Puberty usually sets in very late and is often incotnplete, the genitals being of infantile size.
The soft parts require separate discussion.
The skin is in the first year, or even later, very tense over the adipose tissues and often desquamates at the cheeks and donsal surfaces of the hands. A pronounced hyperwinia of the cheeks and chin reminds onc of a painted clown (Kassowitz). Frequently there is eczema of the face, chiefly around the mouth, on the chin and lips which are nearly always wet with saliva. These eruptions may also spread to other parts and prove very obstinate (Fig. 137). The musculature is flabby, and, until the child has learned to walk, the normal pressure about the legs is absent. With deficient intelligence, and consequent insufficient muscular co ordination, these patients are unable to keep head or body up, stand or walk, ancl ean only with difficulty be adequately supported. All the articulations are relaxed, the fingers can usually be bent back over the hand and the legs flexed alongside the trunk, while the head can nearly be twisted backward (Fig. 131). This peculiar articular relaxation only disappears with advancing age, after the intellect and muscular strength have slowly increased and coordination movements become possible.
The weakness of abdominal pressure often leads to considerable diastasis and umbilical hernia, owing to constipation, which is nearly always present. and probably occasioned by- the refusal of patients to take solid food in the presence of hypertrophied tonsils.
Respiration is usually loud and snorting as early as the first year; snoring during sleep is the rule. The mouth being always open and deglutition difficult, Ivonchitis and bronchopneumonia may supervene which often enough lead to fatal disturbances of respiration.
Insufficiency of thyroid function has repeatedly been found at autops.y and attributed to pathological changes of the thyroid. It is clinically recognized by impaired metabolism, lowered temperature, changes of the skin similar to myxcedema, changes of the hair, dwarfish strueture, thickening of the oral mucous membranes, enlargement of the "mongoloid tongue," which at a later period is traversed by a number of transverse parallel furrows, enlarge ment of the tonsils, and marked physical and mental apathy. The immediate di.sappearanceof all hese manifestations, of the obstinate constipation, of ccleina and conjunctivitis, which has been ob served after a short thyroid medication, is a sufficient proof of the correctness of the diagnosis even without anatomical demonstration (Fig. 138). Intelligence
develops very slowly and usually- per sists on a low level for a number of years, although some development is distinctly perceptible. All degrees of idioey occur, from the slight imbecility to the more severe forms. It is, however, in the nature of the disease that absence of idiocy and normal mental behavior exclude the diagnosis of mongoloid idiocy or mongolism, just :IS positively as the absence of the typical behavior of the eyes in spite of the presence of idiocy.
The most unsatisfactory part of the progress, aside from uncleanli ness, is the inability: to speak at a time when mongoloids may already perfectly understand the spoken word. It is difficult to differentiate in these cases between the effect of training by intelligent example, and independent expressions of demonstrable intelligence in the presence of a considerable degree of idiocy.
The course of mongoloid idiocy is typically marked by stages of developing intellect. Sudden and relatively rapid advances alternate with a standstill of many months. This, together with any possible somatic anomalies, will only yield to thyroid medication, if the case is one of dysthyreosis. An idiot will remain an idiot ; indeed, his mongoloid habitus even more strikingly manifests itself by the application of organo therapy, while athyreotic infants undergo a surprising development of intellect under this medication. The most radical change is from the physical and mental apathy, typieal in the first year, to the restless and lively conduct obtaining in the second, or at most third year. The /non goloid child is now always ready for a joke, and imitation by all kinds of gestures; he will teas, his brothers and sisters, nlay become irate, hitting, scratching and bit ing, but generally speaking he is a gay and harmless idiot. His predilection and nleniory for music are astounding. In the severest cases, how-ever, a new change will occur in more advanced years, with the gradually progressing degree of idiocy the former apathy again returns,the hopefulimprove ment gives way to a complete stand-still and, aside from excep tionally' excitable and vivacious patients, the great majority will persist in permanent physical and psychic stupor. It is hardly passible for theru to attain even tho level of a four- or five-year-old normal child.