It will be seen by reference to the table that usually certain general features in the symptomatology indicate the seat of the lesion. Thus in tumors of the cervical region pain and stiffness of the neck occur, while the first appearances of paresis and sensory disturbances are usually observed in the arms and about the chest. The centres for the forearm and hand lie in the lower portion of the cervical enlargement; that for the upper arm, including the supinator longus, in the upper portion. Mental symptoms are more marked, and in Case 4 several of the cranial nerves were implicated. In the lower cervical and upper dorsal region there are symptoms of dyspnœa, fixation of the chest (Nos. 20, 22, 24), and cough. The girdle symptom is an important indication at any level, as it is due to irritation of the nerves at the lowest level of healthy cord just above the transverse lesion. It has already been discussed under Symptomatology. In many of the dorsal cases (Nos. 32, 37, 38, 39, and 45) the symptoms are almost entirely confined to the legs and lower trunk, the arms escaping entirely. The condition of the bladder is usually given in the table as one of paralysis; this does not indicate whether automatic evacuation existed at the beginning of the case; which condition would indicate that the centre for micturition was below the lesion, and intact. It is probable that later in these cases the bladder is actually paralyzed by destruction of its centre in the cord, and this even when the tumor has been situated some distance above.
With reference to tumors of the cauda equina, Erb10 says that they have in every respect a great resemblance to those which are situated higher and affect the cord proper. “They are hard to distinguish from the latter, but may be in many cases perhaps, if it is borne in mind that tumors of the cauda produce exclusively nerve-root symptoms, and that the signs of compression of the cord, of secondary myelitis, etc. are absent. The higher the tumor, the nearer it approaches the lumbar portion of the cord, the harder will it be to draw the distinction. In respect to tumors seated lower the following points may be attended to: the seat of the pains (which in such cases often attain enormous violence) is strictly localized in certain nerve-districts; all nerves leaving the spinal canal above the tumor are free; thus in myxo-sarcoma telangiectodes of the cauda I observed the pain strictly limited to the district of the sciatica, while the crural and the dorsal nerves were perfectly free; constant violent pain in the sacrum. If palsy occurs the reflex actions necessarily cease at once. Spasms are seldom observed, more frequently contractures. Atrophy of the muscles occurs rather frequently. The palsy and anæsthesia by their localization often give us the opportunity of fixing the upper limit of the lesion. Increase of the reflex acts and marked tendinous reflexions do not occur. Paraplegia, palsy of the bladder, bed-sores, etc. may develop exactly as in tumors occupying a higher seat, but the symptoms of paralysis do not seem to belong necessarily to the disease, as is shown in my case (just mentioned), which terminated fatally before paralysis or anæsthesia occurred.”
10 Op. cit.
PROGNOSIS.—The prognosis of spinal tumors is generally very unfavorable. Syphilitic cases are of course the most hopeful, but even in these cases it is only when they are recognized early that much can be expected. A gumma that has grown to any dimensions will have so compressed the cord that even when the tumor is melted away by specific treatment its effects will remain.
DURATION AND TERMINATION.—Most cases of spinal tumor last from about six months to three years. Occasionally death may result, as from a rapidly-developing sarcoma, in less than six months, and somewhat more frequently in slowly-developing tumors, or in those which are held more or less in abeyance by treatment the sufferings of the patient are prolonged to four or five years or more. Hemorrhages into or around the growths sometimes take place, and are the cause of death, or more frequently of a sudden aggravation and multiplication of severe symptoms. Death sometimes takes place from the complete exhaustion which results from the disease and its accompanying secondary disorders, such as bed-sores, pyelitis, etc. Occasionally death results from intercurrent diseases, such as pneumonia, infectious fevers, etc., whose violence the weakened patient cannot well withstand. Sometimes the symptoms of a rapidly-ascending paralysis appear, probably due to an ascending myelitis or meningo-myelitis.
COMPLICATIONS AND SEQUELÆ.—Spinal tumors are sometimes complicated with other similar growths in the brain or the evidences of the same constitutional infection in other parts of the body. In one case of cysticercus of the cord sclerosis of the posterior columns was also present.
TREATMENT.—The treatment of spinal tumors can be compressed into very small compass. In cases with syphilitic history, or when such history is suspected, although not admitted, antisyphilitic remedies should be applied with great vigor. It should be borne in mind, however, that even in syphilitic cases after destruction of the cord by compression or softening specific remedies will be of no avail. In tubercular cases and in those in which the system is much run down tonics and nutritives are indicated. Bramwell11 advises an operation in any case in which the symptoms are urgent, in which the diagnosis clearly indicates the presence of a tumor, when there is no evidence of malignant disease, when the exact position of the growth can be determined, and when a vigorous antisyphilitic treatment has failed to produce beneficial results. As some meningitis, meningo-myelitis, or myelitis is usually present in cases of spinal tumor, treatment for the complication will assist in relieving the torments of the patient. Anodynes, particularly opium and its preparations, should be used freely in the later stages of the affection. Bromides and chloral are of little value except in association with opiates. Operation offers even less hope than in brain tumor, but in very rare cases should be taken into consideration.
11 Diseases of the Spinal Cord, Edinburgh, 1884.
SYNONYMS.—Essential paralysis of childhood (Rilliet and Barthez); Myogenic paralysis (Bouchut); Acute fatty atrophic paralysis (Duchenne); Atrophic paralysis (Ferrier); Acute anterior poliomyelitis (Kussmaul, Erb, Seguin); Regressive paralysis (Barlow); Tephromyelitis (Charcot).
DEFINITION.—Of all the titles which have been given to the disease it is our purpose to describe, two alone may be considered irreproachable. In the present state of our knowledge it is unnecessary to argue that this disease is not essential—i.e. destitute of characteristic anatomical lesions. Neither can the theory of its myogenic origin be maintained; nor even is fatty degeneration invariably present in the paralyzed muscles. Finally, the disease cannot longer be regarded as peculiar to childhood,1 since cases in adults have been in these last years quite numerously reported2—four with autopsies demonstrating the identity of the lesion. But there are two definitions in our list of synonyms which embrace between them the most striking characteristics of the disease, yet contain no error of fact. Atrophic paralysis describes at once the two most salient symptoms; acute anterior poliomyelitis defines at once the seat and nature of the lesion, classes it with the systematic diseases3 of the spinal cord, and notes the peculiarity in the mode of invasion by which it is so remarkably distinguished from nearly all the organic diseases of this centre.
1 W. H. Barlow, On Regressive Paralysis, 1828. See Brain, April, 1879.
2 In Dec., 1873, I quoted 14 cases of adult spinal paralysis, as follows: Duchenne, 4 cases; Charcot and Petitfils, 3; Moritz Meyer, 2; Bernhardt (Archiv Psych., 1873), 1; Cumming (Dublin Quart. Journ., 1869), 1; Lucas Championnière (by Hallopeau, Archives gén., 1861), autopsy, 1; Gombault (Archives de Psych., 1873), 1; personal, 1.
In 1874, Seguin published a summary of all the foregoing cases except the last, and added 6 personal observations, also 3 from Duchenne and 1 from Hammond. In the enlarged edition of his essay in 1877, Séguin increased the list to 45—by new personal cases, 3; cases related by Frey (Berlin. Wochens., 1874), 4; cases by Erb (Arch. f. Psych. u. Nervenkrank., v.), 4; case by Cornil and Lépine (Gaz. méd., 1875), autopsy, 1; case by Soulier (Lyon méd., 1875), 1; case by D. H. Lincoln (Boston Med. and Surg. Journ., 1875), 1; case by Lemoine (Lyon méd., 1875), 1; case by George M. Beard, 1; case by Leyden (Klinik Ruckenmarks Krankheiten) Bd. iv. 1; case by Hammond (6th ed. Treatise), 4; case by Courty (Gaz. méd., 1876), 1; case by Dejerine (Arch. de Phys., 1876), 1.
To these may be added—case by Goltdammer (Berl. klin. Wochen., 1876), 1; case by Webber (Trans. Amer. Neurol. Ass. for 1875, vol. i.), autopsy, 1; case by Klose (Diss. Breslau, 1876), 1; case by Schultze (Virchow's Archiv, Bd. lxviii.; also Bd. lxxiii.), autopsy, 1; case by Bernhardt (Archiv für Psych., Bd. ix., 1879); case by Sinkler (Amer. Journ. Med. Sci., Oct., 1878), 5; case by Althaus (ibid., April, 1878), 2; case by Ross (Dis. Nerv. Syst., vol. ii. p. 139), 1—total, 57 cases.
Morton (St. Bartholomew's Hospital Reports).
Others have doubtless been published since this date, but, as they do not immediately concern our subject, need no further citation.
3 Vulpian, Leçons sur les Myelitis, 1880.
SUMMARY OF CLINICAL HISTORY.—The clinical features of an acute attack of infantile paralysis are well known. The children affected are usually between eighteen months and four years of age (Henoch). The attack is more likely to occur in summer than in winter, as Sinkler4 found that 47 out of 57 cases began between May and September, and Barlow noted 27 out of 53 in July and August.5
4 Amer. Journ. Med. Sci., April, 1875.
5 Loc. cit., p. 75. Among Sinkler's 57 cases, only 6 furnish autopsies, thus:
Case by Cornil and Lepine and case by Webber (quoted and accepted by Erb in Ziemssen's Handbuch, Bd. xi.); case by Gombault (rejected by Erb and Westphal); case by Schultze; cases by Dejerine and Lucas Championnière (quoted by Hallopeau).
The influence of heat is perhaps shown in the case related by Dyce-Duckworth in the Lancet of 1877: a child two and a half years, after exposure to great heat on a steamboat-landing, became paralyzed in all four limbs, but the paralysis was subsequently confined to the lower extremities. Coincidently, the patient became delirious; suffered from anæsthesia and temporary paralysis of the sphincters. The paralyzed muscles wasted rapidly and lost faradic contractility. Treatment by faradization was begun in a month from the date of the attack, and recovery was complete three months later.
The onset of the paralysis is either really sudden, occurring in the daytime, while the child is under competent observation, or apparently sudden, being discovered in the morning after a quiet night, the child having gone to bed in health (West); or is preceded by some hours or days of fever or of nervous symptoms, especially convulsions, or both. The paralysis is almost always at its maximum of extent and intensity when first discovered, and from this maximum begins, within a few hours or days, to retrocede. The improvement may, however, be delayed much longer. A variable number of muscles remain permanently paralyzed, and in these, within a week (thirty-six hours, according to some observers), faradic contractility is first diminished, then abolished; galvanic reaction is exaggerated, ultimately is characterized by the degeneration signs (entartungs reaction). The temperature of the paralyzed limbs falls; the muscles waste; the atrophy may rapidly become extreme. The paralysis and loss of faradic contractility are complete, however, while the atrophy is only incipient and progressing. The absence of lesions of sensibility, of visceral disturbance, of trophic lesions of the skin, or of sphincter paralysis is as characteristic of the disease as are the positive symptoms above enumerated.
In the third or chronic stage the paralyzed limbs often become contracted and deformed. At other times, and with more complete paralysis, the growth of the bones is arrested, the muscles remain flaccid, the entire limb shrivels, and dangles so loosely from its articulations that it may be dislocated by slight effort (membre de Polichinelle). The general health of the patient remains remarkably good, the intelligence clear, the disposition lively. The duration of life seems to be in nowise shortened by the paralysis. Thus, suddenness of development, intimate association of trophic, motor, and electrical disturbance, absence of cerebral or sensory lesion, peculiar localization and grouping of the permanent paralyses,—such are the salient characteristics of this remarkable disease.
SYMPTOMS IN DETAIL.—Three well-defined stages exist—the initial, the paralytic, and the chronic.