Neuroradiolngv
Neuroradiology 17,207-209 (1979)
© by Springer-Verlag 1979
CASE REPORTS
The Contracting Cord Sign of Multiple Sclerosis V. M. Haughton t, Khang Cheng Ho 2 and R. A. Boedecker 1 Departments of Radiology1 and Pathology2, Medical College of Wisconsin and Milwaukee Country General Hospital, Milwaukee, Wisconsin, USA
Summary. A new sign of multiple sclerosis, the contracting cord sign, is described. The myelographic demonstration of a large cord that subsequently decreases in size may suggest multiple sclerosis. Multiple sclerosis must be considered in the differential diagnosis of an enlarged spinal cord. Distinguishing between the collapsing cord and the contracting cord is discussed.
Key words: Myelography - Multiple sclerosis
Myelographic demonstration of a normal or small spinal cord with multiple sclerosis (MS) is well documented [7]. In this report a new radiographic sign of MS is described: an enlarged spinal cord that subsequently diminishes in diameter. The contracting cord of MS has been observed[ by others [8]. It is Probably a more specific although less common sign of MS than the atrophic spinal cord. The contracting cord must be distinguished from the collapsing cord sign of a spinal cord cyst.
Case Report A female, aged 31, first entered Milwaukee County General Hospital on 8 July 1975 complaining of severe headache, and tingling and weakness in the right arm and leg for 6 weeks. Past history included two episodes of transient blindness in her left eye during the previous year. Neurological examination on admission revealed a stiff neck, horizontal nystagmus with a fast component to the left, right facial weakness, generalized weakness of all extremities, diffusely hyperactive deep tendon reflexes and a Hoffman sign on the right. Computed tomography
(CT), radionuclide brain scan, skull and spine radiographs, and vertebral angiogram were normal. Lumbar puncture revealed normal cerebrospinal fluid under normal pressure. The presumptive diagnosis was a degenerative disease of the central nervous system. Treatment with ACTH was begun and the patient was discharged on the 27th hospital day. The patient was readmitted on 18 September 1975 because of marked respiratory insufficiency and weakness of the right arm and leg. Examination at this time revealed paradoxical movement of the abdomen with respiration, severe spastic paralysis of the right lower extremity, flaccid paralysis of the right upper extremity, loss of pain and temperature sensation on the left side below the level of C2. There was a Babinski sign on the fight and horizontal nystagmus. Paralysis of the diaphragm was confirmed with fluoroscopy. The patient was intubated. An emergency gas myelogram revealed enlargement of the cervical cord (Fig. 1). The cord did not collapse with changes in the patient's position. A vertebral arteriogram revealed no evidence of neovascularity or abnormal staining in the cervical cord. The patient remained on a respirator and received high doses of steroids. The respiratory capacity gradually improved and the patient was discharged on 26 October 1975. There were several subsequent hospital admissions, at each of which the patient had exacerbation of weakness and respiratory difficulty. A repeat gas myelogram was performed on 6 April 1976 approximately six months after the first gas myelogram. At this time the spinal cord was small (Fig. 2). There was no change in the dimensions of the cord with changes in patient position. The patient expired on 12 January 1978. At autopsy the spinal cord was atrophic. Pathological examination revealed multiple demyelinating lesions in the spinal cord, brain stem and optic nerves characteristic of MS (Fig. 3). 0028-3940/79/0017/0207/$01.00
l~g. 1 a and b. Polytomograms from first gas myelogram. Silhouette of the cervical cord (arrows) is large in lateral (a) and A P (b) projections
Fig. 2 a and b. Second gas myelogram. Decrease in size of cord (arrows) is evident (a, lateral, b AP projection; polytomographic sections)
V. M. Haughton et al.: The Contracting Cord Signof Multiple Sclerosis
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Fig. 3. Photograph of transverse section of cervical cord which is small especiallyin AP diameter. Entire section shows demyelination.Onlyin one region (arrows) is myelin evident
Discussion The early pathological changes in a demyelinating lesion of MS are degeneration of the myelin sheath, edema and inflammatory and glial cell infiltration [1, 6]. E d e m a and reactive cell infiltration may be sufficient to enlarge the diameter of the cord on myelography as in our case. T o date no examples of an enlarged spinal cord have been reported with MS because patients infrequently have myelography during the acute stage of the illness. Possibly, edema or abnormal contrast enhancement in the spinal cord of demyelinating diseases may be detected by CT scanning in the future. When edema and infiltration are replaced by gliosis, the atrophic cord may be demonstrated myelographically [5, 7]. An enlarged spinal cord may suggest neoplasm or a cyst [2, 3, 4]. In a patient with an enlarged spinal cord and an atypical history for tumor, repeat myelography may be helpful to distinguish MS from tumor. Contraction of the cervical cord is atypical for tumor but probably characteristic of MS. In some cases, spinal cord angiography may be helpful to exclude neoplasm. T o exclude a spinal cord cyst as a cause of cord enlargement a two position gas myelogram should be performed [2]. The contracting cord sign should be distinguished from the collapsing cord sign. Changes in cord diameter caused by movement of fluid within a cyst of the spinal cord result in the collapsing cord sign. The collapsing cord sign is usually demonstrated during myelography by tilting the patient into different positions [2]. Spinal cord atrophy and reabsorption of
edema fluid are probably the cause of the contracting cord sign. Contraction of the cord is demonstrated, not by changing the patient's position during myelography, but by repeating the myelogram after several weeks. A cyst of the cord might simulate the contracting cord if myelograms, each with a different technique or contrast medium, were performed several weeks apart.
References 1. Dastur, D. K., Singhal, D. S.: Two unusual neuropathologically proven cases of multiple sclerosis from Bombay. J. of Neurol. Sci. 20, 397-414 (1973) 2. Haughton, V. M., Williams, A.L. Cusick, S.F., Meyer, G.: A myelographic technique for cysts in the spinal canal. Radiology 129, 717-719 (1978) 3. Williams, A.L., Haughton, V.M.: The present status of gas myelography: International advances in investigations of the spine. (ed. Judith Donovan Post) 1979 4. Klefenberg, G., Saltzman, G.: Gas myelographic studies in syfingomyelia. Acta Radiol. 52, 129-138 (1959) 5. McDonald, W. I.: Pathophysiologyin multiple sclerosis. Brain 97, 179-196 (1974) 6. Seitelberger, F.: Pathology of multiple sclerosis. Ann. Clin. Res, 5, 337-344 (1973) 7. Vakili, H.: The spinal cord. pp. 316-337. New York: Intercontinental 1967 8. Westberg, G.: Personal communication Received: 29 September 1978 Dr. V. M. Haughton Department of Radiology Milwaukee County Medical Complex 8700 West Wisconsin Avenue Milwaukee, WI 53226, USA
Neuroradiology 17,207-209 (1979)
© by Springer-Verlag 1979
CASE REPORTS
The Contracting Cord Sign of Multiple Sclerosis V. M. Haughton t, Khang Cheng Ho 2 and R. A. Boedecker 1 Departments of Radiology1 and Pathology2, Medical College of Wisconsin and Milwaukee Country General Hospital, Milwaukee, Wisconsin, USA
Summary. A new sign of multiple sclerosis, the contracting cord sign, is described. The myelographic demonstration of a large cord that subsequently decreases in size may suggest multiple sclerosis. Multiple sclerosis must be considered in the differential diagnosis of an enlarged spinal cord. Distinguishing between the collapsing cord and the contracting cord is discussed.
Key words: Myelography - Multiple sclerosis
Myelographic demonstration of a normal or small spinal cord with multiple sclerosis (MS) is well documented [7]. In this report a new radiographic sign of MS is described: an enlarged spinal cord that subsequently diminishes in diameter. The contracting cord of MS has been observed[ by others [8]. It is Probably a more specific although less common sign of MS than the atrophic spinal cord. The contracting cord must be distinguished from the collapsing cord sign of a spinal cord cyst.
Case Report A female, aged 31, first entered Milwaukee County General Hospital on 8 July 1975 complaining of severe headache, and tingling and weakness in the right arm and leg for 6 weeks. Past history included two episodes of transient blindness in her left eye during the previous year. Neurological examination on admission revealed a stiff neck, horizontal nystagmus with a fast component to the left, right facial weakness, generalized weakness of all extremities, diffusely hyperactive deep tendon reflexes and a Hoffman sign on the right. Computed tomography
(CT), radionuclide brain scan, skull and spine radiographs, and vertebral angiogram were normal. Lumbar puncture revealed normal cerebrospinal fluid under normal pressure. The presumptive diagnosis was a degenerative disease of the central nervous system. Treatment with ACTH was begun and the patient was discharged on the 27th hospital day. The patient was readmitted on 18 September 1975 because of marked respiratory insufficiency and weakness of the right arm and leg. Examination at this time revealed paradoxical movement of the abdomen with respiration, severe spastic paralysis of the right lower extremity, flaccid paralysis of the right upper extremity, loss of pain and temperature sensation on the left side below the level of C2. There was a Babinski sign on the fight and horizontal nystagmus. Paralysis of the diaphragm was confirmed with fluoroscopy. The patient was intubated. An emergency gas myelogram revealed enlargement of the cervical cord (Fig. 1). The cord did not collapse with changes in the patient's position. A vertebral arteriogram revealed no evidence of neovascularity or abnormal staining in the cervical cord. The patient remained on a respirator and received high doses of steroids. The respiratory capacity gradually improved and the patient was discharged on 26 October 1975. There were several subsequent hospital admissions, at each of which the patient had exacerbation of weakness and respiratory difficulty. A repeat gas myelogram was performed on 6 April 1976 approximately six months after the first gas myelogram. At this time the spinal cord was small (Fig. 2). There was no change in the dimensions of the cord with changes in patient position. The patient expired on 12 January 1978. At autopsy the spinal cord was atrophic. Pathological examination revealed multiple demyelinating lesions in the spinal cord, brain stem and optic nerves characteristic of MS (Fig. 3). 0028-3940/79/0017/0207/$01.00
l~g. 1 a and b. Polytomograms from first gas myelogram. Silhouette of the cervical cord (arrows) is large in lateral (a) and A P (b) projections
Fig. 2 a and b. Second gas myelogram. Decrease in size of cord (arrows) is evident (a, lateral, b AP projection; polytomographic sections)
V. M. Haughton et al.: The Contracting Cord Signof Multiple Sclerosis
209
Fig. 3. Photograph of transverse section of cervical cord which is small especiallyin AP diameter. Entire section shows demyelination.Onlyin one region (arrows) is myelin evident
Discussion The early pathological changes in a demyelinating lesion of MS are degeneration of the myelin sheath, edema and inflammatory and glial cell infiltration [1, 6]. E d e m a and reactive cell infiltration may be sufficient to enlarge the diameter of the cord on myelography as in our case. T o date no examples of an enlarged spinal cord have been reported with MS because patients infrequently have myelography during the acute stage of the illness. Possibly, edema or abnormal contrast enhancement in the spinal cord of demyelinating diseases may be detected by CT scanning in the future. When edema and infiltration are replaced by gliosis, the atrophic cord may be demonstrated myelographically [5, 7]. An enlarged spinal cord may suggest neoplasm or a cyst [2, 3, 4]. In a patient with an enlarged spinal cord and an atypical history for tumor, repeat myelography may be helpful to distinguish MS from tumor. Contraction of the cervical cord is atypical for tumor but probably characteristic of MS. In some cases, spinal cord angiography may be helpful to exclude neoplasm. T o exclude a spinal cord cyst as a cause of cord enlargement a two position gas myelogram should be performed [2]. The contracting cord sign should be distinguished from the collapsing cord sign. Changes in cord diameter caused by movement of fluid within a cyst of the spinal cord result in the collapsing cord sign. The collapsing cord sign is usually demonstrated during myelography by tilting the patient into different positions [2]. Spinal cord atrophy and reabsorption of
edema fluid are probably the cause of the contracting cord sign. Contraction of the cord is demonstrated, not by changing the patient's position during myelography, but by repeating the myelogram after several weeks. A cyst of the cord might simulate the contracting cord if myelograms, each with a different technique or contrast medium, were performed several weeks apart.
References 1. Dastur, D. K., Singhal, D. S.: Two unusual neuropathologically proven cases of multiple sclerosis from Bombay. J. of Neurol. Sci. 20, 397-414 (1973) 2. Haughton, V. M., Williams, A.L. Cusick, S.F., Meyer, G.: A myelographic technique for cysts in the spinal canal. Radiology 129, 717-719 (1978) 3. Williams, A.L., Haughton, V.M.: The present status of gas myelography: International advances in investigations of the spine. (ed. Judith Donovan Post) 1979 4. Klefenberg, G., Saltzman, G.: Gas myelographic studies in syfingomyelia. Acta Radiol. 52, 129-138 (1959) 5. McDonald, W. I.: Pathophysiologyin multiple sclerosis. Brain 97, 179-196 (1974) 6. Seitelberger, F.: Pathology of multiple sclerosis. Ann. Clin. Res, 5, 337-344 (1973) 7. Vakili, H.: The spinal cord. pp. 316-337. New York: Intercontinental 1967 8. Westberg, G.: Personal communication Received: 29 September 1978 Dr. V. M. Haughton Department of Radiology Milwaukee County Medical Complex 8700 West Wisconsin Avenue Milwaukee, WI 53226, USA
