Rheumatology and Rehabilitation, 1976, 15, 67

ORIGINAL PAPER

ANKYLOSING HYPEROSTOSIS WITH CERVICAL SPINAL CORD COMPRESSION BY T. GIBSON* AND H. RALPH SCHUMACHER

SUMMARY This report describes a patient whose clinical and radiological features conform to those of ankylosing hyperostosis. Posterior bridging osteophytes were also apparent and these have not been previously described in ankylosing hyperostosis. We have considered alternative explanations for this finding and concluded that they were manifestations of the hyperostotic process. This unusual feature occurring in the presence of a comparatively narrow spinal canal resulted in cord compression and a spastic tetraparesis and we would therefore suggest that ankylosing hyperostosis may sometimes have serious neurological consequences. hyperostosis as commonly described is said to be a benign disorder which by definition comprises large anterior osteophytes of the spine with little or no symptoms, few clinical signs and no serious sequelae (Forestier and Lagier, 1971). We report a patient in whom the picture of ankylosing hyperostosis was complicated by the presence of bridging posterior osteophytes and cervical spinal-cord compression. ANKYLOSING

CASE REPORT

H.S., a 66-year-old black male, was admitted to the Philadelphia Veterans Administration Hospital complaining of pain and stiffness in the right hip region of three years' duration. Prior to this illness he had been an active farm worker with good health. He denied antecedent musculo-skeletal symptoms and specifically there was no history of spinal trauma or pain but he had experienced some lumbar stiffness. Examination revealed an elderly man who was unable to stand erect because of spasticity of the right hip flexors. Spasticity and weakness were apparent in all four limbs but were more pronounced on the right side. All tendon reflexes were accentuated except the left biceps which was absent. The plantar response was extensor on both sides. Vibration and joint position senses were absent in his feet but other modalities of sensation were normal. His gait was spastic and in order to walk he required assistance. There was rigidity of his lumbo-sacral and dorsal spines. Neck movement was limited to 5° of flexion, 10° of rotation in either direction and no extension. His pulse was regular and his blood pressure 160/100. His heart, lungs and abdomen were normal. An initial clinical diagnosis of degenerative right hip disease was not substantiated by a pelvic radiograph which showed normal hip and sacroiliac joints. Spinal radiographs indicated a bony mass continuous with the anterior border of the fourth to seventh cervical vertebrae (Fig. la). The intervertebral disc spaces were well maintained but osteophytes, best seen in oblique views of the cervical spine, arose from the posterior margins of the third to seventh cervical vertebrae. These formed posterior bridges between the fourth, fifth and sixth cervical vertebrae (Fig. \b). The sagittal diameter of •Present address: Guy's Arthritis Research Unit, Guy's Hospital, London SE1 9RT. Accepted for publication July 1975. Reprint requests to: Dr. T. Gibson. 67

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University of Pennsylvania School of Medicine and Veterans Administration Hospital, Philadelphia, Pa

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DISCUSSION The paucity of spinal symptoms in conjunction with the striking radiological changes illustrated by this patient are consistent with the picture of ankylosing hyperostosis as described and popularized by Forestier and Rotes-Querol (1970). Primary vertebral osteophytosis (Bick, 1956), spondylosis deformans (Jaffe, 1972) and physiological vertebral ligamentous calcification (Smith et al., 1971) are terms employed by other authors to describe similar or identical changes. Typically, thick broad-based osteophytes are distributed along the anterior border of the vertebrae of elderly subjects, forming bridges across usually well preserved intervertebral disc spaces. In a Finnish population the prevalence of this finding increased in the seventh decade to 8.4 % in men and 4 . 3 % in women (Julkunen et al., 1971), an age and sex distribution which is in accord with those of other observers (Forestier and Lagier, 1971). Identical changes have nevertheless been described in a group of young subjects (DeSeze and Claisse, 1960). An increased incidence has been established among diabetics (Hajkova et al., 1965) and acromegalics without diabetes (Julkunen et al., 1966). Although hyperostosis most commonly affects the dorsal and lumbar spines (Forestier and Lagier, 1971), florid anterior cervical osteophytes such as we describe are not unknown in this condition (Spilberg and Lieberman, 1972). Similar large anterior, bridging osteophytes of the cervical spine have been described in patients with a history of neck injury but these changes have been localized to that area (Bailey and Kato, 1972). Posterior osteophytes are not an acknowledged feature of ankylosing hyperostosis but they commonly accompany intervertebral disc degeneration in spinal spondylosis (Nurick, 1972). There was no convincing narrowing of the intervertebral disc spaces in our patient but we cannot exclude the possibility that disc degeneration insufficient to cause radiological narrowing, co-existed with the changes of hyperostosis. Indeed,

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the cervical spinal canal measured by the method of Burrows (1963) was 14 mm and 15 mm at the C3 and C4 levels respectively. Extension and flexion views of the cervical spine revealed no subluxation. In the dorsal and lumbar spines, anterior intervertebral bridging was seen with particularly thick bone formation anterior to the lower four lumbar vertebrae between which there was also posterior bridging (Fig. 2). Other investigations included haematocrit 41 %; WBC 4300/mm8; ESR (Westergren) 10 mm/h; serum calcium 9.4 mg/100 ml; inorganic phosphate 3.2 mg/100 ml; total protein 7.0 g/100 ml; albumin 4.6 g/100 ml; alkaline phosphatase 80 mu/ml; fasting blood sugar 96 mg/100 ml; two-hour post-prandial blood sugar 107 mg/100 ml; urine calcium 258 mg/24 h; urine inorganic phosphate 490 mg/24 h; serum creatinine 0.8 mg/100 ml; blood urea nitrogen 14 mg/100 ml and serum uric acid 4.3 mg/100 ml. A lumbar puncture revealed an opening pressure of 100 mm. The cerebrospinal fluid was xanthochromic and contained 499 non-crenated red cells/mm8; a normal glucose of 66 mg/100 ml and an elevated protein of 259 mg/100 ml. Myelography demonstrated a complete interruption of the column of contrast material by osteophytes at the third and fourth cervical interspace. There was indentation of the column by osteophytes of all cervical intervertebral spaces below this. A brain scan and an EEG were normal. The patient underwent laminectomy of the third to seventh cervical vertebrae. One month later there was no change in muscle strength but spasticity was diminished, the plantar response was flexor, the left biceps reflex could be elicited and there was return ofjoint position sense in the feet He had by this time resumed walking with the aid of a frame and was free of hip pain.

I

FIG. 1.—Lateral view of cervical spine showing (o) massive anterior connecting osteophytes and (b) posterior osteophytes with bridging between C4, C5, and C6 vertebrae (arrows).

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PLATE I—continued

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FIG. 2.—Lateral view of lumbar spine showing thick anterior bridging and posterior osteophytes spanning the disc spaces between L2, L3, L4 and L5 vertebrae (arrows).

GIBSON AND SCHUMACHER: ANKYLOSING HYPEROSTOSIS

69

ACKNOWLEDGMENT

This study was supported in part by the Barsumian Memorial Fund. REFERENCES

H. L. and KATO, F. (1972) "Paravertebral Ossification of the Cervical Spine". South Med. J., 65, 189. BICK, E. M. (1956) "Primary Vertebral Osteophytosis". New York State J. Med., 56,1255. BURROWS, E. H. (1963) "The Sagittal Diameter of the Spinal Canal in Cervical Spondylosis". Clin. Radiol., 14, 77. CRANDALL, P. H. and BATZDORF, U. (1966) "Cervical Spondylotic Myelopathy". / . Newosurg. 25,57. DESEZE, S. and CLAISSE, R. (1960) "Hyperostose Vertebrale Lombaire Juvenile". Rev. Rhum., 27, 219. BAILEY,

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whether a true distinction can be made between spondylosis and hyperostosis is moot. Forestier and Lagier (1971) have preferred to confine the term spondylosis to those patients in whom osteophytes are clearly associated with disc degeneration whereas Sokoloff (1969) believed they are manifestations of the same degenerative process. The intervertebral bridging of the posterior osteophytes seen in both the lumbar and cervical spines of the case we describe makes us suspect that their formation was related to the same mechanism causing the more striking anterior bony outgrowths. Similar posterior bridging of the intervertebral spaces may be a feature of ankylosing spondylitis but the clinical history, normal sedimentation rate and normal sacroiliac joints preclude this diagnosis in our patient. Both anterior and posterior bridging attributable to ligamentous calcification may occur in fluorosis (Singh et at., 1962) and hypoparathyroidism (Jimenea et ah, 1971). Paraplegia, albeit not demonstrably related to the skeletal changes, has been reported in such a patient with hypoparathyroidism (Gibberd, 1965). In the case we describe there was no additional evidence to suggest either fluorosis or hypoparathyroidism. Isolated calcification of the posterior longitudinal ligament seems to occur more commonly in the Japanese population. Radiologically it resembles the posterior bridging we describe and it is a well recognized cause of spinal cord compression (Hiramatsu and Nobechi, 1971). This entity affects posterior structures exclusively and thus cannot be applied to the case under discussion. The rarity of neurological complications in ankylosing hyperostosis has been stressed (Forestier and Lagier, 1971) but if hyperostosis represents one extreme of a degenerative process which encompasses disc degeneration and posterior osteophytes, this is a surprising observation. The patient we describe had unusual bridging of posterior osteophytes which we attributed to ankylosing hyperostosis and which resulted in cord compression. Posterior osteophytes only rarely cause spinal cord compression but narrow spinal canals predispose to this complication (Payne and Spillane, 1959). It is of interest to note that the sagittal diameters of the canal in our patient were 14 mm and 15 mm at the C3 and C4 vertebral levels, values which are narrower than the mean of normal adults (Nurick, 1972) and comparable to the means found in two series of subjects with cervical cord compression caused by spondylosis (Nurick, 1972; Payne and Spillane, 1959). The response to laminectomy did not result in complete neurological recovery but did include a reduction of spasticity and elimination of the presenting complaint of hip pain. Laminectomy can be expected to produce some improvement in 67% of patients with cervical myelopathy due to spondylosis and as in the case reported here, pain and spasticity are relieved more often than weakness (Crandall and Batzdorf, 1966).

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J. and LAGIER, R. (1971) "Ankylosing Hyperostosis of the Spine". Clin. Orthop., 74, 65. and ROTES-QUEROL, J. (1970) "Senile Ankylosing Hyperostosis of the Spine". Ann. Rheum. Dis., 9, 321. GIBBERD, F. B. (1965) "Idiopathic Hypoparathyroidism with Unusual Bone Changes and Spastic Paraplegia". Acta Endocrinol., 48, 23. HAJKOVA, Z., STREDA, A. and SKRHA, F. (1965) "Hyperostotic Spondylosis and Diabetes Mellitus". Ann. Rheum. Dis., 24, 536. HIRAMATSU, Y. and NOBECHI, T. (1971) "Calcification of the Posterior Longitudinal Ligament of the Spine among the Japanese". Diagn. Radiol., 100,307. JAFFE, H. L. (1972) Metabolic, Degenerative and Inflammatory Disease of Bone and Joints, 1st ed. p. 762. London: Henry Kimpton. JIMENEA, C. V., FRAME, B., CHAYKIN, L. B. and SIGLER, J. W. (1971) "Spondylitis of Hypoparathyroidism". Clin. Orthop., 74,84. JULKUNEN, H., HEINONEN, O. P. and PYORALA, K. (1971) "Hyperostosis of the Spine in an Adult Population: Its Relation to Hyperglycaemia and Obesity". Ann. Rheum. Dis., 30,605. KARAVA, R. and VILJANEN, V. (1966) "Hyperostosis of the Spine in Diabetes Mellirus and Acromegaly". Diabetologia, 2,123. NURICK, S. (1972) "The Pathogenesis of the Spinal Cord Disorder Associated with Cervical Spondylosis". Brain, 95, 87. PAYNE, E. E. and SPILLANE, J. D. (1959) "The Cervical Spine: An Anatomico-pathological Study of 70 Specimens (Using a Special Technique) with Particular Reference to the Problem of Cervical Spondylosis". Brain, 80,571. SINGH, A., DASS, R., HAYREH, S. S. and JOLLY, S. S. (1962) "Skeletal Changes in Endemic Fluorosis". /. BoneJt. Surg., 44B, 806. SMITH, C. F., PUGH, D. G. and POLLEY, H. F. (1971) "Physiological Vertebral Ligamentous Calcification. An Ageing Process". Am. J. Roentgenol. Radium. Ther. Nucl. Med., 74, 1049. SOKOLOFF, I. (1969) The Biology of Degenerative Joint Disease, 1st edit., p. 94. Chicago: University of Chicago Press. SPILBERG, I. and LIEBERMAN, D. M. (1972) "Ankylosing Hyperostosis of the Cervical Spine". Arthritis Rheum., 14, 208. FORESTIER,

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Ankylosing hyperostosis with cervical spinal cord compression.

Rheumatology and Rehabilitation, 1976, 15, 67 ORIGINAL PAPER ANKYLOSING HYPEROSTOSIS WITH CERVICAL SPINAL CORD COMPRESSION BY T. GIBSON* AND H. RALP...
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