Spasmodic torticollis: Its cause and treatment

SPASMODIC

TORTICOLLIS: J. TORRANCE

ITS CAUSE AND TREATMENT* RUGH,

PHILADELPHIA,

A

of the Iiterature of so-caIIed “spasmodic torticoIIis ” reveaIs confusion and uncertainty as to its

the etiology were extant, nameIy, the psychogenic or as it is sometimes termed, the functiona1; and the organic, in which structura1 changes were predicated in the centra1 nerve system. These cIassifications stiI1 obtain despite the disparity between the cIinica1 evidences of two patentIy differing conditions. The first, or psychogenic, theory postuIates abnorma1 emotiona reactions in a patient or subject of distinctIy neurotic type in whom, as a consequence of some menta1 or physica (or both) stress or fatigue, muscle spasms occur. The inherent instabiIity of the system (either congenita1 or acquired) renders the nerve ceIIs in the base of the brain (which suppIy the eIeventh cranial and the first and second cervica1 nerves) more susceptible to the externa1 stimuIi of occupation or surroundings, and result in their hyperactivity. In other words, it is a basicaIIy functiona weakness of the nerve ceIIs, which, under severe mental or physica stress graduaIIy produces a chronic spasm of the muscIes motivated by them. In the deveIopment of this theory, it is highIy probabIe that the ceIIs are in a state of debiIity or exhaustion from overuse which renders them more susceptibIe to irritative and stimuIating externa1 conditions, but which is not foIIowed by degenerative or pathologic changes in the cells. The second, or organic, theory predicates a pathoIogic lesion in the basa1 gangIia of incIuding the striatum, the the brain, capsuIe, the basa1 nucIei or “a disturbed Iabyrinthine function due to a uniIatera1 Iesion of the brain stem” (Grinker’). Grinker aIso beIieves that the condition invoIves the capsuIe and at times the adjacent nucIei. Post-mortem examination in severa cases has shown pathoIogic

I. Cast as appIied.

etioIogy as we11 as hopeIessness in the prognosis and treatment. NeuroIogists and neuroIogic surgeons have faiIed to agree as to the character and Iocation of the processes in the brain, responsibIe for the externa1 evidences of the dystonic disturbance. However, other facts arrest the attention, especiaIIy the cIinica1 descriptions of the affection and the reports of the pathoIogic findings in the few cases that have been studied post-mortem. An outstanding and most disturbing symptom has given the name to the condition and partIy accounts for much of the confusion existing in its discussion and Iiterature and has hindered accurate description and uniform understanding of this most distressing aiIment. Even before any pathoIogic changes had been demonstrated by post-mortem studies, two theories as to * Presented

LL.D.

PENNSYLVANIA

STUDY

Frc.

M.D.,

at the meeting of the American Orthopedic 490

Association,

June 6-8, 1939, Buffalo, New York.

NEW SERIES VOL. XLIX,

No. 3

Rugh-Spasmodic

changes in the “large cells of the caudate and putamen” (AIpers and Drayer”). The writer beIieves it entireIy conceiv-

FIG. 2. First ceIIuIoid brace.

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relieved by proIonged fixation, w-ithout any reIation to underIving pathoIogy but purely with the idea of giving relief from the pain

FIG. 3. Second brace.

able that the Iong-standing and persistent cases of the psychogenic type of pure and uncomphcated spasm might deveIop secondary degenerative changes in the central motivating ceIIs but thus far there have been no published resuIts of post-mortem findings in such cases and no cIinica1 evidences of centraI pathoIogic compIications in this type. In two of the cases treated, the spasm had been present for eight or more years yet there was no evidence of muscIe or fibrous tissue changes and compIete reIief was obtained by the method of treatment to be described. The position of the head in a11 of these cases is anaIogous to that of the ischemic or traumatic wry neck commonIy seen in chiIdhood and foIIowing injury to the sternocIeidomastoid muscIe, but differs in that (I) the spastic type presents no structural shortening of the muscIes or infiItration of the intramuscuIar fasciae such as is aIways present in the traumatic type, and (2) the contraction is cIonic and not constant. From the series of cases treated, the writer beIieves there are two distinct types of the affection and that both theories are correct, each in its respective type. SeveraI of our earlier patients were compIeteIy

American

FIG. 4. End result.

the results of the and spasm. However, treatment and more intensive study of the symptoms of the cases evidenced a marked clinica difference between the two types and aIso suggested the probable cause of the failures foIIowing surgica1 and other methods of treatment. The differentiation of the two types is made from the character of the symptoms present. In the pure or psychogenic type of case, there are no other symptoms than the cIonic spasm of muscIes, singIy or in groups, while the organic type presents svmptoms referabIe to degenerative changes‘in the motor ceils. These changes are characterized by athetoid movements in various parts of the head and neck. Thus, it was felt that, in the psychogenic cases, there was probably some IocaI functiona disturbance rather than a degenerative condition in the motivating ceIIs. This distinction has been the criterion in the seIection and treatment of our cases. In severa of our patients, the spasm had persisted for six or more years, but no other symptoms have developed. In the organic type, however, the athetotic movements deveIoped earIy with the torticoIIis spasm and graduahy involved various parts of the upper body such as the tongue, neck, arms and hands and in these cases

492

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it was feIt that faiIure wouId foIIow the measures which proved successfu1 in the others. We beIieve there a sharp distinc-

TorticoIIis

SEPTEMBER, 1940

the stress of Iife, occupation or environment, deveIoped an asthenia or exhaustion of the basic ceIIs which in turn rendered

FIG. 5. Another type of brace to foIIow cast.

tion must be drawn between the two types and that the so-caIIed psychogenic should be known as the true spasmodic torticoIIis whiIe those cases which present other symptoms characteristic of degenerative changes in the basal gangha and ceIIs or in other parts of the base of the brain, shouId be cIassified by a titIe descriptive of the pathoIogy in those parts. Another strong argument in favor of this distinction Iies in the resuIts of the treatment. Heretofore, the common name of spasmodic torticoIIis carried with it a hopeIess outIook for reIief. Most writers cIaim that a11 methods of treatment, whether mechanica1, eIectrica1, surgica1 or physiotherapeutic, were faiIures or Ieft the patient aImost as badIy crippIed as before (this is especiaIIy evident after neurectomies). It is highly probabIe that where faiIure resuIted, it was due to a Iack of differentiation between these two outstanding types of cases. Stimulated by the faiIures of surgica1 and other measures and impressed by the theory of psychogenesis as exemplified in other conditions, we were led to fee1 that we were deaIing with a badIy balanced nerve system, which under

FIG. 6. End resuk in 46 year oId patient most resistant type.

with

them hypersensitive to externa1 stimuIi thus reffexIy setting up spasms in the muscIes motivated by them. In 1863, HiIton (London) published his Iectures on “Rest and Pain,” a book which remains a cIassic to the present day and provides a basis for the successfu1 treatment of many more recentIy recognized aiIments. He demonstrated that the mechanica1 and physioIogic rest of parts, which are or have been subjected to injury or strain, was one of the most potent factors in the restoration of heaIth to those parts. Acting upon the principle that the centraI ceIIs and the muscles motivated by them compIement each other, we decided to try pIacing the centra1 ceIIs at rest through immobiIization of the parts which they suppIy. This principIe of treatment is not new. It had been tried in this condition many years ago but was discarded as unsuccessfu1. We believe that the reason for faiIure in these attempts was either a Iack of persistence on the part of the physician and patient in carrying out the plan of treatment or faiIure to recognize the dif-

Rugh--Spasmodic ference in the two types of torticohi. More rapid methods of reIief or cure have also been tried, but they too have failed utterly

FIG. 7. Before treatment.

or have Ieft the patient with a loss of head and neck control fuIIy as dire as the original malady. Profiting by the markedly beneficia1 results of the compIete physioIogicaI rest to the nerve ceIIs in cases of compIeteIy or partiaIIy paraIyzed muscIes in infantiIe paralysis, through the remova of a11 stimuli from those cells by externa1 fixation of the motivated parts, we felt that if the head and neck muscIes couId be securely and firmly fixed by pIaster of Paris for a sufficient length of time, the basa1 cells supplying them would be pIaced at rest. With an opportunity to recover their tone, the ceIIs wouId Iose their hypertonicity. The cooperation of patients over varying periods of time has aIIowed us to use the method. Rest&s have proved the correctness of the theory. In one patient, a woman 47 years of age, the spasm of the muscIes was so great that an anesthetic was necessary for hoIding the head straight while the cast was being apphed. This patient developed a pressure sore on the cheek within three days, making it neces-

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Jtrurrr.11 ctISu~-gc,y

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sary to cut out part of the cast. How ‘ever, she persisted with the treatment and after two and one-half years of fixation, was

FIG. 8. Two and one-haIf years after treatment had been started.

reIieved of a11 spasm and remains we11 today (after twelve years). For the application of the cast, the patient is preferabIy seated on a stool, the hair is cut short and stockinette is sIipped over the head and we11 down on the chest, hoIes being cut for the arms, Cotton wadding is then pIaced about the body, neck and head. An assistant hoIds the head in as correct position as possibIe, the arms are heId extended IateraIIy at a right angIe and pIaster bandages are wound snugly about the body, neck and head. Reinforcing slabs are pfaced from the top of the head down the back and pressed closely to the neck. The bandage covers the entire head, but leaves the face open. When the pIaster has set firmIy, the cast is trimmed about the face and about the lower edge of the jaw, aIIowing a slight space for jaw action. The ears are then uncovered and the armhoIes carefuhy trimmed. This cast is worn for at Ieast six months and sometimes a year. In hot weather, it shouId be changed every six months, great care being used to preserve the corrected posture. The comfort foILowing the arrest

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of the spasms insures co6peration of the patient in spite of the discomfort of the weight of the cast and the odors from

FIG. g. Celluloid and metal spIint following pIaster of Paris.

the skin. After about two years, when the third or fourth cast is removed, the patient is abIe to hoId the head quite stiI1 and there is seIdom any puI1 of muscIe except in the presence of excitement. A ceIIuIoid cast is then made over a pIaster mouId and so cut that it can be removed for bathing. This must be worn continuousIy for four or five months and can then be graduaIIy removed for the appIication of a light stee1 support which provides a sense of fixation rather than its reaIity. Within a few months more, this is graduaIIy Iaid aside. In excitement or unusuaI fatigue, these patients may have a sIight sense of tension, but this quickly disappears and in none of our ten cases has there been a recurrence. In one case, a boy of 13, suffering from miId congenita1 spastic dipIegia invoIving the entire body, there was distinct torticoIIis and associated athetosis of the head, neck and arm muscIes. His activities were greatIy impaired and the uncertainty of his movements prevented him from joining in the sports of his pIaymates. In-

SEPTEMBER, 19KJ

TorticoIIis

asmuch as there stil1 remained three or four years for growth and deveIopment and as the influences of these processes often

FIG.

End result after years of treatment.

IO.

three

exert a most beneficia1 effect upon the it was decided to try spastic condition, the fixation treatment. PIaster of Paris was used (about the head and shouIders) for two years and this was foIIowed by a removabIe ceIIuIoid and meta brace. The resuIts have been beyond our expectations. The patient has gained greatly increased contro1 of his head and arms. Now when the brace is removed, he is abIe to contro1 these parts much better and they show much Iess spasticity than previousIy. In addition, the boy’s menta1 reaction to his surroundings has been greatIy improved and he is rapidIy Iosing the inferiority compIex that formerIy handicapped him. He pIays baI1 and has even joined in footbaI1 with his schooImates. AIthough there is evidence of steady improvement, it is not to be expected that the congenita1 brain changes wiI1 be compIeteIy overcome. The proIonged rest has, however, been of great vaIue and there is every reason to fee1 that further growth wiI1 promote greater improvement in his condition.

Rugh-Spasmodic SUMMARY

AND

CONCLUSION

This presentation offers three main points regarding spasmodic torticolIis : (I) the carefuI differentiation between two patentIy separate conditions which present one outstanding symptom in common; (2) the need for efficient and proIonged fixation, preferably by pIaster of Paris, until

TorticoIIis

Amerirnn

.IIIuI-~~~~01 Sttrgc

Iy

4%

spasm is overcome; (3) the necessity of absorute codperation on the part of the patient in folIowing out the treatment. REFERENCES I. GRINKER,

R.

R.

Neurology,

2nd

ed.

19x7.Thomas.

2.

Springfield,

ALPERS, B. J., and DRAYER, C. S. Am. J. M. SC., 193: 37%

‘937.

THE reflex of the tensor fasciae Iatae is unaffected in sciatic neuritis, but is usually Iost in affections of the lumbosacral roots. From-“The Injured Back and Its Treatment” by EIIis (Charles C. Thomas).