ETIOLOGY.—Diseases of the cervical sympathetic ganglia or cord may be of two kinds—either irritative or destructive.61 They are produced by pressure upon the cervical ganglia or upon the sympathetic cord between these ganglia, by tumors, especially aneurisms, and enlarged glands; by abscesses; and by cicatrices of old wounds in the neck. They are also due to extension of inflammation from a thickened pleura in phthisis and chronic pleuritis of the apex. They may be caused by injuries, such as stab-wounds, gunshot wounds, etc. Any disease which produces marked irritation of peripheral branches of the sympathetic in the neck, or of the cerebro-spinal cervical nerves, may cause reflex phenomena resembling the symptoms of actual disease. From such phenomena it is not justifiable to conclude that the sympathetic cord and ganglia are the seat of lesions, and the only cases which will be considered here are those in which actual disease was proven to be present by an autopsy.
61 Ogle, Medico-Chirurgical Transactions, xli. 397-440, 1858, 27 cases; Poiteau, “Le Nerf sympathetique,” Thèse de Paris, 1869, 19 cases; Eulenburg and Guttmann, Die Pathologie der Sympathicus, 1873; Nicati, Le Paralysie du Nerf sympathique-cervicale, 1873, 25 cases; Seeligmüller, Inaug. Dissertation, 1876; Mitchell, Injuries of Nerves; Mobius, “Pathologie der Sympathicus,” Berlin. klin. Woch., 1884, Nos. 15-19.
Inasmuch as the cervical sympathetic is in close anatomical connection with the spinal cord, especially with the eighth cervical to the second dorsal segments (the so-called cilio-spinal centre of Budge), and as the functions of the sympathetic are dependent upon the integrity of the spinal cord, it is evident that any lesion of the nerves uniting it with the cord, or any lesion in the cord itself at the levels mentioned, may produce symptoms which resemble closely those of disease of the sympathetic. Thus, cervical pachymeningitis, myelitis (especially from injury of the cord, or hæmato-myelia), and diseases of the cervical vertebræ which produce either or both conditions, may cause a train of symptoms somewhat similar to those to be described.62 A careful distinction must be made between primary and secondary disease of the sympathetic, between reflex and direct symptoms, between lesions in its substance and lesions in its governing centres in the spinal cord. The symptoms produced by affections of a reflex or central nature are rarely as numerous as those of disease of the sympathetic itself. An example of such a secondary affection is the combination of sympathetic symptoms occurring in progressive muscular atrophy. And, finally, since mental action of an emotional nature may cause flushing or pallor of the face, with profuse sweating and variations in the size of the pupil and prominence of the eyeballs, as well as palpitation or arrest of the heart, there is reason to believe that symptoms of sympathetic disease may be produced by cerebral lesions.
62 Ross, Diseases of the Nervous System, 2d ed., i. 686-688.
PATHOLOGY.—The pathological anatomy of the cervical sympathetic is obscure. This is probably owing to the fact that the ganglia are rarely examined, and pathologists have not been familiar with their histology. Lesions of the cervical sympathetic have been described in almost every imaginable form of disease, and at one time, when many obscure conditions were blindly termed sympathetic, the records were filled with descriptions of fatty degeneration or interstitial inflammation or pigment deposit in the ganglia. As no actual symptoms of disease of the cervical sympathetic, as now understood, were present in such cases, it is impossible to believe that the lesion was other than hypothetical.
The conditions which have been observed in a few carefully-studied cases of primary disease have been—(1) A parenchymatous inflammation of the cells of the ganglia, attended by swelling, loss of nuclei, granular and fatty degeneration, and by atrophy, together with a degeneration of the fibres issuing from the cells. (2) A sclerotic process in the connective tissue in and about the ganglia and in the nerves, resulting in such an increase in the interstitial tissue as to compress and injure the cells and axis-cylinders. These may be observed together in the later stages of the disease. (3) In a number of cases the capillaries within and about the ganglia have been found dilated, tortuous, and varicose, and hemorrhages from them are not rare.
SYMPTOMS.—The symptoms of irritation of the cervical sympathetic are dilatation of the pupil, widening of the palpebral fissure, protrusion of the eyeball, pallor of the entire side of the face and head, with slight fall of local temperature and possibly an increased secretion of perspiration, and an increased frequency of the heart. It is rarely that these are all observed in any case, dilatation of the pupil with slight pallor and rapid pulse being the only signs of irritation as a rule. Such irritation is a less common occurrence than might be supposed, many lesions which produce pressure even of a slight degree on the sympathetic having caused symptoms of a suspension of its function rather than of an increased activity. This is doubtless due to the non-medullated structure of the fibres, which thus lack protection from injury.
The symptoms of destructive disease of the cervical sympathetic are the converse of those just mentioned, and they are all present when the part is seriously involved. The patient will then have a marked contraction of the pupil, which no longer responds to light or to irritation of the skin of the neck, but may change slightly in the act of accommodation. It resists the action of mydriatics. The vessels of the choroid and retina may be dilated, as well as those of the iris, in which case the patient will feel a sense of weariness on any long-continued attempt to use the eyes. There is no actual disturbance of vision, and the cornea is not usually flattened, as was formerly supposed. There is a noticeable narrowing of the palpebral fissure, the upper lid falling slightly as in a mild state of ptosis, and the lower lid being slightly elevated. This is due to the paralysis of the muscles of Müller in the eyelids, which are controlled by the sympathetic. It is present in 90 per cent. of the recorded cases, and in many the apparent size of the eye is reduced a half. Retraction of the eyeball is a less constant symptom, and one which develops only after the disease has existed some time. It is due partly to the paralysis of the orbital muscle of Müller, and partly to the decrease in the amount of fat in the orbit behind the eye. A marked symptom, and one which is constant, is a dilatation of the vessels of the face, conjunctiva, nasal mucous membrane, ear, and scalp. This is attended by redness, a subjective sense of heat, and an actual rise of local temperature, which may exceed that of the other side by 1.5° F., measured in the auditory meatus or nose. This vascular congestion has persisted in some cases for three years. In others it has been followed much earlier (in nine months) by a partial or complete return to the normal condition, and even when the local temperature remains higher on the affected side, the visible congestion and the sensation of heat may have disappeared. The dilatation, succeeded by the contraction (normal tone), of the vessels has led to a division of the disease into two stages, and in a few cases the affected side has become paler than the other in the second stage. In both stages the part affected is less sensitive to changes in the external temperature.
An increased secretion of tears and of perspiration has been supposed to accompany dilatation of the vessels of the skin of the head inevitably. This is not a constant symptom, as the recent cases have demonstrated. And no definite statement of the effect of disease of the cervical sympathetic on the occurrence of dryness or dampness of the face can be made, both conditions having been observed. A difference between the degree of moisture on the two sides of the face on exposure to heat is usually present. Palpitation of the heart has been an annoying symptom to the patient in many cases, and is usually associated with a marked slowing of the pulse. This was reduced from 74 to 66 in Möbius' case,63 and remained slow for some weeks. The frequency of the heart may, however, be increased after the first period of slowing, but never reaches a very high rate (88 in the case cited). A slight atrophy of the affected side of the face has been observed in several cases, appearing after the disease has existed for some time. The muscles of the cheek feel flabby and are slightly sunken; but the condition does not approach in severity true facial hemiatrophy, nor is it sufficiently rapid to be considered due to a trophic disturbance. Changes in the secretion of saliva, dryness of the nasal mucous membrane, and symptoms referable to paralysis of the intracranial vessels, such as might be expected from the result of physiological division of the sympathetic, have only been occasionally observed. Glycosuria has been noted in a few cases.64
63 Berlin. klin. Woch., 1884, No. 16.