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Spontaneous dislocation of the atlanto-axial articulation occurring in ankylosing spondylitis and rheumatoid arthritis
4°
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
Spontaneous Dislocation of the Atlanto-axial Articulation occurring in Ankylosing Spondylitis and Rheumatoid Arthritis T. L. C. PRATT, M.R.C.S., L.R.C.P., D.M.R.D.
Halifax and Huddersfield Hospital Groups -SEVERAL cases of atlanto-axial dislocation due to acute cervical and nasopharyngeal inflammation have been described. Bell first recorded the condition, due to pharyngeal ulceration, in 183o. Watson Jones (1932) describes a dramatic cause of sudden death in a girl who dislocated her atlas while a nurse was dressing an infected sinus of her neck. Atlanto-axial dislocation due to ankylosing spondylitis and rheumatoid arthritis has rarely been
June I5, I95o. Spontaneous atlanto-axlal dislocatlon. Cause not apparent.
Fig, I . - - C a s e I.
recorded. Kornblum, Clayton, and Nash (I952) describe 2 cases, and 2 further cases where the subluxation occurred at a lower level in the cervical spine. Rand (1944) reported a patient with a Brown-Sequard syndrome due to atlanto-axial dislocation who also had ankylosing spondylitis, although association of the two conditions was not recognized. Stammers and Frazer (1933) describe spontaneous atlanto-axial dislocation in a man with a clinical history suggesting ankylosing spondylitis. Margulies, Katz, and Rosenberg (1955) report one case and include the histology of tissue obtained from the upper cervical region. W e m e (1957) describes atlanto-axial dislocation in a girl aged 4 who was suffering from rheumatoid arthritis. He discovered 3 more cases attending the University
of Lund, and 3 fresh cases were diagnosed at the Orthopmdic Policlinic over the following three years. He remarks on delay in diagnosis because of either ignorance of the condition or difficulty in radiographic interpretation. One case of ankylosing spondylitis with atlanto-axial dislocation is described by Voluter and Werner (1956). At a meeting of the British Orthopaedic Association, Purser and Sharp (1957) stated that they had
Fig. 2.--Case I. May 7, I957. Stable atlanto-axial dlslocation.
Advanced ankylosing spondyhtis
seen 17 cases of ankylosing spondylitis and 23 cases of rheumatoid arthritis with atlanto-axial dislocation and they comment upon the scarcity of recorded cases in the literature. T h e aetiology of spontaneous atlanto-axial dislocation is the same whether due to acute or chronic infection. Hyper~emia accompanying cervical infection results in decalcification of the atlas which may be sufficient to allow detachment of the transverse ligament. Finally, the atlas glides forward over the superior articular surfaces of the axis with consequent flexion of the head. Berkheister arid Seidler (1931) describe a sudden atlanto-axial dislocation due to transient effusion into the atlanto-axial joints. This incident occurred
ATLANTO-AXIAL d u r i n g a h y p e r s e n s i t i v i t y reaction to p e r t u s s i s vaccine. Wittek (I9o8) also indicated t h a t an effusion due to metastatic infection d i s t e n d e d the small capsules of the atlanto-axial joints, t h e r e b y relaxing the accessory ligaments. T h i s process has therefore b e e n suggested as a n o t h e r factor p r e c e d i n g atlanto-axial dislocation. Histology of resected tissue f r o m the case quoted b y Margulies and others s h o w e d " r e p l a c e m e n t of b o n e - m a r r o w b y fibro-collagenous tissue containing
DISLOCATION
moved either for months and attributed her complaint to teeth extractions twelve months previously. A radiograph showed anterior subluxation of atlas upon axis ( F i g . i ) . E.S.R. 20 mm. per hour. June x6. A plaster cast was applied and replaced by further casts during the following months in order to try to extend the head gradually. Jan. 8, I95I. Plaster cast removed. No tenderness on palpation of cervical spine. The atlanto-axial dislocation was considered stable.
o._
Fig.
2. July 16, I956. Spontaneous atlanto-axial dislocation with ankylosingspondylitis.
3.--Case
dense infiltrate of p o l y m o r p h o n u c l e a r leucocytes, lymphocytes, occasional p l a s m a cells and m o n o c y t e s " . T h e r h e u m a t o i d condition t h u s involves extra-articular b o n e as well as synovial tissue and o t h e r articular soft tissues. F r o m these pathological processes, it is seen that atlanto-axial dislocation m u s t occur d u r i n g an active p h a s e of the disease. A c u t e n e i g h b o u r i n g infections cause s u d d e n atlanto-axial dislocations w i t h possible neurological signs due to p r e s s u r e o f the o d o n t o i d process of the axis and posterior arch of the atlas on the spinal cord. S u d d e n d e a t h m a y occur. T h e dislocation complicating c h r o n i c infection is a gradual process and K o r n b l u m and others suggest that serious spinal cord damage is rare for this reason. It is remarkable that the spinal cord escaped p e r m a n e n t damage in the severe degrees of atlantoaxial dislocation seen in the following cases.
CASE REPORTS I . - - J u n e i5, 195o. A woman aged 38 complained of pain in the neck and right arm. Her neck was rigid and her head was flexed. She stated that she had not Case
41
W-
"- "
.
.......
V
o
"
:
" -~
F i g . 4 . - - C a s e 2. Dec. 7, 1957. Bone-graftinghas produced a strong bony bridge between C2 and external OCClpltal protuberance.
Jan. 24. Patient complained of dysphagia. Enlarged tonsils and posture thought to be the cause. May 9. Radiographic appearance of cervical spine on review showed early loss of definition in posterior intervertebral joints. June 2o. Examination revealed a small thyroid goitre and there was tremor of the hands. She was treated with thiouracil which controlled the thyrotoxic symptoms and relieved her dysphagia. Dec. i2. Cervical spine again tender. Jan. 5, 1952. Patient four months pregnant. Because of the atlanto-axial dislocation, the pregnancy was terminated and hysterectomy performed. Aug. 7. Patient complained of stiffness of lower lumbar spine. Radiographs showed active ankylosing spondylitis affecting the whote of the spine and the sacro-iliac joints. Aug. 24, 1955. Patient complained of pain in right sacro-iliac joint of two months' duration. Her back was very stiff and the cervical spine was stiff but not tender. A course of deep X-ray therapy relieved her symptoms and since then she has attended hospital on several occasions for physiotherapy. May 7, 1957. Radiographs showed advanced ankylosing spondylitis affecting the posterior articulations of the cervical vertebree and ossification of the longitudinal ligaments. The atlas was still dislocated anteriorly ( F i g . 2).
42
JOURNAL
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Case 2.--April IO, 1946. A man aged 19 complained of pain in the lower back and legs. Aug. 21, 1952. He again attended hospital complaining of pain over the chest and shoulders. Radiographs showed cervical ribs but no evidence of disease in chest or shoulders. E.S.R. normal. Dec. 15, 1954. Twelve months' history of stiffness of spine which had been progressive and spread to neck. Chest expansion was t in. Forward bending was still
OF
RADIOLOGISTS
May 13. Radiographs showed bony consolidation ot bridge between C2 and occiput. Dec. 7. Radiographs showed a good bony bridge between C2 and oceiput. The patient had dispensed with his neck brace and had returned to work as a clerk (Fig. 4). Case 3.--Oct. 9, 1956. A woman aged 56, suffering from rheumatoid arthritis, was admitted to hospital complaining of pain in knees, ankles, and back, and tingling sensations in her hands. She was obese and unable to walk. Her hands, knees, and feet were deformed due to rheumatoid arthritis. Hydrocortisone treatment was started on the following day: IOO mg. for 4 days, followed by 75 mg. for 3 days, and 50 mg. daily until Jan. 2i, 1957. Dec. 18. She complained of weakness of both hands. B.S.R., 36 mm. in one hour. Dee. 29. Radiographs showed "osteophytosis of C2, 3, 4, 5, and 6 with diminished disc spaces. There is anterior displacement of the atlas upon the axis which suggests dissolution of the transverse ligament. This can occur in rheumatoid arthritis". (Fig. 5.) Neurological examination showed no sensory loss in the limbs. The plantar responses were extensor. A cervical collar was applied. Jan. 20, 1057. Marked weakness in arms and hands. Sensations normal, reflexes normal. Plantar responses extensor. Jan. 23. Increasing quadriplegia secondary to dislocation of atlas. Neck traction applied with improvement in strength of arms and legs. March 2o. Atlanto-axial dislocation considered stable. A spinal support with neck piece was ordered. During the following months the patient developed melancholia. She lost weight and occult blood was discovered in her stools. The possibility of neoplasm in the alimentary tract was considered but her condition was too .poor for this to be investigated by radiographic examination. She became incontinent of urine and developed a small pressure sore on the left buttock. Her general condition gradually deteriorated and she died on Oct. 29.
COMMENTARY
Fig. 5.--Case 3. Dee. 29, 1956. Spontaneous atlanto-axial dislocation. Rheumatoidarthritis for twenty years.
possible to a small extent. His general posture was typical of ankylosing spondylitis. Dec. 20. Radiographs showed early changes in the sacro-iliac joints and posterior articulations of the lower lumbar and cervical spine due to ankylosing spondylitis. Jan. 4, 1955. Pain in neck and stiffness. E.S.R. normal. Deep X-ray therapy was given to neck and sacro-iliae joints, with relief of pain and improvement of mobility. May 25. Pain and increasing stiffness in cervical spine was treated by physiotherapy with some relief of symptoms. July 16, 1956. The patient complained that while standing, he felt a sudden 'click' in his neck followed by flexion of his head. This frightening experience occurred on several occasions and was due to atlanto-axial dislocation. When supine the dislocation reduced itself. Radiographs showed anterior dislocation of the atlas upon the axis. Loss of definition in posterior intervertebral joints in upper cervical spine was due to ankylosing spondylitis (Pig. 3). July 17. Plaster jacket and collar were applied. Feb. 15, I957. Bone-chip grafts from right posterior iliac crest were inserted between spinous process of C2 and external occipital protuberance of occiput and the two latter were wired. April 27. Neck brace fitted.
T h e s e t h r e e cases illustrate the i m p o r t a n c e of r e m e m b e r i n g that ankylosing spondylitis and r h e u m a toid arthritis can cause atlanto-axial dislocation. I n Case i the reason for the atlanto-axial dislocation was n o t a p p a r e n t at first, although infection f r o m the t e e t h p r i o r to extraction was considered possible. T h e first c o m p l a i n t of pain in the back occurred two years later and radiographs c o n f i r m e d the diagnosis of ankylosing spondylitis. I f radiological examination of the whole spine and the sacroiliac joints h a d b e e n m a d e w h e n the p a t i e n t first a t t e n d e d hospital, the diagnosis would p r o b a b l y have b e e n a p p a r e n t at that t i m e in spite of the lack of relevant signs and s y m p t o m s . I n any case of s p o n t a n e o u s atlanto-axial dislocation w i t h no a p p a r e n t clinical cause, the whole spine and the sacro-iliac joints s h o u l d therefore be e x a m i n e d radiologically in o r d e r to detect evidence of ankylosing spondylitis. Case 2 emphasizes the i m p o r t a n c e of radiological examination in p r o v e d cases o f ankylosing spondylitis with patients c o m p l a i n i n g of pains in the neck, particularly if the head is b e c o m i n g flexed. I n Case 3 neurological signs p r o g r e s s e d rapidly after the discovery of atlanto-axial dislocation. T h e s e were relieved i m m e d i a t e l y b y neck traction. T h i s patient had received h y d r o c o r t i s o n e t r e a t m e n t since admission to hospital. Progressive osteoporosis and s p o n t a n e o u s fractures can occur after prolonged cortisone and c o r t i c o t r o p h i n therapy. It is unlikely
ATLANTO-AXIAL
that h y d r o c o r t i s o n e t r e a t m e n t for the s h o r t period of two m o n t h s w o u l d p r o d u c e atlanto-axial dislocation. It appears t h a t atlanto-axial dislocation can occur at any time d u r i n g the active course of the disease. I n Case i, the dislocation o c c u r r e d two years before ankylosing spondylitis was s u s p e c t e d clinically. I n Case 2, ankylosing spondylitis had b e e n diagnosed eighteen m o n t h s prior to the dislocation. I n Case 3, r h e u m a t o i d arthritis h a d b e e n p r e s e n t for t w e n t y years before dislocation occurred.
SUMMARY L A review of p u b l i s h e d cases o f atlanto-axial dislocation in ankylosing spondylitis a n d r h e u m a t o i d arthritis is given. z. T h e aetiology of s p o n t a n e o u s atlanto-axial dislocation is discussed. 3. A description is given of 2 cases of ankylosing spondylitis and x case of r h e u m a t o i d arthritis complicated b y atlanto-axial dislocation. 4. C o m m e n t a r y is m a d e on these cases e m p h a sizing the i m p o r t a n c e of b e i n g aware that atlanto-axial dislocation can complicate ankylosing spondylitis and r h e u m a t o i d arthritis and t h a t dislocation can occur at any t i m e if the disease is active.
43
DISLOCATION
A c k n o w l e d g e m e n t s . - - I wish to t h a n k Dr. J. H . Follows a n d M r . J. H u n t e r A n n a n for p e r m i s s i o n to p u b l i s h details of the cases u n d e r t h e i r care. I am also grateful to Dr. R. I. Lewis and Dr. W . N i v e n for their criticism and advice. REFERENCES BELL, C. (I83O), " T h e Nervous System of the Human Body", from papers delivered to the Royal Society of Medicine, 4o3. London: Longman Co. BERKHEISTER, E. J., and SEIDLER, W. (I93I),ff. Amer. reed. Ass., 97, 517 . JONES, R. W. (I932), Proc. R. Soc. Med., 25, 586. KORNBLUM, D., CLAYTON, M. L., and NASH, H. H. (1952), ft. Amer. reed. Ass., 149, 431. MAROULIES, M. E., KATZ, I., and ROSENBERC, M. (1955), Neurology, 5, 290. PURSER, D. W., and SHARP, J. (1957), .,7. Bone Jr. Surg., 39 B, 582. RAND, C. W. (I944), The Neurological Patient: His Problems of Diagnosis and Care, 47. Springfield, II1. : C. C. Thomas. STAMMERS, F. A. R., and FRAZER,P. (I933), Lancet, 2, 12o3. VOLUTER, G., and V~TERNER,A. (I956), J. Radiol. Electrol., 37, 176. WERNE, S. (1957), Acta rheum, scand., 3, lOi. WITTEK, A. (I9O8), Miinch. reed. Wschr., 55, I836.
B O O K RE VIEWS A Text-book o f X-ray Diagnosis. By British Authors, in four volumes. Edited by S. COCHRANE SHANKS, C.B.E., M.D., F.R.C.P., F.F.R., Director, X-ray Diagnostic Department, Umversity College Hospital, London, and PETER KERLEY, C.V.O., C.B.E., M.D., F.R.C.P., F.F.R., D.M.R.E., Director, X-ray Department, Westminster Hospital, London. Third edition. Volume III. 9~ × 7 in. Pp. 883-ff xvi, with 8oz illustrations. I958. London : H. K. Lewis & Co. Ltd. £6 6s. THIS volume is §lightly larger than its predecessor--883 pages and 802 illustrations in place of 802 and 694 respectively--and is divided into six parts : Alimentary Tract (S. Cochrane Shanks, A. S. Johnstone, and Cecil G. Teall) ; Biliary Tract (Peter Kerley) ; The Abdomen (R. A. Kemp Harper) ; Radiology in Obstetrics (E. Rohan Williams) ; Gynsecological Radiology (E. Rohan Williams) ; and Urinary Tract (A. S. Johnstone and P. G. Keates). Essentially, there is little change from the last edition but most parts contain additional information and Peter Allen and J. Blair Hartley have added valuable contributions to the sections concerning Pelvimetry and Cephalometry. Volume I of the third edition was reviewed in the July issue of this JOURNALand the general comm6nts made then are equally true of this volume ; as stated " i t remains an extremely sound, practical teaching b o o k " with a wealth of radiographic illustrations, and it maintains its pre-eminent place in radiological literature, being of particular value to the radiologist in training. The Editors have aimed at describing the fundamental principles in interpretation and at relating them to the dayto-day clinical problems of the average X-ray department. They have certainly achieved their object but, in some respects, this volume could be criticized as being too conservative, with too great an accent on radiological appearances and too little correlation of the clinical aspects. Again, some of the comments on the stomach
and duodenum after operation are open to question ; for instance, the statement that vagotomy " a t the time of writing" is already going out of fashion. Some would claim, and for some years have claimed, that this procedure, in association with pyloroplasty or gastroenterostomy, has a real place in the treatment of duodenal ulcers and is often preferable to gastric resection. These are, however, minor criticisms and this volume remains an outstanding product of British Radiology ; it is beautifully produced and the illustrations are of very high quality. C . N . PULVERTAFT
Etude Radiologique de la Circulation Veineuse du M c m b r e Sup~rieur. By ERCOLE LAVIZZARI and VALERIO OTTOLINI. 9½ × 6½ in. Pp. I22 @ X, with 38 illustrations, i958. Paris : Masson. 2,400 fr. THE intention of this book, according to the authors, is to provide an accurate description of the phlebographic anatomy of the veins of the hand and arm, to illustrate the course of the circulation with particular reference to the drainage from superficial areas to the deep veins, and to describe methods for demonstrating the individual venous trunks with reasonable constancy and accuracy. The findings are based on 228 phlebograms performed on i8o patients referred routinely for intravenous pyelography, and consequently only normal appearances have been described. In general the authors have succeeded well in their aims. The book is clearly written and illustrated, many of the reproductions being of a very high quality, and it will no doubt form a standard reference work for the appearances of normal veins. One is left, however, with the distinct feeling that the clinical applications of phlebography in the arm are likely to be extremely limited. J. D. D o w
[See also p. 2o]
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
Spontaneous Dislocation of the Atlanto-axial Articulation occurring in Ankylosing Spondylitis and Rheumatoid Arthritis T. L. C. PRATT, M.R.C.S., L.R.C.P., D.M.R.D.
Halifax and Huddersfield Hospital Groups -SEVERAL cases of atlanto-axial dislocation due to acute cervical and nasopharyngeal inflammation have been described. Bell first recorded the condition, due to pharyngeal ulceration, in 183o. Watson Jones (1932) describes a dramatic cause of sudden death in a girl who dislocated her atlas while a nurse was dressing an infected sinus of her neck. Atlanto-axial dislocation due to ankylosing spondylitis and rheumatoid arthritis has rarely been
June I5, I95o. Spontaneous atlanto-axlal dislocatlon. Cause not apparent.
Fig, I . - - C a s e I.
recorded. Kornblum, Clayton, and Nash (I952) describe 2 cases, and 2 further cases where the subluxation occurred at a lower level in the cervical spine. Rand (1944) reported a patient with a Brown-Sequard syndrome due to atlanto-axial dislocation who also had ankylosing spondylitis, although association of the two conditions was not recognized. Stammers and Frazer (1933) describe spontaneous atlanto-axial dislocation in a man with a clinical history suggesting ankylosing spondylitis. Margulies, Katz, and Rosenberg (1955) report one case and include the histology of tissue obtained from the upper cervical region. W e m e (1957) describes atlanto-axial dislocation in a girl aged 4 who was suffering from rheumatoid arthritis. He discovered 3 more cases attending the University
of Lund, and 3 fresh cases were diagnosed at the Orthopmdic Policlinic over the following three years. He remarks on delay in diagnosis because of either ignorance of the condition or difficulty in radiographic interpretation. One case of ankylosing spondylitis with atlanto-axial dislocation is described by Voluter and Werner (1956). At a meeting of the British Orthopaedic Association, Purser and Sharp (1957) stated that they had
Fig. 2.--Case I. May 7, I957. Stable atlanto-axial dlslocation.
Advanced ankylosing spondyhtis
seen 17 cases of ankylosing spondylitis and 23 cases of rheumatoid arthritis with atlanto-axial dislocation and they comment upon the scarcity of recorded cases in the literature. T h e aetiology of spontaneous atlanto-axial dislocation is the same whether due to acute or chronic infection. Hyper~emia accompanying cervical infection results in decalcification of the atlas which may be sufficient to allow detachment of the transverse ligament. Finally, the atlas glides forward over the superior articular surfaces of the axis with consequent flexion of the head. Berkheister arid Seidler (1931) describe a sudden atlanto-axial dislocation due to transient effusion into the atlanto-axial joints. This incident occurred
ATLANTO-AXIAL d u r i n g a h y p e r s e n s i t i v i t y reaction to p e r t u s s i s vaccine. Wittek (I9o8) also indicated t h a t an effusion due to metastatic infection d i s t e n d e d the small capsules of the atlanto-axial joints, t h e r e b y relaxing the accessory ligaments. T h i s process has therefore b e e n suggested as a n o t h e r factor p r e c e d i n g atlanto-axial dislocation. Histology of resected tissue f r o m the case quoted b y Margulies and others s h o w e d " r e p l a c e m e n t of b o n e - m a r r o w b y fibro-collagenous tissue containing
DISLOCATION
moved either for months and attributed her complaint to teeth extractions twelve months previously. A radiograph showed anterior subluxation of atlas upon axis ( F i g . i ) . E.S.R. 20 mm. per hour. June x6. A plaster cast was applied and replaced by further casts during the following months in order to try to extend the head gradually. Jan. 8, I95I. Plaster cast removed. No tenderness on palpation of cervical spine. The atlanto-axial dislocation was considered stable.
o._
Fig.
2. July 16, I956. Spontaneous atlanto-axial dislocation with ankylosingspondylitis.
3.--Case
dense infiltrate of p o l y m o r p h o n u c l e a r leucocytes, lymphocytes, occasional p l a s m a cells and m o n o c y t e s " . T h e r h e u m a t o i d condition t h u s involves extra-articular b o n e as well as synovial tissue and o t h e r articular soft tissues. F r o m these pathological processes, it is seen that atlanto-axial dislocation m u s t occur d u r i n g an active p h a s e of the disease. A c u t e n e i g h b o u r i n g infections cause s u d d e n atlanto-axial dislocations w i t h possible neurological signs due to p r e s s u r e o f the o d o n t o i d process of the axis and posterior arch of the atlas on the spinal cord. S u d d e n d e a t h m a y occur. T h e dislocation complicating c h r o n i c infection is a gradual process and K o r n b l u m and others suggest that serious spinal cord damage is rare for this reason. It is remarkable that the spinal cord escaped p e r m a n e n t damage in the severe degrees of atlantoaxial dislocation seen in the following cases.
CASE REPORTS I . - - J u n e i5, 195o. A woman aged 38 complained of pain in the neck and right arm. Her neck was rigid and her head was flexed. She stated that she had not Case
41
W-
"- "
.
.......
V
o
"
:
" -~
F i g . 4 . - - C a s e 2. Dec. 7, 1957. Bone-graftinghas produced a strong bony bridge between C2 and external OCClpltal protuberance.
Jan. 24. Patient complained of dysphagia. Enlarged tonsils and posture thought to be the cause. May 9. Radiographic appearance of cervical spine on review showed early loss of definition in posterior intervertebral joints. June 2o. Examination revealed a small thyroid goitre and there was tremor of the hands. She was treated with thiouracil which controlled the thyrotoxic symptoms and relieved her dysphagia. Dec. i2. Cervical spine again tender. Jan. 5, 1952. Patient four months pregnant. Because of the atlanto-axial dislocation, the pregnancy was terminated and hysterectomy performed. Aug. 7. Patient complained of stiffness of lower lumbar spine. Radiographs showed active ankylosing spondylitis affecting the whote of the spine and the sacro-iliac joints. Aug. 24, 1955. Patient complained of pain in right sacro-iliac joint of two months' duration. Her back was very stiff and the cervical spine was stiff but not tender. A course of deep X-ray therapy relieved her symptoms and since then she has attended hospital on several occasions for physiotherapy. May 7, 1957. Radiographs showed advanced ankylosing spondylitis affecting the posterior articulations of the cervical vertebree and ossification of the longitudinal ligaments. The atlas was still dislocated anteriorly ( F i g . 2).
42
JOURNAL
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THE
FACULTY
Case 2.--April IO, 1946. A man aged 19 complained of pain in the lower back and legs. Aug. 21, 1952. He again attended hospital complaining of pain over the chest and shoulders. Radiographs showed cervical ribs but no evidence of disease in chest or shoulders. E.S.R. normal. Dec. 15, 1954. Twelve months' history of stiffness of spine which had been progressive and spread to neck. Chest expansion was t in. Forward bending was still
OF
RADIOLOGISTS
May 13. Radiographs showed bony consolidation ot bridge between C2 and occiput. Dec. 7. Radiographs showed a good bony bridge between C2 and oceiput. The patient had dispensed with his neck brace and had returned to work as a clerk (Fig. 4). Case 3.--Oct. 9, 1956. A woman aged 56, suffering from rheumatoid arthritis, was admitted to hospital complaining of pain in knees, ankles, and back, and tingling sensations in her hands. She was obese and unable to walk. Her hands, knees, and feet were deformed due to rheumatoid arthritis. Hydrocortisone treatment was started on the following day: IOO mg. for 4 days, followed by 75 mg. for 3 days, and 50 mg. daily until Jan. 2i, 1957. Dec. 18. She complained of weakness of both hands. B.S.R., 36 mm. in one hour. Dee. 29. Radiographs showed "osteophytosis of C2, 3, 4, 5, and 6 with diminished disc spaces. There is anterior displacement of the atlas upon the axis which suggests dissolution of the transverse ligament. This can occur in rheumatoid arthritis". (Fig. 5.) Neurological examination showed no sensory loss in the limbs. The plantar responses were extensor. A cervical collar was applied. Jan. 20, 1057. Marked weakness in arms and hands. Sensations normal, reflexes normal. Plantar responses extensor. Jan. 23. Increasing quadriplegia secondary to dislocation of atlas. Neck traction applied with improvement in strength of arms and legs. March 2o. Atlanto-axial dislocation considered stable. A spinal support with neck piece was ordered. During the following months the patient developed melancholia. She lost weight and occult blood was discovered in her stools. The possibility of neoplasm in the alimentary tract was considered but her condition was too .poor for this to be investigated by radiographic examination. She became incontinent of urine and developed a small pressure sore on the left buttock. Her general condition gradually deteriorated and she died on Oct. 29.
COMMENTARY
Fig. 5.--Case 3. Dee. 29, 1956. Spontaneous atlanto-axial dislocation. Rheumatoidarthritis for twenty years.
possible to a small extent. His general posture was typical of ankylosing spondylitis. Dec. 20. Radiographs showed early changes in the sacro-iliac joints and posterior articulations of the lower lumbar and cervical spine due to ankylosing spondylitis. Jan. 4, 1955. Pain in neck and stiffness. E.S.R. normal. Deep X-ray therapy was given to neck and sacro-iliae joints, with relief of pain and improvement of mobility. May 25. Pain and increasing stiffness in cervical spine was treated by physiotherapy with some relief of symptoms. July 16, 1956. The patient complained that while standing, he felt a sudden 'click' in his neck followed by flexion of his head. This frightening experience occurred on several occasions and was due to atlanto-axial dislocation. When supine the dislocation reduced itself. Radiographs showed anterior dislocation of the atlas upon the axis. Loss of definition in posterior intervertebral joints in upper cervical spine was due to ankylosing spondylitis (Pig. 3). July 17. Plaster jacket and collar were applied. Feb. 15, I957. Bone-chip grafts from right posterior iliac crest were inserted between spinous process of C2 and external occipital protuberance of occiput and the two latter were wired. April 27. Neck brace fitted.
T h e s e t h r e e cases illustrate the i m p o r t a n c e of r e m e m b e r i n g that ankylosing spondylitis and r h e u m a toid arthritis can cause atlanto-axial dislocation. I n Case i the reason for the atlanto-axial dislocation was n o t a p p a r e n t at first, although infection f r o m the t e e t h p r i o r to extraction was considered possible. T h e first c o m p l a i n t of pain in the back occurred two years later and radiographs c o n f i r m e d the diagnosis of ankylosing spondylitis. I f radiological examination of the whole spine and the sacroiliac joints h a d b e e n m a d e w h e n the p a t i e n t first a t t e n d e d hospital, the diagnosis would p r o b a b l y have b e e n a p p a r e n t at that t i m e in spite of the lack of relevant signs and s y m p t o m s . I n any case of s p o n t a n e o u s atlanto-axial dislocation w i t h no a p p a r e n t clinical cause, the whole spine and the sacro-iliac joints s h o u l d therefore be e x a m i n e d radiologically in o r d e r to detect evidence of ankylosing spondylitis. Case 2 emphasizes the i m p o r t a n c e of radiological examination in p r o v e d cases o f ankylosing spondylitis with patients c o m p l a i n i n g of pains in the neck, particularly if the head is b e c o m i n g flexed. I n Case 3 neurological signs p r o g r e s s e d rapidly after the discovery of atlanto-axial dislocation. T h e s e were relieved i m m e d i a t e l y b y neck traction. T h i s patient had received h y d r o c o r t i s o n e t r e a t m e n t since admission to hospital. Progressive osteoporosis and s p o n t a n e o u s fractures can occur after prolonged cortisone and c o r t i c o t r o p h i n therapy. It is unlikely
ATLANTO-AXIAL
that h y d r o c o r t i s o n e t r e a t m e n t for the s h o r t period of two m o n t h s w o u l d p r o d u c e atlanto-axial dislocation. It appears t h a t atlanto-axial dislocation can occur at any time d u r i n g the active course of the disease. I n Case i, the dislocation o c c u r r e d two years before ankylosing spondylitis was s u s p e c t e d clinically. I n Case 2, ankylosing spondylitis had b e e n diagnosed eighteen m o n t h s prior to the dislocation. I n Case 3, r h e u m a t o i d arthritis h a d b e e n p r e s e n t for t w e n t y years before dislocation occurred.
SUMMARY L A review of p u b l i s h e d cases o f atlanto-axial dislocation in ankylosing spondylitis a n d r h e u m a t o i d arthritis is given. z. T h e aetiology of s p o n t a n e o u s atlanto-axial dislocation is discussed. 3. A description is given of 2 cases of ankylosing spondylitis and x case of r h e u m a t o i d arthritis complicated b y atlanto-axial dislocation. 4. C o m m e n t a r y is m a d e on these cases e m p h a sizing the i m p o r t a n c e of b e i n g aware that atlanto-axial dislocation can complicate ankylosing spondylitis and r h e u m a t o i d arthritis and t h a t dislocation can occur at any t i m e if the disease is active.
43
DISLOCATION
A c k n o w l e d g e m e n t s . - - I wish to t h a n k Dr. J. H . Follows a n d M r . J. H u n t e r A n n a n for p e r m i s s i o n to p u b l i s h details of the cases u n d e r t h e i r care. I am also grateful to Dr. R. I. Lewis and Dr. W . N i v e n for their criticism and advice. REFERENCES BELL, C. (I83O), " T h e Nervous System of the Human Body", from papers delivered to the Royal Society of Medicine, 4o3. London: Longman Co. BERKHEISTER, E. J., and SEIDLER, W. (I93I),ff. Amer. reed. Ass., 97, 517 . JONES, R. W. (I932), Proc. R. Soc. Med., 25, 586. KORNBLUM, D., CLAYTON, M. L., and NASH, H. H. (1952), ft. Amer. reed. Ass., 149, 431. MAROULIES, M. E., KATZ, I., and ROSENBERC, M. (1955), Neurology, 5, 290. PURSER, D. W., and SHARP, J. (1957), .,7. Bone Jr. Surg., 39 B, 582. RAND, C. W. (I944), The Neurological Patient: His Problems of Diagnosis and Care, 47. Springfield, II1. : C. C. Thomas. STAMMERS, F. A. R., and FRAZER,P. (I933), Lancet, 2, 12o3. VOLUTER, G., and V~TERNER,A. (I956), J. Radiol. Electrol., 37, 176. WERNE, S. (1957), Acta rheum, scand., 3, lOi. WITTEK, A. (I9O8), Miinch. reed. Wschr., 55, I836.
B O O K RE VIEWS A Text-book o f X-ray Diagnosis. By British Authors, in four volumes. Edited by S. COCHRANE SHANKS, C.B.E., M.D., F.R.C.P., F.F.R., Director, X-ray Diagnostic Department, Umversity College Hospital, London, and PETER KERLEY, C.V.O., C.B.E., M.D., F.R.C.P., F.F.R., D.M.R.E., Director, X-ray Department, Westminster Hospital, London. Third edition. Volume III. 9~ × 7 in. Pp. 883-ff xvi, with 8oz illustrations. I958. London : H. K. Lewis & Co. Ltd. £6 6s. THIS volume is §lightly larger than its predecessor--883 pages and 802 illustrations in place of 802 and 694 respectively--and is divided into six parts : Alimentary Tract (S. Cochrane Shanks, A. S. Johnstone, and Cecil G. Teall) ; Biliary Tract (Peter Kerley) ; The Abdomen (R. A. Kemp Harper) ; Radiology in Obstetrics (E. Rohan Williams) ; Gynsecological Radiology (E. Rohan Williams) ; and Urinary Tract (A. S. Johnstone and P. G. Keates). Essentially, there is little change from the last edition but most parts contain additional information and Peter Allen and J. Blair Hartley have added valuable contributions to the sections concerning Pelvimetry and Cephalometry. Volume I of the third edition was reviewed in the July issue of this JOURNALand the general comm6nts made then are equally true of this volume ; as stated " i t remains an extremely sound, practical teaching b o o k " with a wealth of radiographic illustrations, and it maintains its pre-eminent place in radiological literature, being of particular value to the radiologist in training. The Editors have aimed at describing the fundamental principles in interpretation and at relating them to the dayto-day clinical problems of the average X-ray department. They have certainly achieved their object but, in some respects, this volume could be criticized as being too conservative, with too great an accent on radiological appearances and too little correlation of the clinical aspects. Again, some of the comments on the stomach
and duodenum after operation are open to question ; for instance, the statement that vagotomy " a t the time of writing" is already going out of fashion. Some would claim, and for some years have claimed, that this procedure, in association with pyloroplasty or gastroenterostomy, has a real place in the treatment of duodenal ulcers and is often preferable to gastric resection. These are, however, minor criticisms and this volume remains an outstanding product of British Radiology ; it is beautifully produced and the illustrations are of very high quality. C . N . PULVERTAFT
Etude Radiologique de la Circulation Veineuse du M c m b r e Sup~rieur. By ERCOLE LAVIZZARI and VALERIO OTTOLINI. 9½ × 6½ in. Pp. I22 @ X, with 38 illustrations, i958. Paris : Masson. 2,400 fr. THE intention of this book, according to the authors, is to provide an accurate description of the phlebographic anatomy of the veins of the hand and arm, to illustrate the course of the circulation with particular reference to the drainage from superficial areas to the deep veins, and to describe methods for demonstrating the individual venous trunks with reasonable constancy and accuracy. The findings are based on 228 phlebograms performed on i8o patients referred routinely for intravenous pyelography, and consequently only normal appearances have been described. In general the authors have succeeded well in their aims. The book is clearly written and illustrated, many of the reproductions being of a very high quality, and it will no doubt form a standard reference work for the appearances of normal veins. One is left, however, with the distinct feeling that the clinical applications of phlebography in the arm are likely to be extremely limited. J. D. D o w
[See also p. 2o]