The Neurological Aspects of Deformities of the Spine

THE NEUROLOGICAL ASPECTS OF DEFORMITIES OF THE SPINE PAUL

C. Bucy,

M.D.,

F.A.C.S.,*

AND ROBERT

F.

HEIMBURGER, M.D.t

DEFORMITIES of the spine, particularly scoliosis, are not infrequently associated with disorders of the nervous system. In many instances, however, the underlying or associated neurological disease is not apparent or is overlooked. In still other instal).ces it may be suspected but be unsusceptible of absolute proof. In many of these latter situations it is necessary to apply the unsatisfactory appellation of "idiopathic scoliosis." In order that the relationship existing between these associated abnormalities of the nervous system and of the vertebral column may be understood, various aspects of this complex problem will be reviewed. It is necessary that the general practitioner, the orthopedic surgeon and the neurologist appreciate the many ramifications of the subject in order that they may deal as intelligently as possible with the orthopedic problems, and that they may not overlook any obscure neurological disorders which require independent treatment. The nature of the association of neurological disease and spinal deformities is multiplex. These relationships may be classified as follows:

1. Independent, associated congenital anomalies 2. Neurological disease secondary to spinal deformity: (a) Tuberculous or other spinal infections (b) Neoplasms of the spine (c) Paraplegia resulting directly from scoliosis 3. Spinal deformity resulting from neurological disease: (a) von Recklinghausen's neurofibromatosis (b) Intraspinal extradural cysts (c) Intraspinal neoplasms (d) Progressive muscular dystrophy (e) Syringomyelia (f) Friedreich's ataxia. (g) Anterior poliomyelitis SCOLIOSIS AND DISORDERS OF THE NERVOUS SYSTEM AS ASSOCIATED CONGENITAL ABNORMALITIES

It is not uncommon for congenital abnormalities of the nervous system to be associated with somatic malformations which are neither the result of nor the cause of the neurological involvement. Such association exists because some unfavorable influence acting upon the entire organism has arrested or interfered with the normal development of different parts of From the Department of Neurology and Neurological Surgery of The University of Illinois College of Medicine, and The Chicago Memorial Hospital, Chicago. * Professor of Neurology and Neurological Surgery, University of Illinois College of Medicine; Attending Neurosurgeon, The Chicago Memorial Hospital. t Resident Neurosurgeon, The Chicago Memorial Hospital. 163

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the body at the same time. Thus it is not uncommon to find a meningomyelocele in the lumbosacral region associated with a forked rib, a horseshoe kidney, a harelip or a hypospadias (B ucy2). Similar or other malformations of the spinal cord may also be associated with deformities of the spinal column due to hemivertebrae. In the case reported below several hemivertebrae were present, causing a scoliosis in a patient who suffered from a disturbance of the sympathetic innervation in that region. CASE I.-Excessive sweating left side of face and left upper extremity for eight years. Scoliosis secondary to hemivertebrae and spina bifida of lower cervical and upper thoracic spine. Stellate ganglionectomy with cessation of abnormal sweating. M. S., a 13 year old girl, was admitted to the University of Chicago Clinics on October 20, 1937, because of excessive sweating of the left side of the face and the left upper extremity. At the age of 5! years she had had three periods of unconsciousness without convulsions. At that time her parents first noted that the left side of her face and her left arm and hand perspired more than did the other parts of her body. This continued, until at the age of 10, after she had recovered from pneumonia, the sweating increased, without any alteration in its distribution. It was also noted that the sweating increased with excitement and that the left hand was always wet and colder than the right one. There was no marked seasonal variation in the sweating but she did not sweat as much when asleep. Because of this excessive perspiration it was almost impossible for her to keep her face clean. Dirt clung to it, and it was streaked in appearance. The excessive sweat quickly soiled her dresses and they soon rotted. Because of this annoying condition she refused to take part in any social activities and was rapidly becoming a recluse. Examination.-The left pupil was constantly larger than the right. The left side of the face and the left axilla, arm and hand all perspired freely. At times the perspiration literally ran down her face and dripped from the left side of her jaw and chin (Fig. 83). The left hand was slightly edematous and cyanotic. The skin temperature in these involved areas was 1 to 1! degrees (F.) cooler than corresponding areas on the other side of the body. Microscopic studies of the capillaries of the nail beds showed that the diameter of those on the left hand was five to six times that of those on the right. Plethysmographic records showed normal activity in the right fingers, and markedly increased activity on the left. There was a marked scoliosis of the lower cervical and upper thoracic spine with the convexity toward the left (Fig. 84). The left trapezius muscle was considerably smaller than the right, and the left grasp was slightly weaker. There were no alterations in reflexes or in sensation. The blood pressure was 110/82 on the left and 114/92 on the right. The examination was otherwise negative. Blood count was normal. Roentgenograms.-There was a marked scoliosis of the lower cervical and upper thoracic spine, resulting from a severe congenital malformation of the vertebrae in this region. The right side of the first thoracic vertebra was missing and there was a similar but less marked deficiency of the right side of the bodies of the sixth and seventh cervical and second and third thoracic vertebrae. The third thoracic vertebra articulated with two ribs on the right side. The second thoracic vertebra articulated with the second rib on the left side and with the first rib on the right side. There was a spina bifida involving the laminae of the low(lr c(lrvical vertebrae.

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Operation.-On November 4, 1937, the entire stellate sympathetic ganglion and the second thoracic ganglion were removed from the left side by a posterior approach through the bed of the second rib. Postoperative Course.-Immediately after operation the area which had previously been the site of severe hyperhidrosis was dry and warm (Fig. 85), and plethysmographic studies were almost identical in the two hands. There was a left Horner's syndrome. The patient has been seen from time to time since, the last occasion being on November 13,1947, ten years after the operation. The left side of the face and the left upper extremity were still dry. There was moderately excessive perspiration along the lower border of the sympathectomized area, i.e., in the axilla and along a line across the upper chest, both in front and in back.

Fig. 83 Fig. 84 Fig. 83 (Case I).-There is a marked hyperhidrosis of the left side of the face and neck. The scoliosis is also evident. Fig. 84 (Case I).-The spinous processes of the vertebrae have been marked with ink demonstrating the scoliosis. The Horner's syndrome was still present but was hardly noticeable on casual observation. The scoliosis was likewise still present but was almost perfectly compensated. The patient has lived a normal, active, happy life ever since the operation and is now married.

Comment.-The patient's chief complaint, localized hyperhidrosis, seems obviously to have been the result of a hyperactivity of the sympathetic nervous system in the cervicothoracic region on the left side. The fact that the skeletal system was distorted by a congenital malformation in this same region can hardly have been mere coincidence. Nor is there any reason to believe either that the deformity of the spine was responsible for the hyperactivity of the nervous system or that the

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abnormality of the nervous system had produced this congenital malformation of the spine. Rather, it seems very likely that two malformations occurred in the same region, independent of each other except that

A B Fig. 85 (Case I).-A, Preoperative. The patient has been painted with an iodine compound and then dusted with starch. The hyperhidrosis of the left side of the face, neck and upper chest and of the left upper extremity have moistened the mixture and turned the starch a dark purple. B, Postoperative. The patient has again been painted with an iodine compound and dusted with starch and then given a hot beverage and exposed to hot lamps to cause her to perspire. The former hyperhidrotic area is completely dry even with this provocation. It will be noted, however, that on the left side of the trunk below the level of sympathetic denervation perspiration is more marked than on the right side.

both were doubtless the result of the same unknown etiological factor. It is impossible to state whether the sympathetic hyperactivity was the result of a malformation of the sympathetic ganglionated chain (although

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the part removed appeared normal microscopically) or of the portions of the spinal cord related to the sympathetic system. NEUROLOGICAL DISEASE SECONDARY TO SPINAL DISORDERS

Tuberculous Spondylitis.-Neurological disturbances as the result of disease and deformity of the spine are seen most often with tuberculous spondylitis or Pott's disease. Here the spinal deformity is usually a kyphosis, seldom if ever a scoliosis. Furthermore, the dysfunction of the spinal cord which develops in some cases is only rarely the result of the spinal deformity itself. In those exceptional instances in which it is, careful inquiry usually determines that the deformity of the spinal column was suddenly and markedly increased. Such sudden alterations in the spinal deformity usually occur as the result of trauma. In those cases the spinal cord is already drawn fairly tautly across the apex of the vertebral deformity; any sudden increase in the deformity, and thus in the compression of and tension on the spinal cord, is more than the nervous tissue will tolerate, so that paralysis and sensory changes result. It is, however, rather surprising how rarely dysfunction of the spinal cord arises solely from distortion and compression resulting from the deformation of the spine. Usually the kyphosis develops slowly and gradually, and the spinal cord tolerates such distortion very well. It is not uncommon for patients whose spines are bent almost double to have no neurological abnormalities whatever. The following case is illustrative of a large group of similar patients. CASE H.-Severe deformity of the thoracic spine without any evidence of involvement of the spinal cord. Hospitalized because of left acoustic neurinoma, which was removed successfully. 1. J., a 43 year old woman, was hospitalized on November 4,1944, complaining of blurring of vision of eighteen months' duration, of headaches for six months, progressive deafness and unsteadiness of gait for five months, and numbness of the left side of the face and tongue for three months. A deformity of the back in the thoracic region had been noted in early infancy and had gradually increased in severity. Use of traction to relieve the deformity had been advised during early childhood, but this treatment was never carried out. There were never any symptoms related to the kyphosis, apart from the deformity. Examination.-On admission the patient weighed 62 pounds, compared with her usual weight of 69 pounds. She was very short, and there was marked deformity of the chest and thoracic spine resulting from a kyphosis in that region (Fig. 86), with marked prominence of the sternum and the thoracic spine bent into a hairpin curve. There were the neurological changes commonly associated with a tumor in the left cerebellopontine angle: papilledema, nystagmus, diminished left corneal reflex, left facial paralysis, and deafness and diminution in the responses to caloric tests in the left ear. Gait was unsteady, but there were no evidences of involvement of the spinal cord: sensation, muscular strength and reflex activities were all normal in the lower extremities and below the level of the spinal deformity. Roentgenograms.-X-ray examination of the spine revealed a severe kyphosis of the thoracic spine resulting from extensive destruction of the bodies of many

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of the lower thoracic vertebrae (Fig. 86). The thoracic spine and the lumbar spine formed the two arms of a hairpin curve and were almost parallel with each other. The upper thoracic and the lumbar vertebrae were markedly elongated. Near the apex of the kyphosis at the site of the destroyed vertebral bodies were numerous dense calcific deposits. An ovoid calcification was also seen in the right lumbar gutter and several calcifications were present in the hilar regions of both lungs. Operation.-On November 9, 1944, a large multilocular cystic acoustic neurinoma was removed. The patient made a satisfactory recovery from the operation and was discharged from the hospital December 8,1944.

A

B

Fig. 86 (Case II) .-A, Tuberculous spondylitis. Lateral roentgenogram of the spine demonstrating the very severe kyphosis with marked destruction of the vertebral bodies at the apex of the kyphos. Note the marked elongation of the lumbar vertebral bodies. B, Artists sketch to indicate the site and extent of the vertebral deformity.

Comment.-The kyphosis in this patient has been interpreted, both by the clinicians who examined her and by the roentgenologist, as due to a tuberculous infection of the spine. There is of course no positive proof of that etiologic diagnosis, and the possibility cannot be denied that this spinal deformity might have resulted from involvement of the bones of the spine by von Recklinghausen's neurofibromatosis, a condition which is discussed under Spinal Deformity Resulting from Neurological Disease. This latter possibility must receive particular attention in this case, in view of the discovery of an acoustic neurinoma in the left cerebellopontine angle. For the purposes of our discussion here, however, it matters little what did cause the extremely severe kyphosis. The point is that the spine can be very severely deformed, with resulting severe distortion of the spinal cord, without any evidence of neurological dysfunction.

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Tuberculous Intraspinal Granuloma.-In most cases in which Pott's disease is associated with manifestations of dysfunction of the spinal cord, the neurological disorder is the result of compression of the spinal cord by an extradural tuberculous granuloma arising from and at the level of the infection of the vertebrae. These granulomas encircle and constrict the spinal cord, giving rise to a paraplegia and loss of sensation below the level of the lesion. Because of the encircling nature of these lesions, as well as the fact that vascular changes within the spinal cord commonly result from them, laminectomy and decompression of the lesion and the spinal cord seldom gives beneficial results. Less commonly cold abscesses may appear in association with Pott's disease and compress the spinal cord. Such abscesses not infrequently communicate with even larger collections of pus in the thoracic cavity and the paravertebral tissues. Evacuation of these collections of pus often produces dramatic improvement in the manifestations of the compression of the spinal cord. Pyogenic Infections.-Pyogenic infections may also be associated with collections of pus or of granulation tissue in the spinal canal, with resulting compression of the spinal cord and paralysis of the legs. However, such disorders usually develop much more rapidly and are not commonly associated with any deformity of the spine at the time of the acute compression of the spinal cord. Later, after some of the vertebrae have been destroyed and the neurological symptoms have either -been relieved or become permanent, a deformity of the spinal column may appear. Neoplasms of the Spine.-It is not uncommon for neoplasms which arise in the vertebral bodies to produce deformities of the spine and to extend into the spinal canal, compressing the spinal cord. Myelomas and giant cell tumors of the spine are the primary neoplasms which do this most commonly; metastatic carcinomas may also do so. The spinal deformity thus produced is usually a kyphosis and the clinical picture develops fairly rapidly, making this situation of little importance either in elucidation of the pathogenesis of scoliosis or in the differentiation of "idiopathic" scoliosis from scoliosis arising as the result of neurological disease. Paraplegia Resulting Djrectly from Scoliosis.-As already noted in the discussion of the effects on the spinal cord of the kyphosis resulting from Pott's disease, deformity of the vertebral column of and by itself rarely so affects the spinal cord as to interfere with its function. There are, however, exceptions to this rule. Sachs13 reported one of the earliest cases of this type recognized in America. He found the spinal cord and dural sheath to be very much thinned out and to be compressed sharply against the apex of the spinal deformity. Incision of the dura mater and relief of the compression of the cord by the layer of dura mater lying on the convex side resulted in improvement of the patient's condition and return of his ability to walk. McKenzie10 reported a similar case in an 18 year old boy, with operation resulting in complete recovery. Recently McKenzie ll has seen and operated on still another patient with this unusual disorder. Viets and

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Clifford14 reported another such case in an 18 year old boy, whose scoliosis was the result of an attack of anterior poliomyelitis at the age of 3; the paraplegia began at the age of 17. Operation resulted in improvement and ability to walk, but some spasticity and reflex alterations still persisted. In a review of the literature they found eighteen cases in which paraplegia was thought to have been a direct result of scoliosis produced by various primary causes. They advised laminectomy and decompression of the spinal cord. Ruhlin and Albert12 reported seven cases of this kind. They noted that conservative (nonoperative) treatment did not arrest or improve the symptoms. In fact, paraplegia developed in one case while traction was being applied in an effort to correct the scoliosis. They also noted that spinal fusion did not arrest the progress of the neurological disturbance. Laminectomy was performed on five of their patients, with improvement in four. Heym~n9 reviewed the literature and found twenty-eight cases of this type, 74 per cent of them in males. Conservative treatment resulted in improvement in only 20 per cent of the cases. Ten patients were operated on, with satisfactory recovery in 60 per cent, partial recovery in 30 per cent, and one death. The operation consisted of laminectomy with incision of the dura mater so as to relieve its compression of the spinal cord. Ford6 mentions having seen several cases of this type and gives the details of one. This was a 17 year old girl who had a scoliosis, apparently resulting from neurofibromatosis. Paraplegia developed during the two weeks of application of traction to the spine in an effort to correct the scoliosis. A laminectomy produced no improvement. This case and the similar one reported by Ruhlin and Albert serve two purposes. They constitute a warning of the dangers of trying to correct a scoliosis by traction, which many orthopedic surgeons concede is without benefit, and here is definite evidence as to its danger. They also help to explain the development of paraplegia in these patients. Most persons with scoliosis go through life without neurological involvement, despite the severe distortion of the spine and of the spinal cord. Furthermore, most of them have had the spinal deformity for years without any evidence of neurological disorder. It appears that if the scoliosis develops gradually and is not seriously disturbed in a short period of time the spinal cord withstands the deformation without producing symptoms. However, the amount of leeway is doubtless slight. The spinal cord and its enveloping dura mater are drawn tautly over the vertebral prominence. If the relationships a rematerially and suddenly disturbed, as by traction in these cases or by accidental injury to the spine in some of the others, the spinal cord may not be able to withstand the added strain, its blood supply may also be interfered with, and paralysis and loss of sensation result. It should be reiterated that such cases are very uncommon among the thousands of persons with scoliosis, and that immediate laminectomy and incision of the dura mater give the greatest promise of improvement.

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SPINAL DEFORMITY RESULTING FROM NEUROLOGICAL DISEASE

Many different neurological diseases so affect the spinal column or its musculature as to result in deformity, and in many instances the neurological disorder is not readily apparent. Unless one is aware of the possibility and bears it in mind, the fundamental neurological disease is likely to be overlooked in such cases, particularly in scoliosis arising from involvement of the vertebrae by von Recklinghausen's neurofibromatosis. Scoliosis of the spine also commonly occurs with three well known disorders of the spinal cord. Syringomyelia and Friedreich's ataxia are almost invariably accompanied by this type of spinal deformity; anterior poliomyelitis results in scoliosis only when it causes an asymmetrical weakness of the spinal musculature in a young growing individual. These and other diseases of the spinal cord are discussed in the following pages, with illustrative cases. Von Recklinghausen's Neurofibromatosis.~WeissI5 and Brooks and LehmanI have pointed out that scoliosis not uncommonly occurs as a complication of this diffuse disorder of the nervous system, but we do not agree with them that scoliosis is almost always present with this disease. Neurofibromatosis is a congenital disorder characterized by pigmented areas and pendulous tumors in the skin, subcutaneous nodules and tumors of the peripheral and sympathetic nervous systems, tumors and malformations of the central nervous system and deformities of the skeletal system. The bony abnormalities are frequently cystic and have been interpreted by some observers as resulting from neurofibromas in the periosteum and within the bone itself. In any event primary destructive lesions may appear in the vertebrae, giving rise to scoliosis. In other cases Brooks and Lehman thought that the scoliosis resulted from inequalities in the length of the legs which had been produced by neurofibromatous interference with growth at the epiphyseal lines of the long bones. Among the many manifestations of von Recklinghausen's neurofibromatosis are acoustic neurinomas. For that reason, as was noted earlier, the severe kyphosis present in Case II may have been the result of this disease rather than of tuberculosis as was originally suspected. Intraspinal Extradural Cysts.~In 1937 Cloward and Bucy· described another neurological abnormality which is commonly associated with deformity of the spine~spinal extradural cysts. These are found most commonly in the thoracic region of the spinal canal, although exceptions to this have been seen 4 • They appear to be congenital outpouchings of the arachnoid membrane which penetrate through the spinal dura mater. They usually lie posteriorly to the dural sheath and are filled with cerebrospinal fluid. They communicate with the intradural space by a narrow neck which may be occluded at the time of operation or may still be patent and connect the cyst with the spinal subarachnoid space. They compress the spinal cord and give rise to a slowly developing spastic para-

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plegia. The alterations in sensation are less severe than the motor phenomena. When these cysts occur in adolescents, as they commonly do, they are often associated with destructive changes in the vertebral bodies on a level with the cyst. As a result of the softening of these bodies and the vertebral collapse, a kyphosis or kyphoscoliosis may result. This collapse of the vertebral bodies is often erroneously diagnosed as the result of tuberculosis. Years may be wasted in treating the condition by bed rest, casts and even spinal fusion, while the paralysis of the legs grows worse and the damage to the spinal cord becomes irreversible. Roentgenographically the condition bears a much closer resemblance to kyphosis dorsalis juvenilis or Scheuermann's disease than it does to Pott's disease. Usually several vertebrae are involved and as a result of the partial collapse of three or four vertebral bodies the kyphosis consists of a generally rounded curve rather the sharp gibbus usually seen with Pott's disease. It was our opinion in 1937, and we have had no reason to alter it, that the disintegration of the vertebral bodies, their collapse and the resulting deformity of the spinal column are due to the interference with venous drainage from the vertebral bodies by the intraspinal cyst. Such changes take place very slowly and therefore are only to be seen with an expanding intraspinal lesion which does not progress rapidly. Likewise it appears that such changes take place only in young individuals whose bones are still growing. As Cloward4 has shown, when theiiJe cysts occur in older people no external deformity of the spine is to be expected. In addition to the kyphosis which is commonly seen with these cysts the roentgenograms reveal other changes in the vertebrae which are of diagnostic importance. The inner surfaces of the pedicles are eroded and the spinal canal is thus widened at the level of the cyst. Furthermore, the posterior surface of the vertebral body is also eroded, making it concave and increasing the anteroposterior diameter of the spinal canal. Intraspinal Neoplasms.-Although, as noted above, kyphosis and scoliosis are not commonly associated with intraspinal lesions such as tumors which usually grow more rapidly than do the extradural cysts, there are exceptions to this rule. However, in order for intraspinal neoplasms to produce such deformities of the spine it is necessary that they meet certain rather unusual requirements. First, they must occur in young people; second, they must progress very, very slowly indeed. Exceedingly few spinal tumors fulfill those requirements. Below is reported an unusual case of this type. CASE Ill.-Scoliosis first noted in male at 17 years of age; clumsiness of hands, fibrillations of muscles of back, weakness of his legs and discomfort in his side developed very slowly. Spinal fusion at age 25. Improved until 29 years of age when weakness and loss of sensation in legs developed gradually. Barely able to walk with canes on admission, May 11, 1948. Operation revealed extensive intramedullary intraspinal oligodendroglioma.

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A. Lee., a man 31 years old, was hospitalized on May 11,1948. In 1932 he had several ribs fractured in an automobile accident. About two years later, at the age of 17, he began to have occasional twitching of the muscles of his back, and on inspection his mother noted that his back was crooked. It is not known when this deformity had its onset. About the same time he began to have occasional periods of mild clumsiness of his hands. For two years he was fairly free from symptoms. Then the fibrillations in his back returned and he noted a progressive weakness of his legs and, to a lesser estent, of his hands. His gait was peculiar and one of his shoulders was carried lower than the other. He fell frequently. A discomfort (not a pain) developed in his side, being more marked at night and becoming increasingly annoying. In 1942 an orthopedic surgeon found a scoliosis with the convexity of the curve toward the left and the left shoulder higher than the right. No neurological examination was recorded. On May 20, 1942, fusion was performed in the lower thoracic and upper lumbar region (tenth thoracic through third lumbar vertebrae). Subsequently the wound became infected and drained for four months. Thereafter he was much improved. The discomfort in his side, the fibrillations, and the weakness of his hands and legs are all said to have disappeared. In walking he favored his right leg slightly, but this was attributed to the fact that the tibial graft had been removed from that side. He continued well until about December 1946. At this time he burned his thighs against a radiator without experiencing any pain. His legs gradually grew weaker and by July 1947 he was unable to run. Before long he had to use a cane in walking, and at the time of admission in May 1948 he was barely able to walk with the help of two canes. He also had frequency and urgency of urination and occasional incontinence. Examination.-The patient was a very slender man who, with the aid of canes, dragged his spastic legs forward in an unsteady, uncertain manner. He was unable to feel pinprick below the eleventh thoracic dermatome, and thermal sensibility was lost below the second lumbar segment. Tactile sensibility was intact. There was marked weakness of both lower extremities. Flexor spasms of the legs occurred spontaneously and on stimulation. The lower abdominal reflexes were absent. The knee jerks were hyperactive. The Babinski sign was easily elicited bilaterally. On lumbar puncture the spinal fluid was clear and colorless. The initial pressure was 160 mm. of fluid. The pressure rose rapidly on jugular compression to 300 mm. but fell slowly back to the original level. The total protein in the fluid was 78 mg. per 100 cc. There were two small lymphocytes. The Wassermann test was negative, and the colloidal gold curve was normal. Examination of the blood and urine revealed no abnormality. Roentgenograms.-The scoliosis of the spine began at about the fifth thoracic vertebra and extended to about the third lumbar vertebra (Fig. 87). There was a dense spinal fusion in the thoracolumbar region, making detailed examination of the spine and of the spinal canal impossible. Operation.-On May 13, 1948, the upper part of the old incision in the lower thoracic region of the spine was reopened. The upper part of the bone graft and what was judged to be the laminae of the eighth, ninth and tenth thoracic vertebrae were removed. Although no pus was seen, the marrow within the bone graft did not appear healthy. The dura mater was incised longitudinally, exposing a markedly enlarged spinal cord which was yellowish in color. The vessels on the surface seemed compressed and all of the usual surface markings were obliterated.

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Fig. 87 (Case III).-A composite anteroposterior view of the spine, showing the scoliosis produced by a very slowly growing intramedullary tumor of the spinal cord. Roentgenograms made in 1942 prior to the spinal fusion.

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Aspiration demonstrated no cystic cavity within the spinal cord. A longitudinal incision was made through the posterior column of the spinal cord just to the left of the midline. About 1 mm. beneath the surface a pinkish-gray gelatinous tumor was exposed, which immediately partially extruded from within the cord. There was no line of demarcation between the tumor and normal nervous tissue. Considerable tumor was removed from within the spinal cord, but by no means all of it, and it did not seem worth while to attempt to expose either the upper or lower limit of the tumor. The dura mater was left open, and the other soft tissues were closed in layers as usual. Histologic Examination.-The tumor tissue microscopically was revealed to be that of a typical oligodendroglioma. There were no mitotic figures or other evidences of malignancy. The tissue was relatively cellular, and each cell was

Fig. 88 (Case III).-Microphotograph of the oligodendroglioma of the spinal cord. Each cell is enclosed in a boxlike structure giving the appearance of a cross section of a plant. The walls of the blood vessels are thickened. enclosed in a definite boxlike framework typical of this type of tumor (Fig. 88). The walls of many of the blood vessels were thickened, and some were practically occluded by this intravascular proliferation. Postoperative Course.-Since the operation the paraplegia has been complete. The sensory level descended to about the first lumbar dermatome and has remained there. About a week after the operation a rather indolent infection developed in the wound and a staphylococcic meningitis appeared. Presumably this represented a lighting up of the infection which had been present for several months after the operation in 1942; in any event, it subsided promptly with the administration of antibiotics. The patient is now receiving physical rehabilitation.

Comment.-Obviously this is an unusual situation which could only exist with a very slowly growing intraspinal tumor, such as an oligoden-

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droglioma, in a young individual. Oligodendrogliomas of the spinal cord are very rare 'under any circumstance. (This subject will be reviewed and presented in detail in a separate paper with Russell.) It seems probable that scoliosis can develop only when the related spinal musculature is affected in a growing individual. Obviously destruction of bone can give rise to spinal deformity at any age, but such deformities usually take the form of a kyphosis. Furthermore, primary intraspinal neoplasms rarely invade and destroy the vertebrae. Occasionally another type of intraspinal neoplasm occurring in young people will so affect the vertebrae and the vertebral musculature as to produce another type of vertebral deformity-a lumbar lordosis. Such a case is reported below. CASE IV.-Girl, at age of 8, had deformity of back, with difficulty in walking, and was treated for Pott's disease. At age 11, pain developed in back and legs, and impairment of sphincters. X-ray pictures revealed enlargement of lumbar spinal canal. Operation, partial removal of ependymoma. Death later from urinary infection. H. S., a girl of 12, entered the University of Chicago Clinics in January, 1932· In 1928, when she was 8, her family had noted a deformity of her spine. At about the same time she had some difficulty in walking and a weakness of her left ankle. In 1929, a diagnosis of Pott's disease was made and she was treated in a body cast. There was little change in her condition until September, 1931, when she began to complain of pain in her back and legs and of difficulty in controlling the sphincters of her bowel and bladder. Her family thought that the spinal deformity was increasing. Examination.-There was an unusual prominence of the lower thoracic spine which was originally interpreted as a kyphosis. Study of the roentgenograms, however, revealed that the deformity was the result of a lordosis of the lumbar spine which thrust the thoracic spine backward. There was also a prominence of the sacrococcygeal spine owing to the excessive curvature of the lumbar spine (Fig. 89). There was a moderate scoliosis. Roentgenograms also revealed a dilatation of the spinal canal in the lumbar region, with almost complete erosion of the pedicles of the second and third lumbar vertebrae (Fig. 90, A). The posterior surfaces of the vertebral bodies in this region were also eroded, making them concave (Fig. 90, B). The laminae and pedicles were very thin. There was a complete loss of sensation in the third, fourth and fifth sacral dermatomes, with a partial loss from the fifth lumbar to the second sacral. The dorsiflexors of the left foot and toes were completely paralyzed. There was considerable weakness of the ventral flexors of the trunk, causing the patient to throw the upper part of her body backward to avoid falling forward. This is the same situation which produces an exaggerated lordosis in patients with progressive muscular dystrophy. Knee and ankle jerks were absent. The bladder was grossly distended, and abdominal compression caused urine to dribble from the urethra. Lumbar puncture at various interspaces yielded only blood, except for one small piece of yellowish tissue which on microscopic examination had the structure of an ependymoma. The usual examinations of blood and urine were negative. Operation.-On January 6, 1932, laminectomy was performed from the ninth thoracic to the first sacral vertebra, inclusive. The laminae were very thin and in

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places completely eroded through. When the laminae were cut through the ends sprung apart about 1 cm., as if under great tension. Within the dural sheath was a friable, pinkish-gray neoplastic mass which pushed the roots of the cauda equina to either side of the spinal canal. The tumor was removed as completely as possible. Examination of it confirmed the diagnosis of an ependymoma. Postoperative Course.-Twenty-three x-ray treatments of 275 r each were given to the thoracolumbar spine. The lordosis gradually increased. Pyuria developed in October, 1932, and continued with vari:1ble severity thereafter.

Fig. 89 (Case IV).-Ependymoma in the lumbar spinal canal. The marked lordosis and slight scoliosis are obvious.

She had repeated severe epistaxis, a rising blood pressure and severe headaches. In December, 1934, she was again hospitalized because of headaches, dizziness and vomiting, resulting from renal insufficiency, and died on January 4, 1935. Necropsy.-There was a large intraspinal tumor from the twelfth thoracic vertebra to the first sacral. There was also tumor within the lower part of the spinal cord between the posterior horns. The kidneys were small, with extensive evidence of degeneration and hemorrhage.

Comment.-This uncommon type of malformation of the spine is characteristic of an extensive lesion in the lower spinal canal of a child, which weakens the vertebral column by erosion and the paravertebral

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muscles by paralysis. The clinical and roentgenologic pictures are not those of a tuberculous spondylitis, and yet the prevalence of that condition as a cause of spinal deformity has been so impressed upon the profession that the erroneous diagnosis was made in this case, as it was in the cases of intraspinal extradural cyst reported by Cloward and Bucy5. Scoliosis of the spine may occasionally develop in a child after successful removal of a spinal cord tumor, as the result of a permanent weakness

B A Fig. 90 (Case IV).-Ependymoma of the lumbar spinal canal. The canal is markedly enlarged both anteroposteriorly and laterally. The pedicles and laminae are displaced and eroded. The posterior surfaces of the vertebral bodies are eroded giving them a concave appearance.

of the musculature of one side of the spine. The following case, reported originally in 1935 by Buey and Buehanan 3 and again by Heimburger and Bucy 8 in a study of the regeneration of the bony structure of the vertebrae after the removal of pressure, is a typical example of this type. CASE V.-Male, with weakness of left leg developed at 1 year of age, followed by atrophy and finally paraplegia. Roentgenograms revealed marked dilatation of lumbar spinal canal. Cystic teratoma removed from spinal canal at age 3, followed by complete recovery, except for mild atrophy of left lower extremity and slight scoliosis. R. B., 2 year old boy, was first admitted to the pediatric department of the University of Chicago Clinics on June 8,1933. In October, 1932, he had suddenly

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become irritable and a stiff neck developed. There was no fever, and the condition gradually subsided over a period of six weeks. However, during this time the left leg became weak and atrophic, although he was still able to walk. A diagnosis of anterior poliomyelitis was made. On March 19, 1934, he was again admitted to the University of Chicago Clinics. In the interval he had complained of severe pain on movement of his legs and walking had grown progressively more difficult. Examination.-There were atrophy and weakness of both lower extremities, and he was unable to stand. The left knee jerk was absent, the left ankle and right knee jerks were about normal, the right ankle jerk was hyperactive and sustained clonus could be elicited. There was no demonstrable sensory loss, but the patient was only 3 and not very cooperative. Roentgenograms.-The spinal canal was grossly dilated from the twelfth thoracic to the third lumbar vertebra. There was marked bony erosion but no scoliosis. Operation.-On April 10, 1934, laminectomy was performed and a large teratomatous cyst which extended from the twelfth thoracic to the fourth lumbar vertebra was removed from the spinal canal. Postoperative Course.-His condition steadily improved after operation. In the ensuing 14! years he has grown into a large, healthy, active boy. He has actively engaged in various school sports. Examination has been performed from time to time; on July 19, 1948, he was a well developed, muscular boy who appeared in excellent health, and had no complaints. The entire left leg was definitely smaller than the right, and there was a definite but not prominent scoliosis of the lower spine. Roentgenograms taken since the operation have disclosed a remarkable recalcification of the vertebrae. 8 Films taken on December 8, 1939, five and one half years after operation, showed no scoliosis, but those taken on November 7, 1947 (Fig. 91), and on July 19, 1948, showed a distinct but slight scoliosis of the lumbar spine. Comment.-Apart from the interest associated with this unusual type of intraspinal neoplasm, the gratifying recovery and the striking regeneration of the eroded and decalcified pedicles and vertebral bodies, this case is of distinct interest in a study of the subject of scoliosis. There is no bony abnormality here to account for the spinal deformity. There is on the other hand a definite asymmetrical muscular weakness and atrophy which is most distinct in the left lower extremity but which in all probability involves the lower spinal musculature as well. This is doubtless the result of some permanent damage to the anterior horn cells of the lumbosacral spinal cord on the left side, or to the motor roots arising there. Not only does this case illustrate the development of scoliosis from an asymmetrical muscular weakness in a young growing individual, as has been stressed elsewhere, but it also illustrates the long period of time required for such a development (over five years in this case), as has been previously noted by other observers in instances where scoliosis has resulted from the paralysis produced by anterior poliomyelitis. Progressive Muscular Dystrophy.-As the result of weakness of the muscles of the trunk, particularly those of the back which hold the vertebral column erect, progressive muscular dystrophy is commonly asso-

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ciated with a lordosis of the spine not unlike that which occurred in Case IV as the result of paralysis of the paravertebral muscles. This type of spinal deformity occurs particularly with the Landouzy-Dejerine or facio-scapulo-humeral type of dystrophy but may appear with any form in which the paravertebral muscles are involved. The spinal deformity in these cases appears as an overcompensation for the weakness of the muscles of the back which are unable to hold the spine extended and to prevent its flexion. Therefore, in order to remain upright the patient

Fig. 91 (Case V).-Roentgenograph of the lumbar spine taken thirteen and one-half years after the removal of a teratoma from within the spinal canal. In these intervening years a mild scoliosis has developed.

must throw the upper part of the body farther back than normal and displace the center of gravity backward so that the weight of the upper part of the body cannot flex the spine and cause the patient to fall forward. Syringomyelia.-Syringomyelia is typically a disease of the cervical and upper thoracic spinal cord. It is a progressive disorder characterized by a cystic condition within the substance of the spinal cord. This cystic cavity and the scarred (gliotic) area about it gradually extend, involving various long fiber tracts of the spinal cord, notably the anterolateral spinothalamic tracts and descending motor pathways in the lateral columns of the spinal cord, and the gray matter of the anterior and posterior

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horns. Involvement of the pyramidal tracts and of the extrapyramidal tracts which descend with them in the lateral columns of the spinal cord results in a spastic paralysis below the level of the lesion. This disturbance is most evident in the lower extremities and produces a spastic paraplegia. It is doubtful if this spastic paralysis plays much of a role in the development of the scoliosis. The destruction of the anterior gray horns of the spinal cord results in an atrophic, flaccid paralysis of the muscles innervated by the segment of the spinal cord in which the lesion is located. This flaccid paralysis includes the paravertebral musculature and it is likely that it is this which produces the scoliosis. It is interesting that the scoliosis is often the first symptom of which the patient or members of his family are conscious in this gradually progressive disease. The following case represents a typical example of this group. CASE Vr.-Male, noted scoliosis at about 11,. years of age. At age 23 years began to limp, and difficulty in walking has steadily increased since. At age 30 numbness and weakness of right hand appeared and the left was involved later. Weakness of external rectus muscle of the left eye, and numbness of the right side of the face also developed. B. G., a 33 year old man, was hospitalized on June 16, 1948, having been under observation for several years prior to that time. He first became aware of a curvature of his spine when about 14 years old. This deformity had increased so slowly thereafter as to be practically imperceptible. He noted no other disability until he was about 23, when he began to limp on his right leg. This grew progressively worse and three years later he was forced to use a cane; at 30 years of age he first began to use two crutches, assistance he has required ever since. At this time also he noted a numbness and weakness of his right hand which grew steadily worse, and later a similar disability appeared on the left. For several months prior to admission he had had to use a rubber stamp to sign his name. Also at age 30 diplopia developed, but although the muscular weakness persists he has overcome the diplopia. Since the age of 32 he has noted numbness of the right side of his face. There has been no difficulty with the control of his bowel or bladder, and no impairment of sexual function. He continued to work daily at a desk job until the day of his hospitalization. Examination.-The patient, a rather obese man, had a marked S-shaped thoracolumbar kyphoscoliosis, with the thoracic concavity toward the right and the lumbar concavity toward the left. He was able, with the aid of crutches, to drag his legs in a slow four point gait. He was unable to rise from a prone to a sitting position unaided but was able to maintain a sitting position once it was established. There was complete paralysis of the external rectus muscle of the left eye, and there was a marked nystagmus on looking toward either side. There was diminished pain sensibility over the second and third divisions of the right trigeminal nerve. There were weakness and atrophy of the left half of the tongue and fibrillations bilaterally. Both trapezius muscles were atrophic and weak. No contraction of the intercostal muscles could be detected at any time. The hands were constantly in a flexed position, and there were marked weakness and atrophy of the intrinsic muscles, especially on the right side. The muscles of the arms, except for the right biceps, were practically without strength. There were marked flexor defense reactions in the right leg and practically no voluntary power. All muscles in the left lower extremity functioned but were quite weak.

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In the upper extremities the only reflex which could be elicited was a Hoffmann reflex on the right. In the lower extremities all tendon reflexes were hyperactive; clonus was readily elicited at both the knee and ankle, and Babinski's sign was present bilaterally. Pain sensibility was lost over the second cervical to the second thoracic dermatomes bilaterally and was diminished over the remainder of the trunk and the lower extremities. Thermal sensibility was diminished below the third cervical dermatome on the right side but was intact elsewhere. There was no loss of tactile sensibility. The usual laboratory examinations of the blood and urine showed no abnormality. Examination of the spinal fluid revealed an initial pressure of 180 mm. of fluid with free dynamics, and a final pressure of 135 mm. of fluid. The spinal fluid total protein was 40 mg. per 100 cc. The Wassermann test was negative and the colloidal gold curve was normal. Roentgenograms.-X-ray examination of the skull and spine revealed no evidence of platybasia, no abnormality of the spinal canal and no evidence of any destruction or malformation of the vertebrae other than an S-shaped scoliosis (Fig. 92). Diagnosis.-The condition seemed to be a typical case of syringomyelia. Operation.-Because Dr. W. A. Gustafson had operated on approximately fifteen patients with syringomyelia, finding a platybasia or an Arnold-Chiari malformation (Bucy and Lichtenstein) or both in a very high percentage of these, and had obtained relief in a considerable number of cases by decompressing the abnormal area, such an operation was proposed to the patient, who readily accepted the idea as his condition was obviously growing rapidly worse, he was almost completely incapacitated and no other form of therapy offered any improvement. (X-ray therapy had been tried without benefit.) On June 17, 1948, the laminae of the first and second cervical vertebrae and a small piece of skull in the midline of the lower part of the occipital bone, including the posterior arch of the foramen magnum, were removed. There was a dense band of fibrous tissue formed by a thickening of the dura mater just at the foramen magnum. When the dura mater was incised it was densely adherent to the arachnoid membrane, which was opaque and considerably thickened. The arachnoid was incised and freed from the upper cervical portion of the spinal cord, but at the level of the foramen magnum, over the lower part of the medulla oblongata, it was so thick and so densely adherent to the brain stem and the posteroinferior cerebellar arteries that it seemed unwise to try to free it at that level. Above that point it was again incised and freed, exposing the lower end of the fourth ventricle and the medulla oblongata at the obex and just below it. Although the fourth ventricle appeared to be a little farther caudad than usual, there was no herination of the cerebellum into the spinal canal. As far as could be seen, within the limits imposed by the leptomeningeal thickening, the medulla oblongata and the spinal cord were grossly normal. The dura mater was not closed. Postoperative Course.-An indwelling catheter was required for twenty days after the operation, but bladder control was then recovered. For a week after the operation the patient had fever, with nausea and vomiting, and was quite depressed, apparently as the result of blood in the subarachnoid spaces. He is now again up and about on his crutches but, unfortunately, his condition at the time of this report appears to be about the same as it was before the operation.

Comment.-Although the pathologic process revealed at operation in this case was by no means typical of syringomyelia, the clinical picture

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Fig. 92 (Case VI).-Syringomyelia. A composite roentgenogram of the entire spinal column. The scoliosis is obvious.

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was. The latter included early onset (syringomyelia appearing during adolescence), evidence of a flaccid, atrophic paralysis of the upper extremities, spastic paralysis of the legs, and typical sensory changes. In addition, there was definite evidence of a syringobulbia. The involvement of the spinal cord was, as usual, asymmetrical, and it is reasonable to assume that the paralysis of the spinal musculature was likewise asymmetrical. Such an unbalanced paralysis of the spinal musculature in a growing boy resulted here, as it usually does, in a scoliosis. Friedreich's Ataxia.-This familial and hereditary degenerative disorder of the central nervous system is commonly associated with scoliosis. The degeneration is exceptionally widespread, involving predominantly the long fiber tracts in the posterior half of the spinal cord. Degeneration of the anterior horn cells, although it may occur, is not typical. Nystagmus, dysarthria, facial grimacing and optic atrophy all give evidence of involvement of the central nervous system above the level of the spinal cord. The disease begins early in life, usually between 5 and 15 years of age. The exact neurological deficiency which is responsible for the scoliosis is not clear. There can, however, be little question but that the deformity of the spine is the result of the neurological condition as there is no evidence of any primary involvement of the vertebral column. Anterior Poliomyelitis.-It is a well established fact that scoliosis is a not uncommon sequel to acute anterior poliomyelitis. It would be impossible to state how frequently scoliosis follows this acute infectious disease of the spinal cord, and no study of any series of cases would give a valid answer to this question. The extent and location of the muscular involvement which occurs with acute anterior poliomyelitis varies too widely from epidemic to epidemic, from year to year and from place to place to make any but an all-inclusive study over a long period of time reliable. Even such a study would be meaningless in predicting the outcome in any single epidemic of the disease. However, certain facts regarding the development of scoliosis following an attack of this disease are well established. The deformity of the spine results from an asymmetrical paralysis of the spinal musculature, producing an unequal pull upon the spine which over years of time results in a spinal curvature, if the subject is a young, growing individual. Forkas7 , in his study of this condition, demonstrated that the scoliosis does not appear until four or five years after the acute infection, although the muscular paralysis which is responsible for the scoliosis and the changes in the spinal structures which precede it can be demonstrated shortly after after the acute episode. It is also significant that the paralysis which follows the spinal infection need not be marked or extensive. In fact there may be little or no paralysis demonstrable in the extremities. The following case, reported through the courtesy of Dr. E. L. Compere, is a typical example of the severe scoliosis which may occur with this condition.

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CA·SE VII.-Girl, acute anterior poliomyelitis at age 3! years. Weakness of muscles of trunk and of both lower extremities. Scoliosis first apparent at age 7. Back brace applied at age 8. Scoliosis progressed. Spinal fusion at 16. A. C., a 16 year old girl, was hospitalized June 8, 1948. An acute attack of anterior poliomyelitis at the age of 3! years had left a weakness of all of the muscle groups of the left leg, a right foot drop, and weakness of the musculature of the lower trunk, but no involvement of the upper extremities. When she was 6 years old, operations were performed on both femurs because of malalignment of the legs. W hen she was 7 years old, her mother first noted a curvature of her

Fig. 93 (Case VII).-Anterior poliomyelitis at the age of 3! years was responsible for this scoliosis in this 16 year old girl.

spine, which grew progressively worse. When she was 8 a back brace was applied and she was given a brace for her left leg and a smaller brace to correct the foot drop on the right. She has since been able to get about with the aid of these braces and crutches. However, the scoliosis steadily grew more severe and she was hospitalized for the performance of spinal fusion. Examination of the blood and urine was negative. Examination.-There was an S-shaped spinal curvature with the thoracic portion curved toward the right and the lumbar toward the left. There were also a lordosis and a marked tilting and rotation of the pelvis. The right lower extremity was considerably stronger than the left. The quadriceps extensor muscle was almost normal. All other muscles, although weak, had some power, except

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for the anterior tibial group. On the left there was no voluntary control of the muscles of the leg except for internal and external rotation of the foot. The abdominal muscles seemed strong but there was definite weakness of the musculature of the back. Roentgenograms.-There was marked scoliosis of the entire spine (Fig. 93), but no evidence of any bony destruction or of any abnormality of the spinal canal.

Comment.-Discussion of the operations performed on this patient during June and July, 1948, is not pertinent to this report, which is given solely to illustrate the late appearance of scoliosis in a patient who has had anterior poliomyelitis. DISCUSSION

It is obvious from this presentation that deformities of the spine, notably scoliosis, rarely give rise to any disorder of the spinal cord but frequently occur either in association with or as the direct result of diseases of the nervous system. It may be well, therefore, to summarize some of the characteristics of that etiological relationship. In order for a neurological disorder to give rise to scoliosis 1. The innervation of the spinal musculature must be affected. 2. The involvement must be asymmetrical. 3. The paralysis of the musculature must be either stationary or only slowly progressive. A rapidly progressive neurological lesion or One of only short duration does not provide sufficient opportunity for the scoliosis to develop as the mechanics of the skeleton alone are sufficient to prevent the development of the deformity in a short period. 4. The disorder must occur in a young, growing individual. It would seem that Once the spine is fully formed and is nO longer growing and changing it cannot be deformed by unequal muscular pull. In this Connection it is noteworthy that the scoliosis does not appear until four or five years after the muscular paralysis develops. Two questions must be answered for the illness to be completely understood, from a neurological standpoint, and for the patient with scoliosis to be treated most intelligently. First, does the patient have some associated or related neurological disease which is in need of independent treatment, apart from the scoliosis? Some of the outstanding examples of this are tumor of the spinal cord, syringomyelia and intraspinal extradural cyst (in the case of kyphosis). Second, is the patient's orthopedic condition explained by and better understood because of the demonstration of a causative neurological mechanism? Although we do not feel sufficiently Conversant with the orthopedic problems of "idiopathic scoliosis" to enter into the controversy as to whether this condition is the result of an undetected anterior poliomyelitis, it is only fair to state that from a neurological standpoint there is nothing to controvert such a possibility.

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REFERENCES 1. Brooks, Barney and Lehman, E. P.: Bone Changes in Recklinghausen's Neurofibromatosis. Surg., Gynec. & Obst. 38:587, 1924. 2. Bucy, Paul C.: Spina Bifida and Associated Malformations. Brenneman's Practice of Pediatrics. Hagerstown, Md., W. F. Prior & Co., 1948. Vol. 4, Chap.15. 3. Bucy, Paul C. and Buchanan, Douglas: Teratoma of the Spinal Cord. Surg., Gynec. & Obst. 60:1137,1935. 3a. Bucy, Paul C. and Lichtenstein, Ben W.: Arnold-Chiari Deformity in an Adult without Obvious Cause. J. Neurosurg. 2:245, 1945. 4. Cloward, Ralph B.: Spinal Extradural Cysts. Ann. Surg. 105.'401, 1937. 5. Cloward, Ralph B. and Bucy, Paul C.: Spinal Extradural Cysts and Kyphosis Dorsalis Juvenalis. Am. J. Roentgenol. 38:681, 1937. 6. Ford, Frank R.: Diseases of the Nervous System in Infancy, Childhood and Adolescence. Springfield, Ill., Charles C. Thomas, Publisher, 1944. 7. Forkas, Aladar: Paralytic Scoliosis. J. Bone & Joint Surg. 25:581, 1943. 8. Heimburger, R. F., Bucy, Paul C. and Sammet, Joel F.: Regeneration of Bone Following the Relief of Pressure from Expanding Intracranial and Intraspinal Lesions. To be published. 9. Heyman, Clarence: Spinal Cord Compression Associated with Scoliosis. J. Bone & Joint Surg. 19.'1084, 1937. 10. McKenzie, Kenneth: Paraplegia Associated with Congenital Scoliosis. Arch. Surg. 15:222, 1927. 11. McKenzie, Kenneth: Personal communication . . 12. Ruhlin, Carl W. and Albert, Seymour: Scoliosis Complicated by Spinal Cord Involvement. J. Bone & Joint Surg. 23.'877,1941. 12a. Russell, John R. and Bucy, Paul C.: Oligodendroglioma of the Spinal Cord; Report of a Case. In press. 13. Sachs, Ernest: An Unusual Case of Paraplegia Associated with Marked Gibbus, and a Localized Collection of Fat at the Site of the Gibbus. J. Bone & Joint Surg. 23:709, 1925. 14. Viets, Henry R. and Clifford, Milton H.: Paraplegia Associated with NonTuberculous Kyphoscoliosis: A Case Report and Survey of the Literature. New England J. Med. 200:55, 1932. 15. Weiss, Richard S.: Von Recklinghausen's Disease in the Negro; Curvature of the Spine in Von Recklinghausen's Disease. Arch. Dermat. & Syph. 3.'144, 1921.