Megalocephaly, or as it was called by Virchow, leontiasis ossea, is due to a hypertrophic process in the bones of the cranium. The cases studied by Virchow were diffuse hyperostoses of the cranium. Starr describes what he supposes to be a case of this disease, and proposes the title megalocephaly as preferable to Virchow's term, because the soft parts are also included in the hypertrophic process. A woman of fifty-two, married but having no children, and of negative family history, six years before the time of report showed the first symptoms of the affection, which began with formication in the finger-tips. This gradually extended to the shoulders, and was attended with some uncertainty of tactile sense and clumsiness of movement, but actual anesthesia had never been demonstrated. This numbness had not invaded the trunk or lower extremities, although there was slight uncertainty in the gait. There had been a slowly progressing enlargement of the head, face, and neck, affecting the bone, skin, and subcutaneous tissues, the first to the greatest degree.
The circumference of the neck was 16 inches; the horizontal circumference of the head was 24 inches; from ear to ear, over the vertex, 16 inches; and from the root of the nose to the occipital protuberance, 16 inches. The cervical vertebrae were involved, and the woman had lost five inches in height. It may be mentioned here that Brissaud and Meige noticed the same loss in height, only more pronounced, in a case of gigantism, the loss being more than 15 inches. In Starr's case the tongue was normal and there was no swelling of the thyroid.
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 405mothers 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.
