An Essay on the Shaking Palsy Part 2

[Footnote 10: Corporis agitatio continua, indolens, convulsiva, c.u.m sensibilitate.-_Linn._

Agitatio corporis vel artuum convulsiva continua, chronica, c.u.m integritate sensuum.-_Vogel._

This genus is resolved by Cullen into that of Convulsio.

_Synops. Nosol._ 1803.

Dr. Macbride has given a very interesting and ill.u.s.trative case of this disease.

"Hieranasos, or Morbus Sacer, so called, as being vulgarly supposed to arise from witchcraft, or some extraordinary celestial influence, is a distinct genus of disease, though a very uncommon one; the author once had an opportunity of seeing a case. The patient was a lad about seventeen, who at that time had laboured under this extraordinary disease for more than twelve years. His body was so distorted, and the legs and arms so twisted round it, by the continued convulsive working, that no words can give an adequate idea of the oddity of his figure; the agitation of the muscles was perpetual; but in general he did not complain of pain nor sickness; and had his senses perfectly, insomuch that he used to a.s.sist his mother, who kept a little school, in teaching children to read." _A methodical Introduction to the Theory and Practice of Physic. By David Macbride, M.D. p. 559._]

The latter appears to be referable to that cla.s.s of proteal forms of disease, generated by a disordered state of primae viae, sympathetically affecting the nervous influence in a distant part of the body.

Unless attention is paid to one circ.u.mstance, this disease will be confounded with those species of pa.s.sive tremblings to which the term Shaking Palsies has frequently been applied. These are, _tremor temulentus_, the trembling consequent to indulgence in the drinking of spirituous liquors; that which proceeds from the immoderate employment of tea and coffee; that which appears to be dependent on advanced age; and all those tremblings which proceed from the various circ.u.mstances which induce a diminution of power in the nervous system. But by attending to that circ.u.mstance alone, which has been already noted as characteristic of mere tremor, the distinction will readily be made.

If the trembling limb be supported, and none of its muscles be called into action, the trembling will cease. In the real Shaking Palsy the reverse of this takes place, the agitation continues in full force whilst the limb is at rest and unemployed; and even is sometimes diminished by calling the muscles into employment.

CHAP. IV.

PROXIMATE CAUSE-REMOTE CAUSES-ILl.u.s.tRATIVE CASES.

Before making the attempt to point out the nature and cause of this disease, it is necessary to plead, that it is made under very unfavourable circ.u.mstances. Unaided by previous inquiries immediately directed to this disease, and not having had the advantage, in a single case, of that light which anatomical examination yields, opinions and not facts can only be offered. Conjecture founded on a.n.a.logy, and an attentive consideration of the peculiar symptoms of the disease, have been the only guides that could be obtained for this research, the result of which is, as it ought to be, offered with hesitation.

SUPPOSED PROXIMATE CAUSE.

A diseased state of the _medulla spinalis_, in that part which is contained in the ca.n.a.l, formed by the superior cervical vertebrae, and extending, as the disease proceeds, to the _medulla oblongata_.

By the nature of the symptoms we are taught, that the disease depends on some irregularity in the direction of the nervous influence; by the wide range of parts which are affected, that the injury is rather in the source of this influence than merely in the nerves of the parts; by the situation of the parts whose actions are impaired, and the order in which they become affected, that the proximate cause of the disease is in the superior part of the medulla spinalis; and by the absence of any injury to the senses and to the intellect, that the morbid state does not extend to the encephalon.

Uncertainty existing as to the nature of the proximate cause of this disease, its remote causes must necessarily be referred to with indecision. a.s.suming however the state just mentioned as the proximate cause, it may be concluded that this may be the result of injuries of the medulla itself, or of the theca helping to form the ca.n.a.l in which it is inclosed.

The great degree of mobility in that portion of the spine which is formed by the superior cervical vertebrae, must render it, and the contained parts, liable to injury from sudden distortions. Hence therefore may proceed inflammation of quicker or of slower progress, disease of the vertebrae, derangement of structure in the medulla, or in its membranes, thickening or even ulceration of the theca, effusion of fluids, &c.

But in no case which has been noticed, has the patient recollected receiving any injury of this kind, or any fixed pain in early life in these parts, which might have led to the opinion that the foundation for this malady had been thus laid. On the subject indeed of remote causes, no satisfactory accounts has yet been obtained from any of the sufferers. Whilst one has attributed this affliction to indulgence in spirituous liquors, and another to long lying on the damp ground; the others have been unable to suggest any circ.u.mstance whatever, which, in their opinion, could be considered as having given origin, or disposed, to the calamity under which they suffered.

Cases ill.u.s.trative of the nature and cause of this malady are very rare. In the following case symptoms very similar are observable, so far as affecting the lower extremities. That the medulla spinalis was here affected, and in its lower part, is not to be doubted: but this, unfortunately, was never ascertained by examination. It must be however remarked, that this case differed from those which have been given of this disease, in the suddenness with which the symptoms appeared.

_A. B._ aged twenty-six years, during a course of mercury for a venereal affection, was exposed to severely inclement weather, for several hours, and the next morning, complained of extreme pain in the back, and of total inability to employ voluntarily the muscles of the lower extremities, which were continually agitated with severe convulsive motions. The physician who attended him employed those means which seemed best calculated to relieve him; but with no beneficial effect. The lower extremities were perpetually agitated with strong palpitatory motions, and, frequently, three or four times in a minute, suddenly raised with great vehemence two or three feet from the ground, either in a forward or oblique direction, striking one limb against the other, or against the chairs, tables, or any substance which stood in the way. To check these inordinate motions, no means were in the least effectual, except striking the thighs forcibly during the more violent convulsions. No advantage was derived from all the means which were employed during upwards of twelvemonths.

Full ten years after this period, the unhappy subject of this malady was casually met in the street, s.h.i.+fting himself along, seated in a chair; the convulsive motions having ceased, and the limbs having become totally inert, and insensible to any impulse of the will.

It must be acknowledged, that in the well-known cases, described by Mr. Potts, of that kind of Palsy of the lower limbs which is frequently found to accompany a curvature of the spine, and in which a carious state of the vertebrae is found to exist, no instructive a.n.a.logy is discoverable; slight convulsive motions may indeed happen in the disease proceeding from curvature of the spine; but palpitating motions of the limbs, such as belong to the disease here described, do not appear to have been hitherto noticed.

Whilst striving to determine the nature and origin of this disease, it becomes necessary to give the following particulars of an interesting case of Palsy occasioned by a fall, attended with uncommon symptoms, related by Dr. Maty, in the third volume of the Medical Observations and Inquiries. The subject of this case, the Count de Lordat, had the misfortune to be overturned from a pretty high and steep bank. His head pitched against the top of the coach, and was bent from left to right; his left shoulder, arm, and especially his hand, were considerably bruised. At first he felt a good deal of pain along the left side of his neck, but neither then, nor at any other time, had he any faintings, vomitings, or giddiness.-On the sixth day he was let blood, on account of the pain in his shoulder and the contusion of his hand, which were then the only symptoms he complained of, and of which he soon found himself relieved.-Towards the beginning of the following winter, he began to find _a small impediment in uttering some words, and his left arm appeared weaker_. In the following spring, having suffered considerably from the severities of the winter campaign, he found _the difficulty in speaking, and in moving his left arm, considerably increased_.-On employing the thermal waters of Bourbonne, his speech become freer, but, on his return to Paris, the Palsy was increased, and the arm somewhat wasted.-In the beginning of the next spring he went to Balaruc; when he became affected with _involuntary convulsive motions all over the body_. The left arm withered more and more, _a spitting began_, and now it was _with difficulty that he uttered a few words_. Frictions and sinapisms were successively tried, and an issue, made by a caustic, was kept open for some time without any effect; but no mention is made of what part the issue was established in.

Soon after this, and three years and a half after the fall, Doctor Maty first saw the patient, and gives the following description of his situation. "A more melancholy object I never beheld. The patient, naturally a handsome, middle-sized, sanguine man, of a cheerful disposition, and an active mind, appeared much emaciated, stooping, and dejected. _He still walked alone with a cane, from one room to the other, but with great difficulty, and in a tottering manner_; his left hand and arm were much reduced, and would hardly perform any motion; _the right was somewhat benumbed, and he could scarcely lift it up to his head; his saliva was continually trickling out of his mouth, and he had neither the power of retaining it, nor of spitting it out freely_. What words he still could utter were monosyllables, and these came out, after much struggle, in a violent expiration, and with such a low voice and indistinct articulation, as hardly to be understood but by those who were constantly with him. He fetched his breath rather hard; his pulse was low, but neither accelerated nor intermitting. He took very little nourishment, could chew and swallow no solids, and even found great pain in getting down liquids. Milk was almost his only food; his body was rather loose, his urine natural, his sleep good, his senses, and the powers of his mind, unimpaired; he was attentive to, and sensible of every thing which was said in conversation, and shewed himself very desirous of joining in it; but was continually checked by the impediment in his speech, and the difficulty which his hearers were put to. Happily for him he was able to read, and as capable as ever of writing, as he shewed me, by putting into my hands an account of his present situation, drawn up by himself: and I am informed that he spent his time to the very last, in writing upon some of the most abstruse subjects."

This gentleman died about four years after the accident, when the body was examined by Dr. Bellett and Mons. Sorbier, who made the following report:

"We first examined the muscles of the tongue, which were found extenuated and of a loose texture. We observed no signs of compression in the lingual and brachial nerves, as high as their exit from the basis of the cranium and the vertebrae of the neck; but they appeared to us more compact than they commonly are, being nearly tendinous. The dura mater was in a sound state, but the pia mater was full of blood and lymph; on it several hydatids, and towards the falx some marks of suppuration were observed. The ventricles were filled with water, and the plexus choroides was considerably enlarged, and stuffed with grumous blood. The cortical surface of the brain appeared much browner than usual, but neither the medullary part nor cerebellum were impaired. We chiefly took notice of the Medulla Oblongata, this was greatly enlarged, surpa.s.sing the usual size by more than one third. It was likewise more compact. The membranes, which, in their continuation, inclose the spinal marrow, were so tough that we found great difficulty in cutting through them, and we observed this to be the cause of the tendinous texture of the cervical nerves. The marrow itself had acquired such solidity as to elude the pressure of our fingers, it resisted as a callous body, and could not be bruised. This hardness was observed all along the vertebrae of the neck, but lessened by degrees, and was not near so considerable in the vertebrae of the thorax. Though the patient was but nine and thirty years old, the cartilages of the sternum were ossified, and required as much labour to cut them asunder as the ribs; like these they were spungy, but somewhat whiter. The lungs and heart were sound. At the bottom of the stomach appeared an inflammation, which increased as it extended to the intestines. The ileum looked of that dark and livid hue, which is observed in membranous parts tending to mortification. The colon was not above an inch in diameter, the r.e.c.t.u.m was smaller still, but both appeared sound.-From these appearances, we were at no loss to fix the cause of this gradual palsy in the alteration of the medulla spinalis and oblongata."

Dr. Bellett offers the following explanation of these changes. "I conceive, that, by this accident, the head being violently bent to the right, the nervous membranes on the left were excessively stretched and irritated; that this cause extended by degrees to the spinal marrow, which being thereby compressed, brought on the paralytic symptoms, not only of the left arm, but at last in some measure also of the right. This induration seems to have been occasioned by the constant afflux of the nutritive juices, which were stopt at that place, and deprived of their most liquid parts; the grosser ones being unable to spread in the boney cavity, by which they were confined, could only acquire a greater solidity, and change a soft body into a hard and nearly osseous ma.s.s. This likewise accounts for the increase of the medulla oblongata, which being loaded with more juices than it could send off, swelled in the same manner as the branches of trees, which will grow of a monstrous size, when the sap that runs into them is stopt in its progress. The medulla oblongata not growing so hard as the spinalis, was doubtless owing to its not being confined in an osseous theca, but surrounded with soft parts, which allowed it room to spread. The obstruction from the bulk of this substance must have affected the brain, and probably induced the thickening of the pia mater, the hydatids, and the beginning of suppuration, whereas the dura mater, being of a harder texture, was not injured[11]."

[Footnote 11: Medical Observations and Inquiries, Vol. III.

p. 257.]

In some of the symptoms which appeared in this case, an agreement is observable between it and those cases which are mentioned in the beginning of these pages. The weakened state of both arms; the power first lessening in one arm, and then in a similar manner in the other arm; the affection of the speech; the difficulty in chewing and in swallowing; as well as of retaining, or freely discharging, the spittle; the convulsive motions of the body; and the unimpaired state of the intellects; const.i.tute such a degree of accordance as, although it may not mark an ident.i.ty of disease, serves at least to show that nearly the same parts were the seat of the disease in both instances.

Thus we attain something like confirmation of the supposed proximate cause, and of one of the a.s.sumed occasional causes.

Whilst conjecturing as to the cause of this disease, the following collected observations on the effects of injury to the medulla spinalis, by Sir Everard Home, become particularly deserving of attention. It thence appears, that none of the characteristic symptoms of this malady are produced by compression, laceration, or complete division of the medulla spinalis.

"Pressure upon the medulla spinalis of the neck, by coagulated blood, produced paralytic affections of the arms and legs; all the functions of the internal organs were carried on for thirty-five days, but the urine and stools pa.s.sed involuntarily[12].

[Footnote 12: A coagulum of blood, the thickness of a crown-piece, was found lying upon the external surface of the dura-matral covering of the medulla spinalis, extending from the fourth vertebra colli to the second vertebra dorsi. The medulla spinalis itself was uninjured.]

"Blood extravasated in the central part of the medulla, in the neck, was attended with paralytic affection of the legs, but not of the arms[13].

[Footnote 13: The sixth and seventh vertebra colli were dislocated, the medulla spinalis, externally, was uninjured; but in the centre of its substance, just at that part, there was a coagulum of blood nearly two inches in length.]

"In a case where the substance of the medulla was lacerated in the neck, there was a paralysis in all the parts below the laceration, the lining of the sophagus was so sensible, that solids could not be swallowed, on account of the pain they occasioned[14].

[Footnote 14: The seventh vertebra colli was fractured, and the medulla spinalis pa.s.sing through it, was lacerated and compressed.]

"When the medulla of the back was completely divided, there was momentary loss of sight, loss of memory for fifteen minutes, and permanent insensibility in all the lower parts of the body. The skin above the division of the spinal marrow perspired, that below did not.

The wounded spinal marrow appeared to be extremely sensible[15]."

_Philosophical Transactions_, 1816, p. 485.

[Footnote 15: The spinal marrow, within the ca.n.a.l of the sixth vertebra dorsi, was completely destroyed by a musket ball. The person lived four days.]

In two of the cases already noticed, symptoms of rheumatism had previously existed; and in Case IV. the right arm, in which the palpitation began, was said to have been very violently affected with rheumatic pain to the fingers ends. The consideration of this case, in which the palpitation had been preceded, at a considerable distance of time, by this painful affection of the arm, led to the supposition that this latter circ.u.mstance might be the cause of the palpitations, and the other subsequent symptoms of this disease. This supposition naturally occasioned the attention to be eagerly fixed on the following case; and of course influenced the mode of treatment which was adopted.

_A. B._ subject to rheumatic affection of the deltoid muscle, had felt the usual inconveniences from it for two or three days; but at night found the pain had extended down the arm, along the inside of the fore-arm, and on the sides of the fingers, in which a continual tingling was felt. The pain, without being extremely intense, was such as effectually to prevent sleep: and seemed to follow the course of the brachial nerve. Whilst ascertaining the propriety of this conclusion, the pain was found to ramify, as it were, on the fore and back part of the chest; and was slightly augmented by drawing a deep breath.

These circ.u.mstances suggested the probability of slight inflammation, or increased determination to the origin of the nerves of these parts, and to the neighbouring medulla. On this ground, blood was taken from the back part of the neck, by cupping; hot fomentations were applied for about the s.p.a.ce of an hour, when the upper part of the back of the neck was covered with a blister, perspiration was freely induced by two or three small doses of antimonials, and the following morning the bowels were evacuated by an appropriate dose of calomel. On the following day the pains were much diminished, and in the course of four or five days were quite removed. The arm and hand felt now more than ordinarily heavy, and were evidently much weakened: aching, and feeling extremely wearied after the least exertion. The strength of the arm was not completely recovered at the end of more than twelvemonths; and, after more than twice that time, exertion would excite the feeling of painful weariness, but no palpitation or other unpleasant symptom has occurred during the five or six years which have since pa.s.sed.

The commencement, progress, and termination of this attack; with the success attending the mode of treatment, and the symptoms which followed, seem to lead to the conjecture, that the proximate cause of the disease, in this case, existed in the medulla spinalis, and that it might, if neglected, have gradually resolved itself into that disease which is the object of our present inquiry.

Some few months after the occurrence of the preceding case, the writer of these lines was called to a female about forty years of age, complaining of great pain in both the arms, extending from the shoulder to the finger ends. She stated, that she was attacked in the same manner as is described in the preceding case, about nine months before; that the complaint was considered as rheumatism, and was not benefited by any of the medicines which had been employed; but that after three or four weeks it gradually amended, leaving both the arms and hands in a very weakened and trembling state. From this state they were now somewhat recovered; but she was extremely anxious, fearing that if the present attack should not be soon checked, she might entirely lose the use of her hands and arms.

Instructed by the preceding case, similar means were here recommended.

Leeches, stimulating fomentations, and a blister, which was made for sometime to yield a purulent discharge, were applied over the cervical vertebrae; and in the course of a very few days the pain was entirely removed. It is regretted that no farther information, as to the progress of this case, could be obtained.

On meeting with these two cases, it was thought that it might not be improbable that attacks of this kind, considered at the time merely as rheumatic affections, might lay the foundation of this lamentable disease, which might manifest itself at some distant period, when the circ.u.mstance in which it had originated, had, perhaps, almost escaped the memory. Indeed when it is considered that neither in the ordinary cases of Palsy of the lower extremities, proceeding from diseased spine, nor in cases of injured medulla from fractured vertebrae, any of the peculiar symptoms of this disease are observable, we necessarily doubt as to the probability of its being the direct effect of any sudden injury. But taking all circ.u.mstances into due consideration, particularly the very gradual manner in which the disease commences, and proceeds in its attacks; as well as the inability to ascribe its origin to any more obvious cause, we are led to seek for it in some slow morbid change in the structure of the medulla, or its investing membranes, or theca, occasioned by simple inflammation, or rheumatic or scrophulous affection.

It must be too obvious that the evidence adduced as to the nature of the proximate and occasional causes of this disease, is by no means conclusive. A reference to the test therefore which will be yielded by an examination of some of the more prominent symptoms, especially as to their agreement with the supposed proximate cause, is more particularly demanded. Satisfied as to the importance of this part of the present undertaking, no apology is offered for the extent to which the examination is carried on.

If the palpitation and the attendant weakness of the limbs, &c. be considered as to the order in which the several parts are attacked, it is believed, that some confirmation will be obtained of the opinion which has been just offered, respecting the cause, or at least the seat, of that change which may be considered as the proximate cause of this disease.