Multiple Intramedullary Spinal Sarcoidosis: Case Report

Infection

Multiple Intramedullary Spinal Sarcoidosis: Case Report Shiro Chitoku, M.D.,* Shozo Kawai, M.D.* Yasuharu Watabe, M.D.,* Masaya Nishitani, M.D.,* Kenta Fujimoto, M.D.,* Hiroyuki Otsuka, M.D.,* Hiroaki Fushimi, M.D.,† and Kiyoshi Kotoh, M.D.† Departments of *Neurosurgery and †Pathology, Osaka Prefectural Hospital, Osaka, Japan

Chitoku S, Kawai S, Watabe Y, Nishitani M, Fujimoto K, Otsuka H, Fushimi H, Kotoh K. Multiple intramedullary spinal sarcoidosis: case report. Surg Neurol 1997:48:522– 6. BACKGROUND

According to past reported cases, spinal intramedullary sarcoidosis has the radiologic characteristics of a single enhanced lesion with cord swelling. However, it is difficult to make a diagnosis using only radiologic examinations. Including our case, 20 cases have been reported of intramedullary spinal sarcoidosis. Only six cases were diagnosed as primary spinal sarcoidosis. We present a rare case of multiple spinal intramedullary sarcoidosis without cord swelling. CASE DESCRIPTION

This 63 year-old man was afflicted with progressive paraparesis and numbness of the lower extremities. Magnetic resonance imaging showed intramedullary lesions without cord swelling. The patient underwent biopsy for diagnosis and the specimen showed a noncaseating granuloma mainly composed of epitheloid cells. Postoperative corticosteroid therapy was effective and the lesion disappeared 1 month after the operation. CONCLUSIONS

Biopsy is a less invasive method and is useful for early diagnosis. Early diagnosis is important for spinal sarcoidosis so that high-dose corticosteroid therapy can be commenced while the lesion still has a good chance of being successfully treated. © 1997 by Elsevier Science Inc. KEY WORDS

Sarcoidosis, spinal cord sarcoidosis, intramedullary lesion, corticosteroid therapy.

pinal neurosarcoidosis is a rare disease accounting for about 0.43% of all sarcoidosis [3]. Spinal sarcoidosis usually presents as a single lesion associated with cord swelling, and 70% of the patients also have signs of systemic sarcoidosis. Here we present multiple spinal sarcoidosis without cord swelling or systemic sarcoidosis. Because

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Address reprint requests to: Shiro Chitoku, M.D., Department of Neurosurgery, Osaka Prefectural Hospital, 3-1-56 Bandaihigashi, Sumiyoshi, Osaka, 558 Japan. Received December 11, 1996; accepted February 12, 1997. 0090-3019/97/$17.00 PII S0090-3019(97)00170-5

spinal sarcoidosis is difficult to differentiate preoperatively, surgical biopsy is recommended to allow a definite diagnosis and initiation of appropriate therapy.

Case Report This 63-year-old man had undergone myelography using myodil approximately 30 years ago. The patient was admitted to our hospital in October 1993 with a history of progressive paraparesis and numbness of both lower extremities over an 11month period. Neurologic examination revealed hypesthesia below T5, paraparesis, and urinary incontinence. There were no abnormalities on routine blood tests and the VDRL test was negative. Cervical X ray films showed cervical spondylosis, slight herniation at the level of C4/5, and myodil deposits in the cranium and spinal canal. Magnetic resonance imaging (MRI) demonstrated cervical and thoracic intramedullary lesions without cord swelling. The lesions were isointense on T1-weighted images, hyperintense on T2-weighted images, and showed weak enhancement with gadolinium (Gd-DTPA) (Figure 1). From the radiologic findings, we suspected myelitis, infection, or metastatic spinal tumor. Radioisotope scintigraphy, tumor marker studies, and ultrasonography of the abdomen showed no abnormalities. Because his symptoms were progressive, we performed a biopsy. Laminectomy was done from C6 –T1. The cord was almost normal in appearance. Biopsy was carried out after midline myelotomy at the C7 level. The lesion was soft and almost the same color as the spinal parenchyma. There was no capsule and the margins were unclear. Light microscopic examination showed a nonca© 1997 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010

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(A) Sagittal T2-weighted MRI. The intramedullary lesion is located at C6 –C7 level and is hyperintense with no cord swelling; (B) sagittal gadolinium enhanced MRI. The cervical intramedullary mass is homogeneously enhanced; (C) Sagittal gadolinium enhanced MRI. Same as the cervical lesion, the intramedullary enhanced lesion is located at T8 –T9 level.

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seating granuloma mainly composed of epitheloid cells but also containing lymphocytes (Figure 2). The patient was treated with a short course of high-dose corticosteroid therapy. After steroid therapy, his hypesthesia improved but his paraparesis was not changed. At 1 month after the operation, MRI showed total disappearance of the enhanced lesion (Figure 3).

Discussion Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Neurosarcoidosis ac-

Surgical specimen showed noncaseating granuloma mainly composed of epitheloid cells but also lymphocytes.

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counts for approximately 5% of all sarcoidosis [23] and often affects the leptomeninges, cranial nerves (facial nerve and optic nerve), and pituitary gland [4]. However, spinal intramedullary sarcoidosis is very rare; only 19 cases have been reported (this case is no. 20). Table 1 is a summary of the reported cases of intramedullary sarcoidosis confirmed by pathological examination. The age ranged from 24 – 68 years (mean: 48.3 years). There was female predominance, and most patients suffered from progressive paraparesis and paresthesia of the extremities. In about 80% of patients, the lesion was in the lower cervical region and the remainder were located in the lower thoracic portion. Table 2 is a summary of MRI findings. The lesions are isointense on T1-weighted images, hyperintense on T2-weighted images, and are enhanced by GdDTPA. Most lesions were accompanied by cord swelling, but some were not. Nonmass-forming intramedullary lesions of the spinal cord include sarcoidosis, systemic lupus erythematosus, multiple sclerosis, syphilis, fungal infections, tuberculosis, and radiation myelitis [11]. It is difficult to differentiate infectious disease and myelitis by radiologic examination [8,11,21], but biopsy is useful to obtain a pathologic diagnosis and determine the correct therapy. Most of the reported patients underwent biopsy or decompressive surgery to make a diagnosis and

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Postoperative MRI. The lesions have completely disappeared. (A) Sagittal gadolinium enhanced MRI of cervical portion; (B) sagittal gadolinium enhanced MRI of the thoracic portion.

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relieve the mass effect. High-dose steroid therapy is effective [14] for intramedullary spinal sarcoidosis. Because most of the lesions disappear after steroid therapy and some patients show worsening of their neurologic status after extensive surgical removal [1–3], partial resection is adequate, if the frozen section diagnosis suggests sarcoidosis. Although most of the patients showed improvement of symp-

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toms after steroid therapy, there were some patients with no neurologic change due to the complete damage of the cord. About 70% of the patients had signs of systemic sarcoidosis, so careful preoperative work-up is important. In our case, the neurologic symptoms did not resolve completely, although the lesions disap-

Reported Cases of Intramedullary Spinal Sarcoidosis

SIGN FOR SYSTEMIC NO.

AUTHOR

1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20.

Jefferson [7] Banerjee [1] Semins [20] Ravindra [17] Synder [22] Day [6] Bernstein [2] Kanoff [10] Riffel [18] Rubinstein [19] Hitchon [9] Vighetto [24] Kelly [12] Clifton [5] Levivier [15] Nishiura [16] Kayama [11] Kayama [11] Kinuta [19] Chitoku [present case]

YEAR AGE SEX 1957 1972 1972 1976 1976 1976 1978 1978 1982 1984 1984 1985 1988 1990 1991 1992 1993 1993 1994 1997

66 36 68 43 43 43 31 58 26 24 47 27 52 49 57 27 67 64 62 64

F F F F F M M F F F M M F M F F M F F M

PORTION

THERAPY

OUTCOME

SARCOIDOSIS

C3–C4 C5–C6 T7–T9 Lower C C4–C5 C5–C6 T1–T8 C3–C6 C5–C7 C3–C6 T5–T8 C5–T1 C4–C7 C6–T2 C3–C6 C5–C7 C3–C7 C3–C7 C5–C6 C6–T1, T8–T11

Biopsy Gross removal Gross removal No therapy Partial removal Extensive removal Biopsy Gross removal Biopsy Biopsy Gross removal Biopsy Biopsy Biopsy Biopsy Partial removal Subtotal removal Gross removal Gross removal Biopsy

Dead Improved Improved Dead(A) Dead(A) No change Not described Dead Not described No change No change Improved Improved Improved Improved Improved No change Improved No change Improved

Lung, liver Hilar adenopathy Erythema nodosum N Hilar adenopathy Lung, liver Liver N Liver Kveim test (1) N Lung N Hilar adenopathy N Lung, liver Not described Not described Tuberculin test (1) N

Abbreviations: F 5 female, M 5 male, C 5 cervical, T 5 thoracic, N 5 negative, A 5 autopsy.

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MRI Findings of Reported Intramedullary Spinal Sarcoidosis

NO.

AUTHOR

YEAR

T1

T2

Gd

CORD SWELLING

1. 2. 3. 4. 5. 6. 7. 8.

Kelly [12] Clifton [5] Levivier [15] Nishiura [16] Kayama [11] Kayama [11] Kinuta [13] Chitoku [present case]

1988 1990 1991 1992 1993 1993 1994 1996

Iso Iso ND ND Hyper Hyper Iso Iso

Hyper Hyper Hyper Hyper ND ND ND Hyper

1 1 1 1 1 1 1 1

1 1 1 1 1 1 1 2

Abbreviations: ND 5 not described, T1 5 T1-weighted image, T2 5 T2-weighted image, Gd 5 gadolinium enhancement, iso 5 iso intensity, hyper 5 hyper intensity.

peared on follow-up MRI. Accordingly, we recommend early diagnosis and initiation of high-dose steroid therapy while the symptoms are still reversible. REFERENCES 1. Banerjee T, Hunt WE. Spinal cord sarcoidosis. Case report. J Neurosurg 1972;36:490 –3. 2. Bernstein J, Rival J. Sarcoidosis of the spinal cord as the presenting manifestation of the disease. South Med J 1978;71:1571–3. 3. Bogousslavsky J, Hungerbuhler JP, Regli F, Garf HJ. Subacute myelopathy as the presenting manifestation of sarcoidosis. Acta Neurochir (Wien) 1982;65:193–7. 4. Chapelon C, Ziza JM, Piette JC, Levy Y, Raguin G, Wechsler B, Bitker MO, Bletry O, Laplane D, Bousser MG, Godeau P. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine 1990;69: 261–76. 5. Clifton AG, Stevens JM, Kapoor R. Spinal cord sarcoidosis with intramedullary cyst formation. Br J Radiol 1990;63:805– 8. 6. Day AL, Sypert GW. Spinal cord sarcoidosis. Ann Neurol 1976;1:79 – 85. 7. Jefferson M. Sarcoidosis of the nervous system. Brain 1957;80:540 –56. 8. Goy AMC, Pinto RS, Raghavendra BN, Epstein FJ, Kricheff II. Intramedullary spinal cord tumor. Radiology 1986;161:381– 6. 9. Hitchon PW, Haque AU, Olsen JJ, Jacobs SK, Olson SP. Sarcoidosis presenting as an intramedullary spinal cord mass. Neurosurgery 1984;15:86 –90. 10. Kanoff RB, Ruberg RL. Intramedullary sarcoidosis of the spinal cord. Report of a case. J Am Osteopath Assoc 1978;77:868 –73. 11. Kayama S, Kikuchi S. Intramedullary spinal cord sarcoidosis. Report of two cases. Spine 1993;18:2118 –20. 12. Kelly RB, Mahoney PD, Cowley KM. MR demonstration of spinal cord sarcoidosis. Report of a case AJNR 1988;9:197–9. 13. Kinuta Y, Isaka F, Uemura Y, Kikuchi H. Two cases of spinal sarcoidosis. Spinal Surg 1994;8:147–51. 14. Lexa FJ, Grossman RI. MR of sarcoidosis in head and spine: spectrum of manifestations and radiographic response to steroid therapy. AJNR 1994;15:973– 82. 15. Levivier M, Brotchi J, Baleriaux D, Pirotte B, Durand

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JF. Sarcoidosis presenting as an isolated intramedullary tumor. Neurosurgery 1991;29:271– 6. Nishiura I, Tochio H, Koyama T. Cervical intramedullary sarcoidosis. Neurochirurgia 1992;35:163– 6. Ravindra NMP, Chase PH, Elguezabel A, Weinstein M. Spinal cord sarcoidosis. NY State J Med 1976;76:748 – 52. Riffel B, Wietholtor H, Storh M, Thron A, Eggert R. Zur Sarkoidose des Ruckenmarks. Forschr. Neurol Psychiatr 1982;50:275– 8. Rubinstein I, Hess J, Baum GL. Intramedullary spinal cord sarcoidosis. Surg Neurol 1984;21:272– 4. Semins H, Nugent GR, Chou SM. Intramedullary spinal cord sarcoidosis. Case report. J. Neurosurg 1972;37: 233– 6. Scotti G, Scialfa G, Colombo N, Landoni L. Magnetic resonance diagnosis of intramedullary tumors of the spinal cord. Neuroradiology 1987;29:130 –5. Snyder R, Towfighi J, Gonatas NK. Sarcoidosis of the spinal cord. J Neurosurg 1976;44:740 –3. Stern BJ, Krmholz A, Johns CJ, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol Psychiatry 1985;42:909 –17. Vighetto A, Fischer G, Collet P, Bady B, Trillet M. Intramedullary sarcoidosis of the cervical spinal cord. J Neurol Neurosurg Psychiatr 1985;48:477–9.

COMMENTARY

The authors have presented a very interesting case of multiple intramedullary spinal cord sarcoidosis. This is a very rare disease, but it needs to be recognized and treated appropriately. The MRI findings are nonspecific, but should raise the index of suspicion that such a lesion is present, particularly given the minimal mass effect relative to the size of the MRI alterations. Unless the patient has disseminated sarcoidosis, one cannot assume with certainty that the intramedullary lesion is sarcoidosis. Early biopsy is appropriate so that the appropriate therapy, which is high-dose steroid therapy as recommended by the authors, is instituted early so that disease progression can be prevented and,

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hopefully, some of the myelopathic symptoms and signs reversed.

[2]. Steroid therapy may provide dramatic results, as we observed in one patient [1].

George W. Sypert, M.D. Neurosurgeon Fort Myers, Florida

Jacques Brotchi, M.D., Ph.D. Service de Neurochirurgie Universite Libre de Bruxelles Brussels, Belgium

Chitoku et al. present a rare case of multiple spinal intramedullary sarcoidosis without evidence of systemic sarcoidosis. About 20 cases have been previously reported, but all were isolated lesions and most showed a slightly enlarged spinal cord on MRI, which made the differential diagnosis difficult. In the case reported in this paper, tumor could be eliminated for two reasons: (1) the absence of spinal cord enlargement, and (2) the multiple locations. In our experience [2], we have never observed any spinal intramedullary tumor without spinal cord enlargement. In a patient with multiple lesions and a normal size cord, we first think of multiple sclerosis, even in a 63-year-old man, and we recommend a brain MRI before doing a biopsy. However, we must remember that in isolated spinal cord lesions without systemic sarcoidosis, MRI is helpful [3,6] but not pathognomic in making the diagnosis [4,5]. Histology is mandatory to enable one to offer the appropriate medical treatment to these patients

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