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Congenital Narrowing of the Cervical Spinal Canal

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Congenital Narrowing of the Cervical Spinal Canal J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.38.12.1218 on 1 December 1975. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry, 1975, 38, 1218-1224 Congenital narrowing of the cervical spinal canal JEFFREY T. KESSLER From the Department ofNeurology, The New York Hospital-Cornell Medical Center, New York, New York 10021, U.S.A. SYNOPSIS The clinical and laboratory findings in six patients with congenital narrowing of the cervical spinal canal and neurological symptoms are described. A variable age of onset and an entirely male occurrence were found. Signs and symptoms of spinal cord dysfunction predominated in all but one patient. Symptoms were produced in five patients by increased physical activity alone. Congenital narrowing of the cervical spinal canal may result in cord compression without a history of injury and occasionally without evidence of significant bony degenerative changes. The clinical features may be distinguishable from those found in cervical spondylosis without congenital nar- rowing. Intermittent claudication of the cervical spinal cord appears to be an important feature of this syndrome. Surgery improved four out of five people. guest. Protected by copyright. The cervical spinal canal is a triangular tube can be found in most large series of this disorder that diminishes in size from top to bottom (Payne and Spillane, 1957; Epstein et al., 1963; (Burrows, 1963). The transverse (interpedicular) Crandall and Batzdorf, 1966; Symon and Lav- diameter is nearly twice that of the sagittal ender, 1967; Galera and Tovi, 1968; Wilkinson (anteroposterior) diameter. Thus, the most et al., 1969; Eyssette et al., 1970; Godlewski, critical radiographic measurement in the cer- 1972; Nurick, 1972a). However, clinical features vical region in evaluating for cord compression peculiar to congenitally narrowed canals have is the sagittal diameter (Boijsen, 1954; Wolf not been noted in previous studies (Hinck et al., et al., 1956; Penning, 1962). Average values for 1964; Hinck and Sachdev, 1966; Moiel et al., the sagittal diameter of the cervical spinal canal 1970; Lurati and Mertens, 1971). The present in normal individuals are 22-23 mm for Cl; study reports, in six cases of congenital narrow- 20-20.5 mm for C2; 18.3-18.5 mm for C3, and ing of the cervical spinal canal, clinical and 17.0-17.8 mm for C4 to C7 vertebral levels with radiographic findings which distinguish this little difference among the lower four segments group from the more common type of cervical (Wolf et al., 1956; Burrows, 1963; Lurati and myelopathy due to spondylosis. Mertens, 1971). Values below 14 mm are un- common and fall below two standard deviations METHOD at any cervical segment (Lurati and Mertens, http://jnnp.bmj.com/ 1971). Burrows (1963) found only six values Since no record coding category for congenital spinal below 14 mm in 2 100 individual segmental stenosis existed, 990 charts from The New York in Hospital coded for cervical arthritis, cervical measurements 300 normal subjects and Wolf laminectomy, and hypertrophy of the ligamentum et al. (1956) found 21 such values in 1400 meas- flavum during 1968 to 1973 and 184 charts coded urements in 200 normal subjects. for cervical laminectomy from 1963 to 1967 were The importance of the constitutional sagittal reviewed. Six patients were found whose radio- diameter of the cervical spinal canal to the sub- graphic studies indicated congenital narrowing; two on September 28, 2021 by sequent development of spondylotic myelo- of these were examined personally. Patients were pathy is well documented. Patients with con- considered to have congenital cervical spinal canal genital narrowing of the cervical spinal canal narrowing if the constitutional sagittal diameter on a standard lateral radiograph was less than 14 mm. (Accepted 17 June 1975.) Measurements were made from the posterior margin 1218 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.38.12.1218 on 1 December 1975. Downloaded from Congenital narrowing ofthe cervical spinal canal 1219 of the vertebral body to the cortical line at the site of generative changes were noted at C3-4, C4-5, and fusion of the spinous process and the laminae. C5-6 intervertebral spaces which produced a con- Plain films, tomograms and myelograms were re- siderable degree of obstruction of the subarachnoid viewed with staff neuroradiologists. space with probable spinal cord compression at these levels. The CSF protein was 0.71 g/l and the sugar CASE 1 (NYH 1333207) A 39 year old male taxicab 3.05 mmol/l. There was one mononuclear cell per driver presented with difficulty in walking for two or cubic mm. At surgery, a total cervical laminectomy three months. He was well until three months before uncovered a thinned translucent dura mater which admission when he noted cramps in both calves bulged as the laminectomy was done. No epidural awakening him at night and lasting about 30 seconds. fat was found. Six to eight weeks before admission he noted in- No immediate change was noted postoperatively creasing weakness in both lower extremities, espec- and the patient was lost to follow-up. ially climbing stairs. Numbness lasting about 15 minutes developed after exercise and was greater in CASE 3 (NYH 118905) A 53 year old magistrate the right leg than the left. had been well until three weeks before admission General physical examination was unremarkable. when he noted unsteadiness on his feet and occas- Neurological examination revealed 4/5 strength in ional cramp in his legs. His daughter had thought the hamstrings, hip abductors, and hip flexors with that he seemed unsteady when climbing stairs and increased muscle tone in both legs. Sensory exam- dancing for the past year, though the patient denied ination disclosed only an inconsistent response to this. Two days before admission, while doing 'sit- pin prick below Ti 1 dermatome. Gait was spastic and ups', he suddenly developed weakness of both upper 'scissors-like'. Reflexes were 2+ in the upper ex- and lower extremities without pain, numbness, or tremities, with 3 + knee jerks and 4+ ankle jerks paraesthesias. There was no history of neck pain, guest. Protected by copyright. and bilateral extensor plantar responses. No pain headache, bowel or bladder symptoms. or limitation of neck movement was found. There General physical examination was unremarkable. was no anal wink but the cremasteric responses Neurological examination revealed mild to moderate were intact. Cervical spine radiographs revealed a quadriparesis with spasticity, slightly greater on the narrow canal measuring 13 mm in the neutral posi- left, without wasting or fasciculations. His gait was tion at C4 vertebra. Lumbar iodophendylate (Panto- broad-based and ataxic with bilateral circumduc- paque) myelography revealed an almost complete tion, left greater than right. Position sense was block from C3 to C7 vertebral levels, with no evi- moderately symmetrically diminished in all four dence of osteophytes, ridges, or disc herniation. The extremities. Vibration sense was decreased to the CSF protein was 0.85 g/l and the sugar 2.33 mmol/l. pelvis and sensation to pin and temperature stimula- There were two mononuclear cells per cubic mm. tion were intact. Light touch sensation was slightly Only a bulging dura mater was found at surgery decreased in a C8-T1 dermatome distribution bi- despite careful search for other anomalies. Five laterally. Reflexes were 3 + in the upper extremities months postoperatively his gait had improved to and 4+ in the lower extremities with bilateral normal and only mild hyperreflexia remained with extensor plantar and Hoffman responses. flexor plantar responses. Cervical spine films revealed intervertebral disc space narrowing with degenerative changes and CASE 2 (NYH 1170119) A 47 year old man had a anterior spur formation involving C2, C3, and C4 12 to 18 month history of urinary frequency and evi- vertebrae with narrowing of the intervertebral fora- dence of incomplete voiding on pyelography. He mina and posterior spur formation at C2, C3, and http://jnnp.bmj.com/ had noted intermittent neck ache over the six to 12 C5 vertebrae on the left and C5 and C6.vertebrae on months before admission, as well as decreased the right. Marked narrowing of the anteroposterior ability to sustain an erection. The urinary symptoms canal diameter which appeared congenital in nature were exacerbated by taking alcohol and by shovelling was noted and was maximal at Cl vertebra where it snow. No other medical history was obtained. measured less than 11 mm. Physical examination revealed only a slightly en- Myelography revealed degenerative changes at larged prostrate. Neurological examination revealed C2-C3, C3-C4, C4-C5, and C5-C6 intervertebral brisk deep tendon reflexes at the triceps and in both spaces with considerable narrowing of the subarach- on September 28, 2021 by lower extremities. The rest of the examination was noid space due to osteophytes and a congenitally normal. narrow canal. At Cl vertebra the sagittal diameter Cervical spine films revealed a congenitally nar- was less than 11 mm with even more marked nar- row canal measuring as small as 10 mm at C6 rowing of the subarachnoid space. The CSF con- vertebra. At myelography, mild to moderate de- tained no cells and gave a 1+ Pandy reaction. At J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.38.12.1218 on 1 December 1975. Downloaded from 1220 Jeffrey T. Kessler surgery the C3, C4, C5, C6, and C7 vertebrae were was well until three years before admission when, found to be quite thin. There was no epidural fat while swimming in shallow (waist deep) water, he and the cord was compressed. C2 vertebra was thick suddenly became unable to move his arms, legs, or and Cl even thicker, markedly compressing the trunk and had to be pulled from the water by a cord at the foramen magnum.
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