Synonyms:—Sporadic cretinism; athyria; Gull's disease.
Definition:—This is a constitutional disease, caused by a derangement of the function of the thyroid gland and characterized by an infiltration of mucin into the subcutaneous tissues, resulting in a characteristic swelling, a loss of hair, and perhaps of the nails, progressive mental feebleness, and serious impairment of the circulation.
Etiology:—The disease is due to an impairment of the function of the thyroid gland, an inability to supply the secretions which are essential to normal metabolism, from degeneration, atrophy, and consequent inactivity of the gland. The disease occurs much more frequently in women than in men, and is more common in some countries than in others. It occurs between the ages of thirty-five and fifty, and there seems to be an inclination to its more frequent occurrence among married women at the climacteric, after having borne a number of children.
On the other hand, pregnancy may cause symptoms of this disease which have appeared, to disappear. According to some writers, heredity has some influence in its occurrence. The mother may transmit it to the daughters, and in an occasional case both myxedema and exophthalmic goiter have appeared in the same family.
Symptomatology:—Perhaps the first symtom of this disease is a swollen condition of the face, which may be taken for dropsy; the condition can be readily distinguished from that disorder by the fact that the skin is firm and resistant to pressure, is rough, dry and inelastic, and does not pit upon pressure. Ascites, however, may exist coincidently in the later stages. The infiltration is not confined to the face, but will be found to be nearly uniform at times over the body, which is markedly increased in size. The patient loses facial expression, and an appearance of imbecility is quickly observed. He is stupid, inactive, dull of comprehension, is difficult to interest in the surroundings, or he takes an interest in very childish things. The hair falls out, the teeth are loosened, the mucous membranes of the mouth, with the structures of the nose, lips and tongue, become thickened, and there is an important change in the character and tone of the voice.
Both mental and muscular feebleness increase as time passes, and muscular control is seriously impaired. The head droops; the patient looks out from under the eyebrows with a silly expression; he walks with a slow, heavy, waddling, uncertain gait, and falls readily, being unable at times to retain his equilibrium, because of faults of coordination. As the mental failure progresses, the disposition becomes erratic; the patient is at times petulant, and irritable or excitable; at other times, dull, listless, apathetic, despondent, and subject to delusions or illusions. Occasionally there are hemorrhages, both from the nose and gums, and not infrequently from the stomach and bowels. Albuminuria occurs in frequent cases, and hematuria is not impossible.
Diagnosis:—The dull, expressionless, swollen countenance, which does not pit on pressure, is the characteristic diagnostic feature of this disease.
Prognosis:—The disease is not amenable to medical treatment. It progresses slowly and may not terminate fatally for perhaps a dozen years.
Treatment:—No medical treatment has been of any benefit. Glandular remedies offer the most encouragement, but success has not followed their use. There is almost an entire absence of pronounced specific indications for any of our remedies. The disease, however, has yielded to the use of the thyroid gland of calves and sheep. The gland is given usually in a raw state. It is prepared in the form of a powder, which may be compressed into tablets; or it is prepared as a glycerin extract. The gland may be thoroughly ground or minced, and spread raw upon bread that has been buttered. This should be taken at stated intervals, of such quantity that from one-fourth to one-third, or in adults, one-half, of a gland be taken in twenty-four hours. In cases of simple atrophy, especially in the early stage, there is a prompt response to the treatment. If the gland is diseased or has been removed, the response is slower, and it becomes necessary to administer the substance at intervals, even after the patient has apparently recovered.
Some patients will do as well or better on a very small quantity of the gland; others need a large quantity. Too much can be readily given, when rapid heart action, flushed face, nervous excitability and symptoms of an induced cerebral hyperemia, with vomiting and vertigo, and occasionally severe headache, will be the result. If these symptoms appear, the remedy should be stopped for a while, and the patient should be put on tonic treatment, and later if the remedy is resumed, it should be administered in very small doses and increased to a point where the results are satisfactory. It is thought that a cumulative influence from the gland substance will occur as a result of overfeeding with it.
This condition is a form of myxedema which depends upon congenital atrophy of the thyroid, or an entire absence of that gland, may be found upon examination of a new-born child. As a result of this malformation these patients develop very slowly, are misshapen, and are often repulsive in appearance; their heads are large, and their faces are bloated and expressionless; the mouth is constantly open and usually the tongue protrudes. They have broad, thick, pendulous abdomens, and their limbs are more or less distorted; there is seldom any great degree of mental development. If they learn to talk, they use but few words and these are incorrectly pronounced. Their locomotion is similar to that induced by myxedema. These children seldom live to adult age. If they do, they are still infants in development.
Treatment:—Nothing had been beneficial in these cases until the use of the thyroid extract mentioned above was introduced. Dr. Sidney Kuh, of Chicago, a few years ago recommended the use of iodothyrin in place of the dried thyroid, believing that the unpleasant results of an overdose of the substance were thus avoided.