1990, The British Journal of Radiology, 63, 805-808 Case reports any flexion, extension or rotation involving the cervical spine. A severe flexion deformity was present. Similarly, there was limitation of abduction of both shoulders to about 70 or 80 degrees, and he has a flexion deformity in both elbows. Surprisingly, there was very minimal sclerodactylia in comparison to his skin changes elsewhere. There was no evidence of persistent or recurrent tumour. Investigations included a barium swallow examination, which could not be completed satisfactorily owing to regurgitation into the bronchial tree. His immunological profile was consistent with scleroderma, with ANA being highly positive, titre greater than 1 in 2560, with the immunofluorescent pattern being homogenous, nucleolar and speckled. The usual extractable nuclear antigens were not detected. Anti-DNA antibodies were not elevated. Skin biopsy from an unirradiated area was typical of scleroderma, but pulmonary function studies were more compatible with his pre-existing emphysema than with scleroderma. Marked air trapping improved by bronchodilators and a reduced carbon monoxide diffusion were noted. He was referred to a rheumatologist who recommended D-Penicillamine, 125 mgbd, increasing at monthly intervals by 125 mg to a maximum of 500-625 mg daily. Dietary advice was obtained, with some early weight gain. Subsequent follow-up at this stage has been too short to assess the utility of systemic therapy. Discussion The vascular and fibrotic changes of scleroderma are well documented, though their precise aetiology is poorly understood (Gilliland, 1988). Similarly, the late normal tissue effects of radiotherapy and their variations with such factors as total dose, fraction size,
volume are well known and documented (Hall, 1988). When occurring together, significant morbidity may develop secondary to dense subcutaneous and softtissue fibrosis. In this case, diffuse scleroderma manifested some 2 years after the completion of radical irradiation to the head and neck region. Whilst small areas received greater than 70 Gy, the bulk of the neck received only 50 Gy in 25 fractions. Nevertheless, major morbidity developed because of severe bilateral fibrosis involving all structures in the neck. Importantly, however, no evidence of tumour recurrence is to be found. Response to D-Penicillamine therapy will be monitored. This is only the second case report we have been able to identify regarding the adverse interaction between radiation therapy and scleroderma. Acknowledgment Mrs Kim Lusis is thanked for preparing the manuscript. References CALDWELL,
D.
S.
&
MCCALLUM,
R.
M.,
1986.
Rheumatological manifestations of cancer. Medical Clinics of North America, 70, 385-417. GILLILAND, B. C , 1988. Progressive systemic sclerosis (Diffuse scleroderma). In Harrisons Principles of Internal Medicine, 11th edn (McGraw Hill, Maidenhead), pp. 1428-1432. HALL, E. J., 1988. Radiobiology for the Radiobiologist, 3rd edn (J. B. Lippincott, Philadelphia), pp. 57-58; 239-261. RANSOM, D. T. & CAMERON, F. G.,
1987. Scleroderma—a
possible contraindication to lumpectomy and radiotherapy in breast carcinoma. Australasian Radiology, 31, 317-318.
Spinal cord sarcoidosis with intramedullary cyst formation By A. G. Clifton, MA, MRCP, FRCR, J. M. Stevens, DRACR, FRCR, *R. Kapoor, MA, MRCP and *P. Rudge, FRCP Departments of Radiology and *Neurology, The National Hospitals for Nervous Diseases, Maida Vale, London W9 (Received November 1989 and in revised form February 1990)
We report a case of spinal cord sarcoidosis with surgically confirmed localized cyst formation and probably syringomyelia involving almost the entire spinal cord, an association which has not been documented previously. The magnetic resonance (MR) findings before and after gadolinium diethylenetriamine-pentaacetic acid (Gd-DTPA) are described. There has been only one previous report of the MR findings in histologically confirmed spinal cord sarcoidosis, and in this case Gd-DTPA was not given. Case report A 49-year-old man presented with a 3-week history of progressive urinary hesitancy and weakness of both legs. Three Vol. 63, No. 754
years previously he was diagnosed as having sarcoidosis on the basis of bilateral hilar adenopathy on plain chest radiography, and a positive Kveim test. Ten months before this admission he had developed granulomatous uveitis. Clinical findings On initial examination there was moderate reduction of muscle power in all four limbs, whilst tone and co-ordination were normal. Reflexes were generally brisk, abdominal reflexes were absent, and there were bilateral extensor plantar responses. Vibration and joint position sensation were absent bilaterally in his feet, and there was a spinothalamic sensory level at C8 on the right and D6 on the left. Routine blood tests were normal. The erythrocyte sedimentation rate was 70 mm/h. Serum angiotensin converting enzyme
805
Case reports activity was 15 iu/1. At lumbar puncture, the cerebrospinal fluid (CSF) pressure was 140 mm and the fluid was xanthochromic; the CSF protein concentration was 14 g/1 and glucose 2.1mmols (blood glucose 8mmols/l). There were 21 mature lymphocytes and one red blood cell per cubic mm. Oligoclonal studies were negative.
Magnetic resonance imaging (Fig. 3): Tt and T2 weighted images showed expansion of the spinal cord from the medulla oblongata down to the tenth thoracic vertebra, with maximum expansion from about C6 to the lower border of Tl. On T] weighted images the region of maximum expansion showed
Radiology Imaging revealed abnormalities on the following tests. Myelogram (Fig. 1): marked expansion of the spinal cord was shown from the foramen magnum to the third thoracic vertebra. Post-myelography computed tomography (CT) (Fig. 2): immediate images showed symmetrical expansion of the spinal cord from the foramen magnum to the upper thoracic region, maximal in the cervical region. Delayed CT 20 h after the myelogram showed pathological accumulation of contrast medium in the spinal cord from the level of the third to the 10th thoracic vertebrae, but not in the cervical region. The spinal cord was slightly expanded throughout the thoracic region.
Figure 1. Conventional myelography using water soluble contrast medium. Contrast medium was introduced by lumbar puncture. The expanded cervical cord is shown in the anteroposterior and lateral projections. Note the mild undulation of the lateral margins of the spinal cord in the upper thoracic and lower cervical regions.
806
Figure 2. (a) Computed myelography showing a series of 5 mm thick axial sections through the mid-cervical region immediately after the myelogram. The spinal cord is symmetrically expanded, (b) A series of axial 5 mm thick CT sections through the thoracic region made 20 h after the myelogram, showing a conspicuous accumulation of contrast medium in the central part of a mildly expanded spinal cord.
The British Journal of Radiology, October 1990
Case reports
Figure 3. (a) Mid-sagittal T, weighted images of the cervical spine and adjacent regions (TR/TE 500/26). (b) The same scanning parameters as in (a) but after intravenous Gd-DPTA. (c) T2 weighted mid-sagittal image of the cervical spine and adjacent regions (TR/TE 1500/80). Vol. 63, No. 754
807
Case reports diffusely increased signal in which were located two circumscribed areas of low signal. Extended both cranially and caudally from this region were central areas of slightly reduced signal, suggestive of syringomyelia, reaching at least as high as C2 and to T10 below. After intravenous Gd-DTPA, the relatively hyperdense areas in the region of maximum expansion showed well circumscribed enhancement. On T2 weighted images there was diffusely increased signal from the level of the foramen magnum down to the upper thoracic region. In the region of maximum expansion, signal was inhomogenous, and with some irregular, poorly circumscribed areas of reduced signal, not definitely indicating cystic change. The MR study shows mild cerebellar ectopia, but on the computed myelogram using overlapping 5 mm thick sections there was no evidence of cerebellar ectopia. We conclude that probably there was minimal and almost certainly insignificant cerebellar ectopia.
Clinical management The patient was treated with Dexamethasone 4 m g q.d.s, prior to operation which consisted of laminectomy between C5 and T l . Intramedullary cysts containing clear xanthochromic fluid were incised at C5 and at C7 to T l . Biopsy of abnormal solid material revealed, on histological examination, intense astrocytic gliosis surrounding non-caseating granulomas composed of centrally placed epithelioid cells and a peripheral rim of lymphocytes, consistent with sarcoidosis. There were a few multinucleated giant cells, but neither caseation nor acid fast bacilli were seen. Subsequent cultures of this material and of the CSF were also negative for acid fast bacilli. There was considerable clinical improvement, with return of normal bladder function. A mild sensory type ataxia, right leg weakness and impaired position sensation in the feet persisted. Treatment with oral steroids was continued.
Miller et al, 1988), but our literature review has revealed only one biopsy proven case with spinal cord involvement (Kelly et al, 1988), which did not include imaging after intravenous injection of Gd-DPTA. This case demonstrated expansion of the cervical cord, and diffuse signal increase on T2 weighted images extending from the medulla oblongata into the upper thoracic cord. Pathologically, central nervous system involvement is in the form of either sarcoid tissue in meninges or nervous tissue, or infarction secondary to occlusion of small vessels by granulomata (Herring et al, 1969). On MRI both appear as increase in TK and T2 relaxation times (Miller et al, 1988). The enhancement characteristics of intramedullary disease after Gd-DTPA are similar to those in disease of the brain, enhancement depending on the presence of an intact blood supply and the state of the physiological barriers that regulate the passage of contrast medium into the extracellular space (Sze et al, 1989). In this case enhancement assisted in establishing the presence of pathological solid elements in the spinal cord. Cystic change was confirmed surgically in the lower cervical region. The more extensive involvement of the spinal cord in the form of circumscribed central persistent contrast accumulation on delayed post-myelography CT and signal changes on MRI were most likely to represent syringomyelia but could conceivably have represented only central necrosis (myelomalacia). The presence of mild expansion of the affected region of the spinal cord on CT, however, makes the presence of syringomyelia almost certain (Williams et al, 1987).
Discussion
Sarcoidosis is a common idiopathic granulomatous disease which may involve any organ system, resulting in varied manifestations. The overall frequency of clinical neurological involvement is 5% (Delaney, 1977). Although patients usually have other stigmata of the disease, neurological dysfunction can be the presenting feature. Involvement of the spinal cord is much less frequent than the brain and peripheral nerves. Hitchon et al (1984) described only 24 cases of histologically confirmed sarcoidosis documented in the literature up to 1984. Our search of the literature has revealed no case with a syrinx. An intramedullary mass lesion is the most common manifestation; however arachnoiditis and intradural-extramedullary mass lesions have also been described (Kelly et al, 1988). Biopsy is usually needed for diagnosis because the myelographic appearance of an expanded spinal cord is non-specific; and may be seen with, for example, intramedullary neoplasms, syringomyelia, multiple sclerosis and other inflammatory lesions. Operative diagnosis is necessary and may permit a useful decompression as in this case. Subsequent treatment with systemic steroid has been shown to be beneficial in spinal cord sarcoidosis (Hitchon et al, 1984). The MR findings of spinal cord sarcoidosis are poorly documented. There have been several reports in intracerebral sarcoidosis (Poole, 1984; Ketonen et al, 1987;
References DELANEY, P., 1977. Neurologic manifestations in sarcoidosis. Annals of Internal Medicine, 87, 336-346. HERRING, A. B. & URICH, H., 1969. Sarcoidosis of the central
nervous system. Journal of Neurological Sciences, 9, 405-422. HITCHON, P. W., U L HAQUE, A., OLSON, J. J., JACOBS, S. K. &
OLSON, S. P., 1984. Saracoidosis presenting as an intramedullary spinal cord mass. Neurosurgery, 15, 86-90. KELLY, R. B., MAHONEY, P. D. & CAWLEY, K. M., 1988. MR
demonstration of spinal cord sarcoidosis: report of a case. American Journal of Neuroradiology, 9, 197-199. KETONEN, L., OKSANEN, V. & KUVLIALA, I., 1987. Preliminary
experience of magnetic resonance imaging neurosarcoidosis. Neuroradiology, 29, 127-129.
in
MILLER, D. H., KENDALL, B. E., BARTER, S., JOHNSON, G., MACMANUS, D. G., LOGSDUIL, S. J., ORMEROD, I. E. C. &
MCDONALD, W. I., 1988. Magnetic resonance imaging in central nervous system sarcoidosis. Neurology, 38, 378-383. POOLE, C. J. M., 1984. Argyll Robertson pupils due to neurosarcoidosis, evidence for site of lesions. British Medical Journal, 289, 356. SZE, G., BRAVO, S. & KROL, G., 1989. Spinal lesions:
Quantitative and qualitative temporal evolution of gadopentate dimeglumine enhancement in MR imaging. Radiology, 170, 849-856. WILLIAMS, A. L., HAUGHTON, V. M. & POJUNAS, K. W., 1987.
Differentiation of intramedullary neoplasms and cysts by MR. American Journal of Neuroradiology, 8, 527-523.
The British Journal of Radiology, October 1990
volume are well known and documented (Hall, 1988). When occurring together, significant morbidity may develop secondary to dense subcutaneous and softtissue fibrosis. In this case, diffuse scleroderma manifested some 2 years after the completion of radical irradiation to the head and neck region. Whilst small areas received greater than 70 Gy, the bulk of the neck received only 50 Gy in 25 fractions. Nevertheless, major morbidity developed because of severe bilateral fibrosis involving all structures in the neck. Importantly, however, no evidence of tumour recurrence is to be found. Response to D-Penicillamine therapy will be monitored. This is only the second case report we have been able to identify regarding the adverse interaction between radiation therapy and scleroderma. Acknowledgment Mrs Kim Lusis is thanked for preparing the manuscript. References CALDWELL,
D.
S.
&
MCCALLUM,
R.
M.,
1986.
Rheumatological manifestations of cancer. Medical Clinics of North America, 70, 385-417. GILLILAND, B. C , 1988. Progressive systemic sclerosis (Diffuse scleroderma). In Harrisons Principles of Internal Medicine, 11th edn (McGraw Hill, Maidenhead), pp. 1428-1432. HALL, E. J., 1988. Radiobiology for the Radiobiologist, 3rd edn (J. B. Lippincott, Philadelphia), pp. 57-58; 239-261. RANSOM, D. T. & CAMERON, F. G.,
1987. Scleroderma—a
possible contraindication to lumpectomy and radiotherapy in breast carcinoma. Australasian Radiology, 31, 317-318.
Spinal cord sarcoidosis with intramedullary cyst formation By A. G. Clifton, MA, MRCP, FRCR, J. M. Stevens, DRACR, FRCR, *R. Kapoor, MA, MRCP and *P. Rudge, FRCP Departments of Radiology and *Neurology, The National Hospitals for Nervous Diseases, Maida Vale, London W9 (Received November 1989 and in revised form February 1990)
We report a case of spinal cord sarcoidosis with surgically confirmed localized cyst formation and probably syringomyelia involving almost the entire spinal cord, an association which has not been documented previously. The magnetic resonance (MR) findings before and after gadolinium diethylenetriamine-pentaacetic acid (Gd-DTPA) are described. There has been only one previous report of the MR findings in histologically confirmed spinal cord sarcoidosis, and in this case Gd-DTPA was not given. Case report A 49-year-old man presented with a 3-week history of progressive urinary hesitancy and weakness of both legs. Three Vol. 63, No. 754
years previously he was diagnosed as having sarcoidosis on the basis of bilateral hilar adenopathy on plain chest radiography, and a positive Kveim test. Ten months before this admission he had developed granulomatous uveitis. Clinical findings On initial examination there was moderate reduction of muscle power in all four limbs, whilst tone and co-ordination were normal. Reflexes were generally brisk, abdominal reflexes were absent, and there were bilateral extensor plantar responses. Vibration and joint position sensation were absent bilaterally in his feet, and there was a spinothalamic sensory level at C8 on the right and D6 on the left. Routine blood tests were normal. The erythrocyte sedimentation rate was 70 mm/h. Serum angiotensin converting enzyme
805
Case reports activity was 15 iu/1. At lumbar puncture, the cerebrospinal fluid (CSF) pressure was 140 mm and the fluid was xanthochromic; the CSF protein concentration was 14 g/1 and glucose 2.1mmols (blood glucose 8mmols/l). There were 21 mature lymphocytes and one red blood cell per cubic mm. Oligoclonal studies were negative.
Magnetic resonance imaging (Fig. 3): Tt and T2 weighted images showed expansion of the spinal cord from the medulla oblongata down to the tenth thoracic vertebra, with maximum expansion from about C6 to the lower border of Tl. On T] weighted images the region of maximum expansion showed
Radiology Imaging revealed abnormalities on the following tests. Myelogram (Fig. 1): marked expansion of the spinal cord was shown from the foramen magnum to the third thoracic vertebra. Post-myelography computed tomography (CT) (Fig. 2): immediate images showed symmetrical expansion of the spinal cord from the foramen magnum to the upper thoracic region, maximal in the cervical region. Delayed CT 20 h after the myelogram showed pathological accumulation of contrast medium in the spinal cord from the level of the third to the 10th thoracic vertebrae, but not in the cervical region. The spinal cord was slightly expanded throughout the thoracic region.
Figure 1. Conventional myelography using water soluble contrast medium. Contrast medium was introduced by lumbar puncture. The expanded cervical cord is shown in the anteroposterior and lateral projections. Note the mild undulation of the lateral margins of the spinal cord in the upper thoracic and lower cervical regions.
806
Figure 2. (a) Computed myelography showing a series of 5 mm thick axial sections through the mid-cervical region immediately after the myelogram. The spinal cord is symmetrically expanded, (b) A series of axial 5 mm thick CT sections through the thoracic region made 20 h after the myelogram, showing a conspicuous accumulation of contrast medium in the central part of a mildly expanded spinal cord.
The British Journal of Radiology, October 1990
Case reports
Figure 3. (a) Mid-sagittal T, weighted images of the cervical spine and adjacent regions (TR/TE 500/26). (b) The same scanning parameters as in (a) but after intravenous Gd-DPTA. (c) T2 weighted mid-sagittal image of the cervical spine and adjacent regions (TR/TE 1500/80). Vol. 63, No. 754
807
Case reports diffusely increased signal in which were located two circumscribed areas of low signal. Extended both cranially and caudally from this region were central areas of slightly reduced signal, suggestive of syringomyelia, reaching at least as high as C2 and to T10 below. After intravenous Gd-DTPA, the relatively hyperdense areas in the region of maximum expansion showed well circumscribed enhancement. On T2 weighted images there was diffusely increased signal from the level of the foramen magnum down to the upper thoracic region. In the region of maximum expansion, signal was inhomogenous, and with some irregular, poorly circumscribed areas of reduced signal, not definitely indicating cystic change. The MR study shows mild cerebellar ectopia, but on the computed myelogram using overlapping 5 mm thick sections there was no evidence of cerebellar ectopia. We conclude that probably there was minimal and almost certainly insignificant cerebellar ectopia.
Clinical management The patient was treated with Dexamethasone 4 m g q.d.s, prior to operation which consisted of laminectomy between C5 and T l . Intramedullary cysts containing clear xanthochromic fluid were incised at C5 and at C7 to T l . Biopsy of abnormal solid material revealed, on histological examination, intense astrocytic gliosis surrounding non-caseating granulomas composed of centrally placed epithelioid cells and a peripheral rim of lymphocytes, consistent with sarcoidosis. There were a few multinucleated giant cells, but neither caseation nor acid fast bacilli were seen. Subsequent cultures of this material and of the CSF were also negative for acid fast bacilli. There was considerable clinical improvement, with return of normal bladder function. A mild sensory type ataxia, right leg weakness and impaired position sensation in the feet persisted. Treatment with oral steroids was continued.
Miller et al, 1988), but our literature review has revealed only one biopsy proven case with spinal cord involvement (Kelly et al, 1988), which did not include imaging after intravenous injection of Gd-DPTA. This case demonstrated expansion of the cervical cord, and diffuse signal increase on T2 weighted images extending from the medulla oblongata into the upper thoracic cord. Pathologically, central nervous system involvement is in the form of either sarcoid tissue in meninges or nervous tissue, or infarction secondary to occlusion of small vessels by granulomata (Herring et al, 1969). On MRI both appear as increase in TK and T2 relaxation times (Miller et al, 1988). The enhancement characteristics of intramedullary disease after Gd-DTPA are similar to those in disease of the brain, enhancement depending on the presence of an intact blood supply and the state of the physiological barriers that regulate the passage of contrast medium into the extracellular space (Sze et al, 1989). In this case enhancement assisted in establishing the presence of pathological solid elements in the spinal cord. Cystic change was confirmed surgically in the lower cervical region. The more extensive involvement of the spinal cord in the form of circumscribed central persistent contrast accumulation on delayed post-myelography CT and signal changes on MRI were most likely to represent syringomyelia but could conceivably have represented only central necrosis (myelomalacia). The presence of mild expansion of the affected region of the spinal cord on CT, however, makes the presence of syringomyelia almost certain (Williams et al, 1987).
Discussion
Sarcoidosis is a common idiopathic granulomatous disease which may involve any organ system, resulting in varied manifestations. The overall frequency of clinical neurological involvement is 5% (Delaney, 1977). Although patients usually have other stigmata of the disease, neurological dysfunction can be the presenting feature. Involvement of the spinal cord is much less frequent than the brain and peripheral nerves. Hitchon et al (1984) described only 24 cases of histologically confirmed sarcoidosis documented in the literature up to 1984. Our search of the literature has revealed no case with a syrinx. An intramedullary mass lesion is the most common manifestation; however arachnoiditis and intradural-extramedullary mass lesions have also been described (Kelly et al, 1988). Biopsy is usually needed for diagnosis because the myelographic appearance of an expanded spinal cord is non-specific; and may be seen with, for example, intramedullary neoplasms, syringomyelia, multiple sclerosis and other inflammatory lesions. Operative diagnosis is necessary and may permit a useful decompression as in this case. Subsequent treatment with systemic steroid has been shown to be beneficial in spinal cord sarcoidosis (Hitchon et al, 1984). The MR findings of spinal cord sarcoidosis are poorly documented. There have been several reports in intracerebral sarcoidosis (Poole, 1984; Ketonen et al, 1987;
References DELANEY, P., 1977. Neurologic manifestations in sarcoidosis. Annals of Internal Medicine, 87, 336-346. HERRING, A. B. & URICH, H., 1969. Sarcoidosis of the central
nervous system. Journal of Neurological Sciences, 9, 405-422. HITCHON, P. W., U L HAQUE, A., OLSON, J. J., JACOBS, S. K. &
OLSON, S. P., 1984. Saracoidosis presenting as an intramedullary spinal cord mass. Neurosurgery, 15, 86-90. KELLY, R. B., MAHONEY, P. D. & CAWLEY, K. M., 1988. MR
demonstration of spinal cord sarcoidosis: report of a case. American Journal of Neuroradiology, 9, 197-199. KETONEN, L., OKSANEN, V. & KUVLIALA, I., 1987. Preliminary
experience of magnetic resonance imaging neurosarcoidosis. Neuroradiology, 29, 127-129.
in
MILLER, D. H., KENDALL, B. E., BARTER, S., JOHNSON, G., MACMANUS, D. G., LOGSDUIL, S. J., ORMEROD, I. E. C. &
MCDONALD, W. I., 1988. Magnetic resonance imaging in central nervous system sarcoidosis. Neurology, 38, 378-383. POOLE, C. J. M., 1984. Argyll Robertson pupils due to neurosarcoidosis, evidence for site of lesions. British Medical Journal, 289, 356. SZE, G., BRAVO, S. & KROL, G., 1989. Spinal lesions:
Quantitative and qualitative temporal evolution of gadopentate dimeglumine enhancement in MR imaging. Radiology, 170, 849-856. WILLIAMS, A. L., HAUGHTON, V. M. & POJUNAS, K. W., 1987.
Differentiation of intramedullary neoplasms and cysts by MR. American Journal of Neuroradiology, 8, 527-523.
The British Journal of Radiology, October 1990
