PART VII.

DISEASES OF THE NERVOUS SYSTEM WITHOUT KNOWN ANATOMICAL BASIS.

A LARGE number of diseases clearly related to the nervous system and manifested on its part by disturbance of functional control are not marked by changes in the central or peripheral apparatus as yet discovered. They are denominated neuroses, or functional nervous diseases. It is better to avoid the term functional, as it tends to distract attention from the probably existing anatomical basis of these diseases. There is little doubt that sooner or later they will be histologically classified. The present arrangement is for utility only. The following table serves to group the neuroses for descriptive purposes:

CHAPTER I.

TROPHONEUROSES.

In the group of neuroses marked by disturbances of nutrition some abnormality of the vasomotor control is commonly present. This may furnish nearly the entire symptomatology, as in angioneurotic edema, Raynaud's disease, and exophthalmic goiter. In other instances the vascular element is apparently small, and metabolism and catabolism must be incriminated. The relation of nutrition to vascularity is always intimate. We can not as yet positively say through what individual strand of fibers trophic control is maintained, but it must be in close touch functionally with the vasomotor nervous apparatus, which is mainly represented in the so-called sympathetic system.

ACROMEGALIA.

In 1886 P. Marie 1 called attention to two cases presenting acquired symmetrical enlargement of the hands, feet, and face, and proposed the name acromegalia, which has now become current. His description of

these cases was so full that little has since been added to the clinical side of it. Similar cases were at once recognized all over the world. In 1892 Collins 2 was able to collect about ninety cases from literature. Since then the material has rapidly increased, and about a score of autopsies have been put on record.

Etiology. The causation of the disease is extremely obscure. Alleged or supposed inciting causes are almost as numerous as recorded cases, and embrace well-nigh every ordinary and exceptional experience of human life; consequently, no importance at present attaches to any of them. Both sexes are about equally affected. The disease makes its appearance customarily between the ages of eighteen and thirty. Exceptional cases have developed in advanced life and others in childhood. Several have appeared so early that they have been considered congenital. It has been found in association with tabes, syringomyelia, rachitis, gigantism, exophthalmic goiter, goiter, and various psychoses.

Klebs, impressed by his first case in which the thymus gland persisted, and Erb by the area of sternal dullness in other cases, were disposed to consider the changes in the bones and soft parts as due to a thymic angiomatosis. Later cases failed to present the enlarged thymus, and the sternal dullness is probably sometimes due to thickening of the bone itself. Some, with Freund, have attributed the somatic changes of acromegalia to inversion in the evolution of genital life. Recklinghausen considered it an angioneurosis dependent upon central changes. These changes are not found, and angiomatosis is not evident in the tissues.

1 "Revue de Méd.," 1886

2Jour. Ment. and Nerv. Dis.," N. Y., 1892.

The usual adenomatous enlargement of the pituitary body and the frequent diminution of the thyroid have led to the belief that acromegalia is a trophoneurosis dependent upon modifications of the functions of these glands, especially of the pituitary. Rogowitsch, for example, considers that these glands destroy certain substances which have a toxic influence on the central nervous system. Others think that they secrete certain substances needful to the proper action of the trophic apparatus. All that can now be said is that the prehypophysis cerebri is nearly if not always hypertrophied, but whether as a cause or result is not determined. It is true, however, that tumors of the pituitary have been found without acromegalia.1

Morbid Anatomy.-The bones of the face, cranium, extremities, and to a lesser degree those of the trunk, show hypertrophy. The frontal and occipital bones are commonly thickened and their markings exaggerated. The frontal and maxillary sinuses are enlarged and the

Fig. 186.-1, Base of cranium showing excavation of the sella and, 2, pituitary tumor in a case of acromegalia (Marie).

The inferior maxilla, the

malars, the zygomatic arches, and the superciliary ridges of the frontal are particularly enlarged. There is commonly elongation of the spinous processes in the cervicodorsal region, and the bodies of the vertebræ are sometimes increased in the anteroposterior diameter. The ribs, clavicles, and sternum are commonly, and the shoulder-blades occasionally, enlarged. In the limbs the hypertrophy is most marked toward the distal ends. The long bones of the leg and forearm are most changed at their lower ends. The metacarpals, metatarsals, and phalanges are particularly involved. Histologically, the hypertrophy is a true one, bone being deposited under the thickened periosteum and the central canal enlarged by the action of osteoblasts.

The pituitary gland is enlarged and hypertrophic, filling up the distended and enlarged sella turcica and commonly compressing the optic

1 Jokoloff, Virchow's "Archiv," Bd. xliii.

chiasm and adjoining structures. It practically constitutes an endocranial tumor. Strümpell 1 is inclined to think it an essential finding. In many cases it has been found to have undergone changes, probably secondary in point of time and nature. Tamburini 2 believes the first stage is one of enlargement and increased function, followed by adenomatous, sarcomatous, or cystic degeneration. A number of cases showing such degenerative changes are cited by Strümpell 3 and reported by others.

In the extremities and other portions affected by the hypertrophic enlargement the skin in its epithelial, dermal, glandular, and muscular parts is hypertrophically exaggerated. The connective tissue particularly is increased. The subcutaneous nerve-filaments are sometimes degenerated as a result of the fibrous hyperplasia, but this does not extend into the nerve-trunks. The mucous membrane of the nasopharynx,

Fig. 187.-Skiagraphs of hands: A, Case of acromegalia; B, normal hand.

larynx, and tongue are similarly affected. The kidneys often show a moderate chronic parenchymatous nephritis, with interstitial fibrosis. The spleen and lymphatic glands may be sclerosed; the thyroid is usually atrophic; the thymus may persist and even be enlarged. It is to be noticed that, while parenchymatous glandular structures generally are atrophic, the pituitary is hypertrophic.

Symptoms.-Among the most common symptoms of acromegalia we may name hypertrophy of the face, hands, and feet, visual defects, dorsal kyphotic deformities, persistent headache, sexual failure in men, and amenorrhea in women. The face is notably deformed. The greatly enlarged, overhanging brows are marked by shaggy, coarse eyebrows; thickened eyelids; sometimes exophthalmos; a heavy, large, flabby nose;

1"Deutsche Zeit. f. Nervenheilk.," 1897.

2 "Centralblatt f. Nervenh.," Dec., 1894.

3 Loc. cit.

great cheek-bones; an enormous mouth, correspondingly thickened lips, and a much enlarged and prognathic lower jaw are some of its characteristics. The heavy features, puffy face, and accentuated nasolabial folds give an expression of grief and suffering. When the mouth is opened, it is seen that the hypertrophic enlargement of the jaws, espe cially of the mandible, causes the teeth to stand separately and at considerable intervals. The mucous membrane is also thickened and the tongue is notably enlarged, in some instances reaching an enormous size. The soft palate, pharynx, and larynx equally share in the hyperplasia, and the tonsils and follicular structures are also increased in size. The ears and occiput are sometimes enlarged, sometimes not. The scalp is thickened, the hair coarse, thick, and harsh. The complexion is usually sallow

Fig. 188.-Face in acromegalia. Note enlarged superciliary ridge, thickened lips, massive jaw, and general grossness.

in color and the parts are elastically doughy to the touch, showing no pits on pressure.

The hands are greatly enlarged in all their dimensions, but particularly in width. The proportion of fingers to hand and hand to wrist is retained; they are, therefore, enlarged symmetrically. The fingers are thick and sausage-shaped, the hand thick and beefy, the thenar and hypothenar eminences greatly but proportionately enlarged. The whole hand is described as "spade-like or battledore-shaped." This appearance is sometimes intensified by a comparative thinness of the forearms. The joints are never limited in their range of motion, and the parts are surprisingly supple and flexible. The palmar furrows are usually much deepened, the skin thick and resilient, the hairs coarse, the nails broadened, thick, and strongly striated longitudinally.

The feet show similar changes, and usually there is a heavy welt of fibro-elastic increase around the heel and down the outer border of the foot.