PROGRESSIVE LOCOMOTOR ATAXY. No very obvious paralysis of the blad-der or lower bowel.

“ No necessary impairment of sexualpower.

“No tingling or kindred phenomena.“No marked impairment ofmuscular nu-

trition and irritability.“ No impairment of the mental faculties.“ Occasional injection ofthe conjunctive,

with contraction of the pupils.”Dr. Radcliffe takes no notice, in this sum-

mary, of the usual order of precedence inthe various symptoms. This, as describedby Duchenne, is shown in the first of thefollowing cases. The first period is charac-terized by “ paralysis of one or several ofthe motor nerves of the eye, complicatedwith paralysis of the optic nerve, and byerratic, boring pains ; the second, by the

of defects of coordination, and,soon after or simultaneously, by muscularand cutaneous insensibility, generally inthe lower limbs, or sometimes in the upperlimbs; the third, by generalization of thedisease.”

Case I.—Mr. came to me last Feb-ruary, in accordance with the suggestionof Dr. Knight. He said he wished to knowwhether electricity would be of any use inrestoring his lost power. He seemed tohave very little faith in any kind of treat-ment, and his mental condition was one ofgreat despondency. As he expressed him-self, he thought he might as well die of thisdisease as of any other, if he was going todie at all.

He is about 59 years of age, has beenmarried thirty years and had one child,which is dead. His business has been toconstruct and fit the wood-work for machi-nery. His father died of paralysis. Hesays he has had rheumatism very severelyand frequently in every part of his body forthe last thirty years. He is not aware ofany cardiac trouble. He has many timesduring the last thirty years strained him-self by lifting heavy weights—has at timesbeen laid up for several days on that ac-count; as he said, has had a “crick” in

Read before the Boston Society for Medical Observationby S. G. Webber, M.D., Boston.

This diseasewas knowm formerlyas tabes dor-salis, and as such is well describedby Rom-berg ; but the whole of the group of symp-toms belonging to it was not fully recog-nized until Duchenne (of Boulogne) com-municated a memoir on that subject to theSociety of Medicine of Paris in 1857. Thismemoir is reproduced in all its essentialparticulars in his work “ L’Electrisation

Since that date, observers inother places have continued the investiga-tions, and an extensive literature on thesubject has been created.

The two cases here related illustrate twodifferent forms of the disease ; but in nei-ther do all the symptoms occur wjiiqh aro

metimes met.’ For a luller descriptionreference may be made to Trousseau’s Cli-nique Medicale de l’H6tel Dieu, t. ii. ; to St.George’s Hospital Reports, vol. i.; an arti-cle by Dr. J. L. Clarke; or to Reynolds’sSystem of Medicine, vol. ii. Dr. Radcliffe,in the last work, sums up the distinctivesymptoms as follows:—

“A peculiar gait, arising from want ofcoordinating motor power in the lower ex-tremities—a gait precipitate and stagger-ing, the legs starting hither and thither ina very disorderly manner, and the heelscoming down with a stamp at each step.

“No true paralysis in the lower extremi-ties or elsewhere.

“ Characteristic neuralgic pains, erratic,paroxysmal, in the feet and legs chiefly—-pains of a boring, throbbing, shooting cha-racter, like those caused by a sharp electricshock.

“ More or less numbness in the feet andlegs chiefly, in all forms of sensibility, ex-cepting that by which differences of tem-perature are recognized. •

“ Frequent impairment of sight or hear-ing, one or both.

_

“ Frequent transitory or permanent stra-bismus or ptosis, one or both.

2

his back by turns for several years. Butnothing of this kind had occurred for monthsprevious to his paralysis.

For many years he has had attacks ofnumbness in his right thumb, so that hecould not write, especially in winter. Forthis he had rubbed his thumb with alcohol.If the attack was very severe, he might haveonly one dui’ing the winter ; if less severe,he might have more.

Three or four years ago, his eyes wereaffected, on which account he consultedDr. Williams, who told him he had amauro-sis. The right eye was the worst, and wasalmost blind. The affection of the eyescame on rather suddenly. Subsequently,the amaurosis improved. He acknowledgesno trouble in the head and no vertigo.

About one year ago last January, he no-ticed, while at work, that he had suddenlylost power over the left leg. He could not,while sitting on his stool, extend his leg.He did not fall down when he tried to walk,but could draw his leg after him. If hestumbled, however, he could not recoverhis balance. At the same time with themotor paralysis, there was a slight loss ofsensation in the same leg. He seems sub-sequently to have regained considerablecontrol over the muscles of his leg, or elsethe paralysis was not so complete as hestated, since, when 1 saw him, notvery marked.

For several months there was a slow ad-vance in the paralytic symptoms. The pa-ralysis extended to his right leg, and thatbecame worse than the left. It graduallyextended up the right side to the right armand then to the left arm.

Last December, he had paralysis of mo-tion and somewhat of sensation in the rightside of his face, and ptosis of the righteyelid. This did not last long. He wethis face in the morning in cold water, andused friction with a coarse salted towel;to this he ascribes the impi'ovement whichtook place in his facial paralysis.

He thinks that since December he hasbeen losing ground slowly, and that the dis-ease has been gradually advancing.

Now, he has some difficulty in seeing,especially with his right eye, which reactsrather sluggishly under the influence oflight. He has no abnormal sensations, asof itching, tingling, formication, &c. Hehas a sensation as of a cord bound roundabout the middle of the left arm. Thissensation exists nowhere else. He com-plains of a sensation of cold nearly all thetime, especially in the feet, up to the mid-dle of the leg, and to a less degree in the

body, but most marked in the left great toeand along the inside of the foot to the heel,and here it is accompanied with numbness.It exists to a less degree in correspondingparts of the right foot. The numbness alsoextends partly up the leg.

He balances tolerably well, and with hiseyes shut can walk, though with a veryunsteady gait. He can stand with his feettogether and eyes shut, but sways back-wards and forwards more than is naturalin health. He thinks he has most difficultyin moving his legs in the morning, afterlying a-bed all night.

When walking, he feels as though hemust go fast, so as not to fall forwards, sothat his feet may keep up with his head.

Sensation is not very good in the back ofhis head, and he is very sleepy. He feelsbest when lying down.

His appetite is poor; he is constipated,but is not troubled with flatus.

The examination ceased here, owing tothe lateness of the hour, and I told him Iwould call the next day at his house for thepurpose of examining the condition of thevarious sensations. The next day, excusewas made that his wife was sick, and Ihave not heard from him since.

Case II.—0. McG., a laborer, I saw atthe City Hospital, in the service of Dr.Dial . wi » has kindly permitted me tomal jf the case tu further illustrathe subject. The hospital record statesthat he was a hard drinker till eight yearsago, and since that time has indulged im-moderately in whiskey, but has alwaysbeen well and strong till last May. Aboutone month before that time he was knockeddown by a horse, which trod upon his rightside. He is not aware of any injury to hishead or back, though after the accident hehad much pain in his side on breathing.

About May 1st, he first experienced acold sensation in the left foot, as of a wetstocking drawn over it. In a month, theright foot was affected in a similar way.The sensation gradually extended, and inthree months had reached the knees, whenhe was obliged to give up work.

He had no headache nor any trouble withhis head for three months after the troublein the lower extremities commenced. Hethen had sharp, darting pains in teeth, faceand head, and since that time has felt light-headed.

At present, the sensation of coldness hasinvaded the whole of the lower extremitiesand the body below a line passing throughthe lower ribs. There is also marked im-pairment of sensibility, which is worst at

3

extremities more frequently and more se-verely than elsewhere. It would also bevery unusual that one should have rheuma-tism repeatedly, and the pains be sosevere that their recollection was disagree-able, and yet be aware of no cardiac com-plication. I ought to say, however, that Idid not make any examination, intendingto do so while he was in bed next day. Thedisease is sometimes of very long duration ;

thus Mr. Carre refers to cases which hadlasted 20, 21, 24 and even 28 years.

In the 2d volume of Reynolds’s Systemof Medicine, Dr. C. B. Radcliffe does notrecognize the fact that the upper extremi-ties may be involved. Yet in the first casethere was partial loss of sensation in theright thumb, occurring before the loss ofmotion in the lower limbs. Many othercases are on record, one by Duchenne, inwhich the abnormal condition was perceiv-ed in the upper extremity. Also, at postmortems, changes have been recognized inthe cervical region of the cord. In a postmortem by Dr. J. L. Clarke, recorded in theLancet, June 10, 1865, the change wasnoticed as high as the second cervicalnerve.

The change usually found after death, asdescribed by various observers, is degene-ration of the posterior columns of the spi-nal cord., chiefly in the lumbar region, occa-sionally extending as high as the cervical,rarely confined to the latter region. Thisdegeneration may extend to the neighbor-ing posterior cornua and gray matter ; itmay even involve the posterior roots. Thislast is not a constant lesion, but is usuallyfound where there is loss of sensation.“ The degeneration is essentially charac«terized by gradual and progressive atrophyof the nervous elements, and by a gradualand progressive development of the con-nective tissue which separates them.”—Carre, p. 189.

Similar changes have been found in theoptic nerves, and also other cranial nerveswhen these have been affected, but moreespecially the motor nerves of the eyes.

Various influences have been assigned asthe cause of this disease. Comparativelya large number of patients have sufferedeither from strains of the back or fatigue,or have been exposed to wet and cold dur-ing a long period of time. In both thecases herewith reported, there is a historyof injury ; in the first case often repeatedstraining, and the first pains appeared atabout the same period as the first strain, orthirty years ago. In the second case, thereis a history of an injury to the side, and

the feet and decreases upwards. Reflexaction is very much diminished or lost inthe feet. Sensation and motion of the up-per extremities seem to be impaired. Eye-sight but little affected ; he sometimes seesspecks floating before his eyes. April 1st,the day of entrance into the hospital, in theafternoon, he complained of a cloudiness ofvision for the first time. Pupils act natu-rally. Taste and smell unimpaired.

He is obliged to micturate quite frequent-ly,, and sometimes when he feels the desireto do so, he has a passage of urine unwit-tingly. He never passes urine whileasleep.

His gait, when walking, is peculiaidycharacteristic of this affection ; much moreso than in the previous case. When at-tempting to walk, even if supported oneach side, his legs are moved about in avery disorderly manner, sometimes side-ways, sometimes forwards and backwards,sometimes crossing each other, and the heelcomes down with emphasis upon the floor.If his eyes are shut, he cannot stand whenthe feet are brought together.

He became dissatisfied and homesick,and left the hospital in two days.

Only occasionally, as at the commence-ment of the attack in the first case, is therereal paralysis of motion; the patient isunable to walk, and is perhaps confined tothe bed, but can movo bis linibs withpower,though in a disorderly manner and perhapsby jerks, not being able to check them atjust the point desired.

The affection with which locomotor ataxyis most likely to be confounded is diseaseof the cerebellum, especially one of slowgrowth, as a tumor. But in an affection ofthe cerebellum there is found vertigo, some-times vomiting, headache, or perhaps throb-bing sensations in the head. The defectin motion is also different. The gait is un-steady rather from lack of power over themuscles, or on account of the vertigo, thanfrom defect of coordinating power. Thevision may be affected in both diseases.

The second case is one of the more erra-tic cases, commencing with the second pe-riod, the symptoms of the first period ap-pearing only intercurrently.

It is certainly a long durationfor the dis-ease to have dated back thirty years, yetthat is the date at which the rheumaticpains were first felt, and according to hisdescription they seemed to resemble thetearing and boring pains of locomotor ataxymore than those of rheumatism. Thoughnot stated in the notes, I believe he com-plained of the pains having been in his lower

4The despondency in the first case, and

the similar feeling of homesickness in thesecond, are phenomena worthy of notice.

If the disease is considered a derange-ment of the vaso-motor system, much thatis otherwise anomalous ceases to be so.Many of the earlier symptoms are explain-ed by the existence of congestion of thenerve centres. In the first case, there wasa sudden loss of power over the left leg.Previous to that, amaurosis had occurred.Both these symptoms had diminished verymuch in intensity. The numbness of theright thumb had occurred and disappearedagain and again. Later, there was paraly-sis of the right side of the face, which hadnearly or quite disappeared when I saw thepatient. In other cases, certain symptomsare found occurring and then disappearing,as strabismus in M. Duchenne’s case No.122 ; also incontinence of urine. Occa-sionally the amaurosis, which occurs earlyin the disease, so far disappears that thepatient does not think to speak of it with-out being questioned. Congestion maywell be supposed to be the cause of suchtemporary symptoms.

Another proof of the truth of this theoryis given by M. Trousseau, in the congestedstate of the conjunctiva and contraction ofthe pupil which he observed in many -

Hcntp.; bn* :' ■ conf r'ri >n disappeared . Ithe pupih diUted-v.-Heis here was an e -

cess of severe pain. M Duchcnnt* andRadclifte have observed the same.

The means used to dissipate the numb-ness of the thumb and the paralysis of theface might well act by causing reflex chan-ges in the vessels of the cord :—stimula-ting friction to the parts affected and coldwater to the face.

Another fact directly bearing upon thispoint may be found in Trousseau’s Clinique,in an account by Luys of the microscopicappearances. “ In following into the greysubstance of the fourth ventricle the inves-tigation upon the trunk of the external mo-tor nerve of the eye, up to its point of realorigin, a series of large vascular trunkswere seen in the tract of the primitivefibres of this nerve, which they probablymust have compressed in a notable degree.”

The coldness complained of by both thesepatients is a proof of vaso-motor distur-bance. That this coldness is not merely asubjective sensation there is nothing record-ed in recent writers to show ; but Hassestates that there is a reduction of l°-2° R.in the part.

One case, at least, is on record where thepatient, dying of some acute disease, little

consequent difficulty of breathing, about amonth before the peculiar coldness of thefeet was noticed.

Duchenne states briefly—“Some pa-tients accuse sudden or too prolonged cool-ing, suppression of transpiration ; thus, forexample, there is a sportsman who oftenhas remained a long time with the feet andlegs in the water. Once, the phenomenaof this disease have shown themselves aftera too prolonged sitz bath. Trousseau men-tions hereditary taint, also, as a predispos-ing cause. This may appear in the firstcase, where it is stated that the father diedof paralysis.

Out of 132 cases referred to by Carre,some of which, however, were not of thisdisease, 66 have no cause assigned, and noantecedents are given. In 17 there was aprevious history of syphilis, in 8 of rheu-matism, 6 had been exposed to unusual andfrequent fatigue, 23 had been subjected tocold and dampness in various ways—eitherdwelling in damp houses, sleeping in theopen air on damp ground, working in dampplaces, or were subject to sudden coolingwhile perspiring freely, and immediatelyafter felt the first signs of the disease. In9, there had been emotional excitement orlong-continued anxiety. In 5, disease ofthe nervous system had existed among theancestors. In 5, abuse of wine or venerealexcess had been noted.

Topinard, who has collected a very largenumber of cases, says that among predis-posing causes, “ there are few so efficacious,when united together, as disappointment,emotion, and bad hygiene.” He also looksupon rheumatism as playing a certain rdlein this relation. With regard to dampness,he says :—“ But the most common cause isdampness and sudden cooling, since 16times it is indicated as determining and 15times as predisposing, that is to say 31times in all.” He concludes the chapteron Etiology: — “Progressive locomotorataxy is a disease peculiar to adult age ;

more frequent in males, in those who areaddicted to excesses of all kinds, who areexposed to dampness and to great fatigue.Among temperaments, the nervous alone iscertainly disposed to a particular form ofthe disease. Among diatheses, the rheu-matic is the only one whose influence is in-contestable.” In some cases, he thinks itseems to be hereditary.

Eisenmann recognizes essentially thesame causes, only concussion of the spinalcord through falls and strains he considersas perhaps causing rather a local disease,and not properly locomotor ataxy.

or nothing has been found to account forthe ataxic symptoms.

I quote from Topinard the appearancefound after death. The patient had hadsymptoms of locomotor ataxy since thirteenyears, and for six months had been unsteadyin his gait. Cutaneous sensibility was al-tered. He died during an attack of variola,in the last part of which he had true mus-cular paralysis. “ At the autopsy, abso-lutely nothing was found to the naked eyein the encephalon, cerebellum, the roots orcolumns of the spinal cord. The wholecord was very much injected ; the opticnerves were grey, semi-transparent, softenedbetween the papillae and the corpora geni-culata exclusive. One of the common mo-tor nerves was flattened, diminished in vol-ume, but was not grey. The microscopicexaminationfound the posteriorcolumns androots healthy, and the usual alteration ofthe optic nerves. Having myself compareda transverse section of the lumbar enlarge-ment of this patient with a similar sectionfrom theataxic patient ofwhom we had madethe autopsy fifteen days before in the ser-vice of M. Trousseau, I have been able totestify to that integrity. However, in moreclosely examining it, it has seemed to methat the posterior border of the grey com-missure, in place of being suddenly arrest-ed, was diffuse and insensibly confoundedwith Listr ceiitie of the posterior column,as if the iatter commenced to be altered intheir depth.”

Several cases are recorded in which thesymptoms of locomotor ataxy occurred, butwhich recovered entirely. It can scarcelybe supposed that in such the nerve fibreshad been completely disorganized.

The causes above referred to are calcu-lated to produce derangement in the circu-lation. The effect of damp cold in derang-ing the circulation is well known, and thevaso-motor nerves are peculiarly suscepti-ble to influences of an emotional nature

From these facts in regard to some of thefugitive symptoms of the disease, from thecase in which such accurate observers asMM. Gubler, Luys and Duchenne foundnothing but congestion of the posteriorcords, from the fact that patients have en-tirely recovered after the second stage ofthe disease has made great progress, andfrom the nature of the causes which act toproduce it, I am inclined to look upon it asan affection primarily of the vaso-motornerves, producing congestion, which beingonly moderate in degree and being longcontinued causes proliferation of connectivetissue, and, as it were, strangles the nervefibres, causing their absorption.

The question arises, what is the cause ofthis congestion, of this vaso-motor distur-bance ? The sympathetic is looked uponas the regulator of the bloodvessels, anddisturbance of its function or lesion of itsstructure interferes materially with the dis-tribution of the blood. M. Duchenne is in-clined to refer the changes of the cord tochanges in that nerve. In an article in theGazette Hebdomadaire, Feb. 19 and March4,1864, he advocates thisview. He foundshis belief principally on the vascular phe-nomena connected with the eye, which hasbeen already referred to, and on the symp-toms connected especially with the bowels,bladder and other viscera. He thinks thatif the ganglia connected with the lowerpart of the cord were examined, changesmight be found to throw light upon thesclerosis of the cord.

One case is recorded by Donnezan in theGazette Hebdomadaire, May 6, 1864, inwhich, though the autopsy was not com-plete, a partial answer is given on thispoint. M. M was attacked with thepains of this disease in 1858, and in 1860was unable to walk without support. Ob-stinate constipation caused a distressingsensation of a girdle around the body.Towards the last, he lost all motor power,and died in 1864. At the autopsy, the pos-terior columns in the cervical region werefound somewhat affected ; the dorsal andlumbar regions were extensively changed.Only the upper cervical sympathetic gang-lion could be obtained for examination,owing to the opposition of friends. It wasfound to be harder, more resisting, and ofa more yellowish white than in health.The cells did not differ from those of ahealthy ganglion. The communicatingbranch, however, had become tendinous;the proper nervous tissue had disappeared.

Friedreich, on the other hand, gives avery detailed account of an autopsy, andstates that the sympathetic was normal.—Virchow's Archives, vol. xxvi.

In an examination made by Luys, andquoted by Trousseau, the ganglia on theposterior roots in the lumbar region wereall increased in size, and unusually red andvascular. Their enveloping membrane wasconsiderably thickened. On section, thecapillaries were found dilated. The gang-lionic corpuscles (cells) were covered withbrown pigment-granules, and some weretorn and shrunken ; others were voluminousand pale. No part of the sympathetic wasexamined.

So far as I have been able to learn, thisis the extent of the investigation in this di-

6rection. Much can be said in opposition tothe theory of the sympathetic origin of thedisease. Nothing can be settled till a largernumber of cases have been examined withparticular reference to this point.

Treatment.—The benefit to be anticipateddepends much upon the view taken of thepathology. If the disease is, in its earlierstages, essentially avaso-motor disturbance,a simple congestion, the expectation ofbenefit would be greater the earlier treat-ment was commenced.

Cases have been recorded where the lossof coordinating power was so great thatthe patient could not stand ; yet in three orfour months restoration was complete. Itis not necessary, then, to despair of benefit-ing a patient, even when he seems to be inthe advanced stages of the disease.

Electricity seems to do good occasionally,but at best the benefit from it is very un-certain.

The best treatment seems to be, withproper diet and hygiene, that proposed byWunderlich—nitrate of silver in quarter-grain doses three times a day for four orfive weeks; after that time it would bewell to omit the drug for a week or so, to iavoid coloring the skin. The same coursemay then be repeated. It may be !necessary to give the nitrate in sm' n rdoses at first, on account of gastric irtion, and occasionally it must be entfi r jomitted. If no benefit follows, the do.should be increased. Althaus gives, withthe nitrate, the hypophosphite of soda, andthinks it better than the nitrate alone.

Many have been relieved by this treat-

ment. In only a few cases has completecure been obtained. One such is to be foundrecorded in the Gaz. des Hop., Jan. 4, 1863.

Carre mentions 16 cases where a curewas obtained, or the benefit was considera-ble. In one of these, the nitrate was ad-ministered hypodermically, near the spinalcolumn. In 6 cases, the benefit was onlypartial; either a relapse occurred, or aftera while the drug lost its power, or couldnot be tolerated. In 4 cases, no benefit atall was obtained.

The good effect of the drug is usuallyobserved at the end of a week or fortnight.The first favorable effect is relief of thepain, subsequently return of sensibility,and finally return of coordinating power.Sometimes sightisrestored. Simultaneous-ly the general health is benefited, diges-tion is improved and constipation ceases.

If the congestive theory is correct, bro-mide of potassium, belladonna and ergotmight be of benefit.

Note.—It will be noticed that I have usedthe term “ congestion ” to express the pri-mary morbid condition. It is considered bymany to be a chronic myelitis. Withoutintending to deny this, I have used “ con-gestion to express the view that hyperas;-mia of the part was the cause of the subse-quent change, and to bring out more clear-ly the role p, fed by tin vaso-motor nerves.Tne reason ■ poster : o columns are generally attack; d may that there is agreater abundance of vessels and intersti-tial tissue naturally in them. See Archivesde Physiologie Normale et Pathologique, No.2, p. 329.