Calculi, Part 6

Anomalies and Curiosities of Medicine
by George M. Gould, M.D., Walter L. Pyle, M.D.

(Page 28 of 36)

Cretinism is an endemic disease among mountainous people who drink largely of lime water, and is characterized by a condition of physical, physiologic, and mental degeneracy and nondevelopment, and possibly goiter. The subjects of this disease seldom reach five feet in height, and usually not more than four. The word cretin is derived from the Latin creatura. They are found all over the world. In Switzerland it is estimated that in some cantons there is one cretin to every 25 inhabitants. In Styria, the Tyrol, and along the Rhine cretins are quite common, and not long since cases existed in Derbyshire. These creatures have been allowed to marry and generate, and thus extend their species. In "Le Medicin de Campagne," Balzac has given a vivid picture of the awe and respect in which they were held and the way in which they were allowed to propagate.

Speaking of the endemic cretins, Beaupre says: "I see a head of unusual form and size, a squat and bloated figure, a stupid look, bleared, hollow, and heavy eyes, thick, projecting eyelids, and a flat nose. His face is of a leaden hue, his skin dirty, flabby, covered with tetters, and his thick tongue hangs down over his moist, livid lips; his mouth, always open and full of saliva, shows teeth going to decay. His chest is narrow, his back curved, his breath asthmatic, his limbs short, misshapen, without power. The knees are thick and inclined inward, the feet flat. The large head droops listlessly on the breast; the abdomen is like a bag." The cretin is generally deaf and dumb, or only able to give a hoarse cry. He is indifferent to heat and cold, and even to the most revolting odors. The general opinion has always been that the sexual desire and genital organs are fully developed.

A quotation under our observation credits Colonel Sykes with the following statistics of cretinism, which show how in some locations it may be a decided factor of population. In December, 1845, in a population of 2,558,349 souls (the locality not mentioned), there were 18,462 people with simple goiter. Of the cretins without goiter there were 2089. Of cretins with goiter there were 3909; and cretins in which goiter was not stated 962, making a total of 6960. Of these 2185 had mere animal instincts; 3531 possessed very small intellectual faculties; 196 were almost without any; 1048 not classified. Of this number 2483 were born of healthy and sane fathers; 2285 from healthy mothers; 961 from goitrous fathers; 1267 from goitrous mothers; 49 from cretin fathers; 41 from cretin mothers; 106 from cretin fathers with goiter; 66 from cretin mothers with goiter; 438 fathers and 405 mothers were not specified.

Sporadic cretinism, or congenital myzedema, is characterized by a congenital absence of the thyroid, diminutiveness of size, thickness of neck, shortness of arms and legs, prominence of the abdomen, large size of the face, thickness of the lips, large and protruding tongue, and imbecility or idiocy. It is popularly believed that coitus during intoxication is the cause of this condition. Osler was able to collect 11 or 12 cases in this country. The diagnosis is all-important, as the treatment by the thyroid extract produces the most noteworthy results. There are several remarkable recoveries on record, but possibly the most wonderful is the case of J. P. West of Bellaire, Ohio, the portraits of which are reproduced in Plate 11. At seventeen months the child presented the typical appearance of a sporadic cretin. The astonishing results of six months' treatment with thyroid extract are shown in the second figure. After a year's treatment the child presents the appearance of a healthy and well-nourished little girl.

Myxedema proper is a constitutional condition due to the loss of the function of the thyroid gland. The disease was first described by Sir William Gull as a cretinoid change, and later by William Ord of London, who suggested the name. It is characterized clinically by a myxedematous condition of the subcutaneous tissues and mental failure, and anatomically by atrophy of the thyroid gland. The symptoms of myxedema, as given by Ord, are marked increase in the general bulk of the body, a firm, inelastic swelling of the skin, which does not pit on pressure; dryness and roughness which tend, with swelling, to obliterate the lines of expression in the face; imperfect nutrition of the hair; local tumefaction of the skin and subcutaneous tissues, particularly in the supraclavicular region. The physiognomy is remarkably altered; the features are coarse and broad, the lips thick, the nostrils broad and thick, and the mouth enlarged. There is a striking slowness of thought and of movement; the memory fails, and conditions leading to incipient dementia intervene. The functions of the thoracic and abdominal organs seem to be normal, and death is generally due to some intercurrent disease, possibly tuberculosis. A condition akin to myxedema occurs after operative removal of the thyroid gland.

In a most interesting lecture Brissaud shows the intimate relation between myxedema, endemic cretinism, sporadic cretinism, or myxedematous idiocy, and infantilism. He considers that they are all dependent upon an inherited or acquired deficiency or disease of the thyroid gland, and he presents cases illustrating each affection. Figure 285 shows a case of myxedema, one of myxedema in a case of arrested development - a transition case between myxedema of the adult and sporadic cretinism - and a typical case of sporadic cretinism.

Cagots are an outcast race or clan of dwarfs in the region of the Pyrenees, and formerly in Brittany, whose existence has been a scientific problem since the sixteenth century, at which period they were known as Cagots, Gahets, Gafets, Agotacs, in France; Agotes or Gafos, in Spain; and Cacous, in Lower Brittany. Cagot meant the dog of a Goth; they were of supposed Gothic origin by some, and of Tartar origin by others. These people were formerly supposed to have been the descendants of lepers, or to have been the victims of leprosy themselves. From the descriptions there is a decided difference between the Cagots and the cretins. In a recent issue of Cosmos a writer describes Cagots as follows: -

"They inhabit the valley of the Ribas in the northwestern part of the Spanish province now called Gerona. They never exceed 51 1/2 inches in height, and have short, ill-formed legs, great bellies, small eyes, flat noses, and pale, unwholesome complexions. They are usually stupid, often to the verge of idiocy, and much subject to goiter and scrofulous affections. The chief town of the Ribas Valley is Ribas, a place of 1500 inhabitants. about 800 feet above sea-level. The mountains rise about the town to a height of from 6000 to 8000 feet, and command an amazingly beautiful panorama of mountain, plain, and river, with Spanish cities visible upon the one side and French upon the other. The region is rich, both agriculturally and minerally, and is famous for its medicinal springs. In this paradise dwell the dwarfs, perhaps as degraded a race of men and women as may be found in any civilized community. They are almost without education, and inhabit wretched huts when they have any shelter. The most intelligent are employed as shepherds, and in summer they live for months at an elevation of more than 6000 feet without shelter. Here they see no human creature save some of their own kind, often idiots, who are sent up every fifteen or twenty days with a supply of food.

"It is said that formal marriage is almost unknown among them. The women in some instances are employed in the village of Ribas as nurses for children, and as such are found tender and faithful. Before communication throughout the region was as easy as it is now, it was thought lucky to have one of these dwarfs in a family, and the dwarfs were hired out and even sold to be used in beggary in neighboring cities. There are somewhat similar dwarfs in other valleys of the Pyrenees, but the number is decreasing, and those of the Ribas Valley are reduced to a few individuals."