassowitz twice in seventy-cases, and myself three times in thirty. Hernite, supernumerary fingers and toes, cleft palate, and exostoses have been described. Dwarfish structure, increasingly apparent with advanc ing age, may be considerable. I observed a child which at the age of five years was only 83 em. in height. Height, however, may also be normal, but as a rule there is always moderately arrested growth.

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