Surg Neurol 1992;38:309-14
309
Multiple Spinal Neurinomas Presenting Visual Disturbance as the Initial Symptom: Case Report Susumu Oikawa, M.D., Kazuhiko Kyoshima, M.D., Toshiki Takemae, M.D., and Shigeaki Kobayashi, M.D. Department of Neurosurgery, Shinshu University School of Medicine, Asahi 3-1-1, Matsumoto 390, Japan
Oikawa S, Kyoshima K, Takemae T, Kobayashi S. Multiple spinal neurinomas presenting visual disturbance as the initial symptom: case report. Surg Neurol 1992;38:309-14.
A case of multiple spinal neurinomas with visual disturbance is reported. A 63-year-old man was admitted with a complaint of progressive visual disturbance due to papilledema without spinal symptoms and signs. The neuroimaging studies demonstrated communicating hydrocephalus and two mass lesions in the cauda equina. Both tumors were found to be neurinomas. Intracranial hypertension secondary to spinal tumors is unusual, and multiple spinal neurinomas are rare. In the patient without spinal symptoms and signs, it is difficult to make a diagnosis of spinal tumor. Importance of checking for a spinal cord lesion by magnetic resonance imaging in such a case is stressed.
at a bright object. H e visited the Ophthalmology Service of Shinshu University Hospital on March 14, 1990, and was found to have bilateral papilledema o f an advanced stage. Visual acuity was 20/40 in the right and 20/50 in the left. A computed tomography (CT) scan and an MRI of the head disclosed no intracranial mass lesion. Mild enlargement of lateral ventricles and bilateral sylvian fissures and empty seUa were observed (Figure 1). His visual disturbance progressed to 20/130 in the right eye and 20/250 in the left during the following two weeks. A lumbar puncture revealed that the cerebrospinal fluid (CSF) pressure was 420 mm H 2 0 . Immediate surgery was advised but was not accepted by the patient. H e was readmitted to our service for surgery on April 6, 1990. H e had had no symptoms of intracranial hypertension or o f a spinal lesion.
K E Y WORDS: Multiple spinal neurinomas; Papilledema; Intracranial hypertension; Magnetic resonance imaging
Examination Intracranial hypertension or hydrocephalus secondary to spinal tumor is well known but unusual [ 1 - 8 , 1 2 , 1 7 - 2 1 , 2 3 - 2 5 , 2 8 - 3 1 ] . In a case with no symptoms and signs o f a spinal lesion except visual disturbance and papilledema, diagnosis o f spinal tumor is likely to be missed. A rare case with multiple spinal neurinomas presenting with visual disturbance as the initial symptom is reported. Difficulty in diagnosis and usefulness of magnetic resonance imaging (MRI) are discussed. Case Report A 63-year-old man without any previous illness or familial history o f note was referred to our service with a chief complaint o f progressive bilateral visual disturbance. H e noticed blurred vision in February 1990, when looking Address reprint requests to: Susumu Oikawa, M.D., Department of Neurosurgery, Shinshu University School of Medicine, Asahi 3-1-1, Matsumoto 390, Japan. Received February 11, 1992; accepted June 12, 1992.
© 1992 by Elsevier Science Publishing Co., Inc.
O n admission, the neurological examination revealed no abnormal findings except bilateral visual disturbance and papilledema. Mental deterioration, gait disturbance, and urinary dysfunction were not observed. Neither motor nor sensory disturbance was recognized. Deep tendon reflexes were normal. Analysis of the CSF revealed xanthochromia, a high protein concentration (235 mg/dL), a high-normal sugar level (73 mg/dL), and normal cell counts. CT cisternography disclosed ventricular reflux 6 hours after infusion o f iotrolan, and retention o f the contrast medium in the ventricles over 48 hours. Intravenous digital subtraction angiography (IVDSA) revealed no abnormal findings. Sinus or venous thrombosis was not identified. These findings on CT cisternography and IVDSA supported the diagnosis o f communicating hydrocephalus unrelated to vascular diseases. A ventriculoperitoneal (VP) shunt was done on April 8, 1990. The spinal MRI taken after the VP shunt disclosed two enhanced mass lesions (Figure 2). One was approximately 3 cm in its major axis, with a clear margin, and occupied the intradural space at the L 5 - $ 1 level. The other mass was round, 4 mm in 0090-3019/92/$5,00
310
Surg Neurol 1992;38:309-14
Oikawa et al
A
Figure 1. Cerebral C T and MRI scans with contrast material showing absence of mass lesion. (A) A C T scan showing mild enlargement of the bilaterial ventricles and the bilateral sylvian fissures. (B) A coronal MR1 showing empty sella.
Figure 2. MRI scans of lumbosacral area. (A) Sagittal Tl-weighted image disclosing a small, round isointense mass at L 1 - 2 levd (single arrow) and a large isointense mass at L5-S1 level (paired arrows). (B) Gadolinium-enhanced MRI showing homogeneous enhancement of both masses.
A
B
Multiple Spinal Neurinomas
Surg Neurol 1992;38:309-14
311
A C
Figure 3. Myelogram and C T myelogram. (A) Myelogram demonstrates a filling defect at L5-S1 level, but fails to show a small mass at L 1 - 2 level. (13) C T myelogram discloses a small mass at L 1 - 2 level as a low-density spot. (C) Nerve roots (arrows) around the large mass at LS-S1 level are recognized with C T myelography.
diameter, and located at the L I - 2 level, just below the conus medullaris. Myelography demonstrated a large filling defect at the L 5 - $ 1 level but failed to reveal the small mass at the L 1 - 2 level. Correlation between the large mass and nerve roots was not clear on myelography. CT myelography revealed that the small mass was a round, hypodense lesion, and the cauda equina was compressed dorsally by the large mass at the L 5 - $ 1 level (Figure 3).
Operation
U n d e r the diagnosis of e p e n d y m o m a or neurinoma, surgery was performed on July 2, 1990. The patient was
placed in a prone position, and two midline linear skin incisions were made in the upper lumbar and lumbosacral areas. Laminectomies were perfo~rmed separately at L1 and L 5 - $ 1 . In the upper wound, a yellowish 1-cm round tumor was found in the right side o f the dural opening. It had incorporated a fine nerve rootlet, which was resected with the tumor. The dura of the lower wound was opened. The tumor was immediately found, covered by the cauda equina. By electric stimulation, the root involved in the tumor was confirmed to be a sensory nerve. Although an attempt was made at preserving the sensory root, it was finally resected for the sake of total removal. Both tumors were completely resected. Histological diagnosis of both tumors was neurinoma (Figure 4). Postoperatively, no further neurological deficits, including sensory disturbance, were detected. Four months after the VP shunt, the patient's visual acuity improved to 20/60 in the right eye and 20/200 in the left, and bilateral papilledema disappeared; however, optic atrophy of both eyes was evident on fundoscopy.
312
Surg Neurol 1992;38:309-14
Oikawa et al
Figure 4. Photomicrographs of surgical specimen showing typical neurinomas. (A) Large tumor at L5-S1 level. (B) Small tumor at L1-2 level. Both specimens reveal spindle-shaped cells arranged in interlacing fascicles and slight myxoid degeneration. (Hematoxylin and eosin stain, original magnification × 80.)
A
B
Discussion In this case o f multiple spinal neurinomas, three interesting problems were mentioned: One was that the onset was visual disturbance; second was multiplicity o f spinal neurinoma not associated with skin lesions or particular familial history, as is characteristic o f von Recklinghausen's disease; and third was that the patient did not complain of headache or spinal symptoms.
Intracranial hypertension or hydrocephalus caused by spinal tumors is relatively rare [1]. Thirty-four cases of spinal tumors with intracranial hypertension or hydrocephalus have been reported in the literature with detailed description [ 1 - 8 , 1 2 , 1 7 - 2 1 , 2 3 - 2 5 , 2 8 , 2 9 , 3 1 ] . The onset was visual disturbance in six cases [3,6,8,24,28,29], o f which only two [8,29] had no spinal symptoms and signs. One o f the two cases was diagnosed
Multiple Spinal N e u r i n o m a s
as spinal tumor 3 months after admission, when the patient started to complain of pain in the lower back and right leg [29]. In a patient without spinal symptoms or signs, it is difficult to make a diagnosis of spinal tumor. The pathogenesis of intracranial hypertension or hydrocephalus with spinal tumors remains unknown. One of the most widely accepted mechanisms is impaired absorption of the CSF. The causes for the malabsorption of the CSF include tumor infiltration into the basal cistern [20], subarachnoid hemorrhage from tumor [29], tumor compressing the venous plexus [7], and raised CSF protein [6,19]. In 39 cases of spinal tumor with C SF analysis [ 1 - 8 , 1 2 , 1 7 - 2 1 , 2 3 - 2 5 , 2 8 - 3 1 ] , 36 cases (92%) showed high CSF protein. On the other hand, several cases of GuiUian-Barr6 syndrome with high CSF protein presented with signs of intracranial hypertension [13,22]. High CSF protein in a case of spinal tumor may be mostly responsible for obstruction of the CSF outflow. Gibberd et al [6] have suggested that increased immunoglobulin in the CSF due to the spinal tumor impairs absorption of the CSF in the arachnoid villi. However, at least three spinal cord tumors with normal CSF protein have been reported [8,29,30]. Therefore, mechanisms of intracranial hypertension or hydrocephalus may be multifactorial. CSF analysis may be useful for diagnosis. In a patient with high CSF protein, spinal tumor should not be ruled out from the differential diagnosis. MRI is a noninvasive and useful examination for detecting spinal lesions without bone artifact and can detect a small lesion that conventional or CT myelogram may fail to disclose. Spinal MRI should be performed for all cases of communicating hydrocephalus of unknown origin. Most common tumors of the cauda equina are ependymoma and neurinoma [10,14]. Both tumors were found to be a cause for intracranial hypertension and/or hydrocephalus [12,19,23,31]. Ependymoma, especially myxopapillary ependymoma, of the cauda equina often metastasizes to the subarachnoid space and is recognized as multiple tumors. Multiple spinal neurinomas are relatively rare. They have been found in some cases of von Recklinghausen's disease. The presence of "forme fruste" of central neurofibromatosis was proposed, and multiple spinal neurinomas without skin lesions might be regarded as some type of von Recklinghausen's disease [1 i]. This hypothesis is disputable; multiple spinal neurinomas without skin lesions and/or remarkable familial history is rare. Only four cases are reported in the literature [9,15,16,27]. Spinal neurinoma can present in many forms. MRI is regarded as a useful screening tool for detecting multiple spinal tumors. Surgical resection under the operating microscope is considered the sole treatment for spinal neurinoma.
Surg N e u r o l 1992;38:309-14
313
References 1. Arseni C, Maretsis M. Tumors of the lower spinal cord associated with increased intracranial pressure and papilloedema. J Neurosurg 1967;27:105-10. 2. Bamford CR, Labadie EL. Reversal of dementia in normotensive hydrocephalus after removal ofa cauda equina tumor.J Neurosurg 1967;45:104-7. 3. Bcrgesen SE, Scrensen SC, Olsen J. Gjerris F. Spinal tumours associated with increased intracranial pressure. Acta Neurol Scand 1977;56:263-8. 4. Feldmann E, Bromfield E, Navia B, Pasternak GW, Posner JB. Hydrocephalic dementia and spinal cord tumor. Arch Neurol 1986;43:714-8. 5. Gelabert M, Bollar A, Paseiro MJ, Allut AG. Hydrocephalus and intraspinal tumor in children. Childs Nerv Syst 1990;6:110-2. 6. Gibberd FB, Ngan H, Swann GF. Hydrocephalus, subarachnoid haemorrhage and ependymomas of the cauda equina. Clin Radiol 1972;23:422-6. 7. Glasauer F. Throacic and lumbar intraspinal tumors associated with increased intracranial pressure. J Neurol Neurosurg Psychiatry 1964;27:451-8. 8. Hansen K, Gjerris F, S0rensen S. Absence of hydrocephalus in spite of impaired cerebrospinal fluid absorption and severe intracranial hypertension. Acta Neurochir (Wien) 1987;86:93-7. 9. Hida K, Akino M, Isu T, Iwasaki Y, Abe H, Saito H. Multiple neurinoma of the spinal cord: case report. No Shinkei Geka 1988;16:1489-93. 10. HogenEsch RI, Staal MJ. Tumors of the cauda equina: the importance of an early diagnosis. Clin Neurol Neurosurg 1988; 90:343-8. 11. Hori A. 13ber intraspinale Schwannosen der Zona terminalis (Lissauer). (Formes fruste der Neurofibromatose Recklinghausen oder "reaktiv"?). Acta Neuropathol (Berl) 1973;25:89-94. 12. lob I, Andrioli GC, Rigobello L, Salar G. An unusual onset of a spinal cord tumour: subarachnoid bleeding and papilloedema. Neurochirurgia (Stuttg) 1980;23:112-6. 13. Joynt RJ. Mechanism of production of papilloedema in the Guillain-Barr6 syndrome. Neurology 1958;8:8-12. 14. Ker NB, Jones CB. Tumors of the cauda equina. J Bone Joint Surg [Br] 1985;67B:358-62. 15. Kernohan JW. Tumors of the spinal cord. Arch Pathol 1941;31:843-83. 16. Koyama T, Ishikawa J, Kondo A. Zwei Neurinome der Cauda equina bei einem Patienten mit einem vermuteten Bandscheibenvorfall. Neurochirurgia (Stuttg) 1978;21:172-8. 17. Love JG, Wagener HP, Woltman HW. Tumors of the spinal cord associated with choking of the optic disks. Arch Neurol Psychiatry 1951;66:171-7. 18. Lusins J, Kotsilimbas D. Normal-pressure hydrocephalus associated with markedly elevated CSF protein~ N Y State J Med 1983;83:1151-2. 19. Maeda M, Shimazaki K, Hirohata K, Kurihara A, Kataoka O. Cauda equina tumor causing increased intracranial pressure. Kobe J Med Sci 1986;32:97-104. 20. Maurice-Williams RS, Lucey JJ. Raised intracranial pressure due to spinal tumours: 3 rare cases with a probable common mechanism. BrJ Surg 1975;62:92-5. 21. Messer HD, Brinker RA. Hydrocephalus and dementia complicating spinal tumor. J Neurosurg 1980;53:544-7. 22. MorleyJB, Reynolds EH. Papilloedema and the Landry-GuillainBarr6 syndrome. Brain 1966;89:205-22. 23. Neil-Dwyer G. Tentorial block of cerebrospinal fluid associated with a lumbar neurofibroma. J Neurosurg 1973;38:767-70.
314
Surg Neurol 1992;38:309-14
24. Nicola GC, Nizzoli V. Increased intracranial pressure and papilloedema associated with spinal tumors. Neurochirurgia (Stuttg) 1969;12:138-44. 25. Nishida K, Ueda S, Matsumoto K, Kusaka K, Takeuchi R. Cauda equina neurinomaassociated with normal pressure hydrocephalus. Neurol Med Chit (Tokyo) 1990;30:258-62. 26. Norstom CW, Kernohan JW, Love JG. One hundred primary caudal tumors. JAMA 1961;178:1071-7. 27. Pardatscher K, Iraci G, Cappellotto P, Rigobello L, Pellone M, Fiore D. Multiple intramedullary neurinomas of the spinal cord. J Neurosurg 1979;50:817-22.
Oikawa et al
28. Rath S, Mishra M. Papilloedema caused by spinal tumour in a case of optic nerve glioma. Indian J Ophthalmol 1984;32:175-6. 29. Ridsdale L, Moseley I. Thoracolumber intraspinal tumours presenting features of raised intracranial pressure. J Neurol Neurosurg Psychiatry 1978;41:737-45. 30. Schijiman E, Zficcaro G, MongesJA. Spinal tumor and hydrocephalus. Childs Brain 1981;8:401-5. 31. Ucar S, Fl6rez G, GarciaJ. Increased intracranial pressure associated with spinal cord tumours. Neurochirurgia (Stuttg) 1976;19:265-8.
309
Multiple Spinal Neurinomas Presenting Visual Disturbance as the Initial Symptom: Case Report Susumu Oikawa, M.D., Kazuhiko Kyoshima, M.D., Toshiki Takemae, M.D., and Shigeaki Kobayashi, M.D. Department of Neurosurgery, Shinshu University School of Medicine, Asahi 3-1-1, Matsumoto 390, Japan
Oikawa S, Kyoshima K, Takemae T, Kobayashi S. Multiple spinal neurinomas presenting visual disturbance as the initial symptom: case report. Surg Neurol 1992;38:309-14.
A case of multiple spinal neurinomas with visual disturbance is reported. A 63-year-old man was admitted with a complaint of progressive visual disturbance due to papilledema without spinal symptoms and signs. The neuroimaging studies demonstrated communicating hydrocephalus and two mass lesions in the cauda equina. Both tumors were found to be neurinomas. Intracranial hypertension secondary to spinal tumors is unusual, and multiple spinal neurinomas are rare. In the patient without spinal symptoms and signs, it is difficult to make a diagnosis of spinal tumor. Importance of checking for a spinal cord lesion by magnetic resonance imaging in such a case is stressed.
at a bright object. H e visited the Ophthalmology Service of Shinshu University Hospital on March 14, 1990, and was found to have bilateral papilledema o f an advanced stage. Visual acuity was 20/40 in the right and 20/50 in the left. A computed tomography (CT) scan and an MRI of the head disclosed no intracranial mass lesion. Mild enlargement of lateral ventricles and bilateral sylvian fissures and empty seUa were observed (Figure 1). His visual disturbance progressed to 20/130 in the right eye and 20/250 in the left during the following two weeks. A lumbar puncture revealed that the cerebrospinal fluid (CSF) pressure was 420 mm H 2 0 . Immediate surgery was advised but was not accepted by the patient. H e was readmitted to our service for surgery on April 6, 1990. H e had had no symptoms of intracranial hypertension or o f a spinal lesion.
K E Y WORDS: Multiple spinal neurinomas; Papilledema; Intracranial hypertension; Magnetic resonance imaging
Examination Intracranial hypertension or hydrocephalus secondary to spinal tumor is well known but unusual [ 1 - 8 , 1 2 , 1 7 - 2 1 , 2 3 - 2 5 , 2 8 - 3 1 ] . In a case with no symptoms and signs o f a spinal lesion except visual disturbance and papilledema, diagnosis o f spinal tumor is likely to be missed. A rare case with multiple spinal neurinomas presenting with visual disturbance as the initial symptom is reported. Difficulty in diagnosis and usefulness of magnetic resonance imaging (MRI) are discussed. Case Report A 63-year-old man without any previous illness or familial history o f note was referred to our service with a chief complaint o f progressive bilateral visual disturbance. H e noticed blurred vision in February 1990, when looking Address reprint requests to: Susumu Oikawa, M.D., Department of Neurosurgery, Shinshu University School of Medicine, Asahi 3-1-1, Matsumoto 390, Japan. Received February 11, 1992; accepted June 12, 1992.
© 1992 by Elsevier Science Publishing Co., Inc.
O n admission, the neurological examination revealed no abnormal findings except bilateral visual disturbance and papilledema. Mental deterioration, gait disturbance, and urinary dysfunction were not observed. Neither motor nor sensory disturbance was recognized. Deep tendon reflexes were normal. Analysis of the CSF revealed xanthochromia, a high protein concentration (235 mg/dL), a high-normal sugar level (73 mg/dL), and normal cell counts. CT cisternography disclosed ventricular reflux 6 hours after infusion o f iotrolan, and retention o f the contrast medium in the ventricles over 48 hours. Intravenous digital subtraction angiography (IVDSA) revealed no abnormal findings. Sinus or venous thrombosis was not identified. These findings on CT cisternography and IVDSA supported the diagnosis o f communicating hydrocephalus unrelated to vascular diseases. A ventriculoperitoneal (VP) shunt was done on April 8, 1990. The spinal MRI taken after the VP shunt disclosed two enhanced mass lesions (Figure 2). One was approximately 3 cm in its major axis, with a clear margin, and occupied the intradural space at the L 5 - $ 1 level. The other mass was round, 4 mm in 0090-3019/92/$5,00
310
Surg Neurol 1992;38:309-14
Oikawa et al
A
Figure 1. Cerebral C T and MRI scans with contrast material showing absence of mass lesion. (A) A C T scan showing mild enlargement of the bilaterial ventricles and the bilateral sylvian fissures. (B) A coronal MR1 showing empty sella.
Figure 2. MRI scans of lumbosacral area. (A) Sagittal Tl-weighted image disclosing a small, round isointense mass at L 1 - 2 levd (single arrow) and a large isointense mass at L5-S1 level (paired arrows). (B) Gadolinium-enhanced MRI showing homogeneous enhancement of both masses.
A
B
Multiple Spinal Neurinomas
Surg Neurol 1992;38:309-14
311
A C
Figure 3. Myelogram and C T myelogram. (A) Myelogram demonstrates a filling defect at L5-S1 level, but fails to show a small mass at L 1 - 2 level. (13) C T myelogram discloses a small mass at L 1 - 2 level as a low-density spot. (C) Nerve roots (arrows) around the large mass at LS-S1 level are recognized with C T myelography.
diameter, and located at the L I - 2 level, just below the conus medullaris. Myelography demonstrated a large filling defect at the L 5 - $ 1 level but failed to reveal the small mass at the L 1 - 2 level. Correlation between the large mass and nerve roots was not clear on myelography. CT myelography revealed that the small mass was a round, hypodense lesion, and the cauda equina was compressed dorsally by the large mass at the L 5 - $ 1 level (Figure 3).
Operation
U n d e r the diagnosis of e p e n d y m o m a or neurinoma, surgery was performed on July 2, 1990. The patient was
placed in a prone position, and two midline linear skin incisions were made in the upper lumbar and lumbosacral areas. Laminectomies were perfo~rmed separately at L1 and L 5 - $ 1 . In the upper wound, a yellowish 1-cm round tumor was found in the right side o f the dural opening. It had incorporated a fine nerve rootlet, which was resected with the tumor. The dura of the lower wound was opened. The tumor was immediately found, covered by the cauda equina. By electric stimulation, the root involved in the tumor was confirmed to be a sensory nerve. Although an attempt was made at preserving the sensory root, it was finally resected for the sake of total removal. Both tumors were completely resected. Histological diagnosis of both tumors was neurinoma (Figure 4). Postoperatively, no further neurological deficits, including sensory disturbance, were detected. Four months after the VP shunt, the patient's visual acuity improved to 20/60 in the right eye and 20/200 in the left, and bilateral papilledema disappeared; however, optic atrophy of both eyes was evident on fundoscopy.
312
Surg Neurol 1992;38:309-14
Oikawa et al
Figure 4. Photomicrographs of surgical specimen showing typical neurinomas. (A) Large tumor at L5-S1 level. (B) Small tumor at L1-2 level. Both specimens reveal spindle-shaped cells arranged in interlacing fascicles and slight myxoid degeneration. (Hematoxylin and eosin stain, original magnification × 80.)
A
B
Discussion In this case o f multiple spinal neurinomas, three interesting problems were mentioned: One was that the onset was visual disturbance; second was multiplicity o f spinal neurinoma not associated with skin lesions or particular familial history, as is characteristic o f von Recklinghausen's disease; and third was that the patient did not complain of headache or spinal symptoms.
Intracranial hypertension or hydrocephalus caused by spinal tumors is relatively rare [1]. Thirty-four cases of spinal tumors with intracranial hypertension or hydrocephalus have been reported in the literature with detailed description [ 1 - 8 , 1 2 , 1 7 - 2 1 , 2 3 - 2 5 , 2 8 , 2 9 , 3 1 ] . The onset was visual disturbance in six cases [3,6,8,24,28,29], o f which only two [8,29] had no spinal symptoms and signs. One o f the two cases was diagnosed
Multiple Spinal N e u r i n o m a s
as spinal tumor 3 months after admission, when the patient started to complain of pain in the lower back and right leg [29]. In a patient without spinal symptoms or signs, it is difficult to make a diagnosis of spinal tumor. The pathogenesis of intracranial hypertension or hydrocephalus with spinal tumors remains unknown. One of the most widely accepted mechanisms is impaired absorption of the CSF. The causes for the malabsorption of the CSF include tumor infiltration into the basal cistern [20], subarachnoid hemorrhage from tumor [29], tumor compressing the venous plexus [7], and raised CSF protein [6,19]. In 39 cases of spinal tumor with C SF analysis [ 1 - 8 , 1 2 , 1 7 - 2 1 , 2 3 - 2 5 , 2 8 - 3 1 ] , 36 cases (92%) showed high CSF protein. On the other hand, several cases of GuiUian-Barr6 syndrome with high CSF protein presented with signs of intracranial hypertension [13,22]. High CSF protein in a case of spinal tumor may be mostly responsible for obstruction of the CSF outflow. Gibberd et al [6] have suggested that increased immunoglobulin in the CSF due to the spinal tumor impairs absorption of the CSF in the arachnoid villi. However, at least three spinal cord tumors with normal CSF protein have been reported [8,29,30]. Therefore, mechanisms of intracranial hypertension or hydrocephalus may be multifactorial. CSF analysis may be useful for diagnosis. In a patient with high CSF protein, spinal tumor should not be ruled out from the differential diagnosis. MRI is a noninvasive and useful examination for detecting spinal lesions without bone artifact and can detect a small lesion that conventional or CT myelogram may fail to disclose. Spinal MRI should be performed for all cases of communicating hydrocephalus of unknown origin. Most common tumors of the cauda equina are ependymoma and neurinoma [10,14]. Both tumors were found to be a cause for intracranial hypertension and/or hydrocephalus [12,19,23,31]. Ependymoma, especially myxopapillary ependymoma, of the cauda equina often metastasizes to the subarachnoid space and is recognized as multiple tumors. Multiple spinal neurinomas are relatively rare. They have been found in some cases of von Recklinghausen's disease. The presence of "forme fruste" of central neurofibromatosis was proposed, and multiple spinal neurinomas without skin lesions might be regarded as some type of von Recklinghausen's disease [1 i]. This hypothesis is disputable; multiple spinal neurinomas without skin lesions and/or remarkable familial history is rare. Only four cases are reported in the literature [9,15,16,27]. Spinal neurinoma can present in many forms. MRI is regarded as a useful screening tool for detecting multiple spinal tumors. Surgical resection under the operating microscope is considered the sole treatment for spinal neurinoma.
Surg N e u r o l 1992;38:309-14
313
References 1. Arseni C, Maretsis M. Tumors of the lower spinal cord associated with increased intracranial pressure and papilloedema. J Neurosurg 1967;27:105-10. 2. Bamford CR, Labadie EL. Reversal of dementia in normotensive hydrocephalus after removal ofa cauda equina tumor.J Neurosurg 1967;45:104-7. 3. Bcrgesen SE, Scrensen SC, Olsen J. Gjerris F. Spinal tumours associated with increased intracranial pressure. Acta Neurol Scand 1977;56:263-8. 4. Feldmann E, Bromfield E, Navia B, Pasternak GW, Posner JB. Hydrocephalic dementia and spinal cord tumor. Arch Neurol 1986;43:714-8. 5. Gelabert M, Bollar A, Paseiro MJ, Allut AG. Hydrocephalus and intraspinal tumor in children. Childs Nerv Syst 1990;6:110-2. 6. Gibberd FB, Ngan H, Swann GF. Hydrocephalus, subarachnoid haemorrhage and ependymomas of the cauda equina. Clin Radiol 1972;23:422-6. 7. Glasauer F. Throacic and lumbar intraspinal tumors associated with increased intracranial pressure. J Neurol Neurosurg Psychiatry 1964;27:451-8. 8. Hansen K, Gjerris F, S0rensen S. Absence of hydrocephalus in spite of impaired cerebrospinal fluid absorption and severe intracranial hypertension. Acta Neurochir (Wien) 1987;86:93-7. 9. Hida K, Akino M, Isu T, Iwasaki Y, Abe H, Saito H. Multiple neurinoma of the spinal cord: case report. No Shinkei Geka 1988;16:1489-93. 10. HogenEsch RI, Staal MJ. Tumors of the cauda equina: the importance of an early diagnosis. Clin Neurol Neurosurg 1988; 90:343-8. 11. Hori A. 13ber intraspinale Schwannosen der Zona terminalis (Lissauer). (Formes fruste der Neurofibromatose Recklinghausen oder "reaktiv"?). Acta Neuropathol (Berl) 1973;25:89-94. 12. lob I, Andrioli GC, Rigobello L, Salar G. An unusual onset of a spinal cord tumour: subarachnoid bleeding and papilloedema. Neurochirurgia (Stuttg) 1980;23:112-6. 13. Joynt RJ. Mechanism of production of papilloedema in the Guillain-Barr6 syndrome. Neurology 1958;8:8-12. 14. Ker NB, Jones CB. Tumors of the cauda equina. J Bone Joint Surg [Br] 1985;67B:358-62. 15. Kernohan JW. Tumors of the spinal cord. Arch Pathol 1941;31:843-83. 16. Koyama T, Ishikawa J, Kondo A. Zwei Neurinome der Cauda equina bei einem Patienten mit einem vermuteten Bandscheibenvorfall. Neurochirurgia (Stuttg) 1978;21:172-8. 17. Love JG, Wagener HP, Woltman HW. Tumors of the spinal cord associated with choking of the optic disks. Arch Neurol Psychiatry 1951;66:171-7. 18. Lusins J, Kotsilimbas D. Normal-pressure hydrocephalus associated with markedly elevated CSF protein~ N Y State J Med 1983;83:1151-2. 19. Maeda M, Shimazaki K, Hirohata K, Kurihara A, Kataoka O. Cauda equina tumor causing increased intracranial pressure. Kobe J Med Sci 1986;32:97-104. 20. Maurice-Williams RS, Lucey JJ. Raised intracranial pressure due to spinal tumours: 3 rare cases with a probable common mechanism. BrJ Surg 1975;62:92-5. 21. Messer HD, Brinker RA. Hydrocephalus and dementia complicating spinal tumor. J Neurosurg 1980;53:544-7. 22. MorleyJB, Reynolds EH. Papilloedema and the Landry-GuillainBarr6 syndrome. Brain 1966;89:205-22. 23. Neil-Dwyer G. Tentorial block of cerebrospinal fluid associated with a lumbar neurofibroma. J Neurosurg 1973;38:767-70.
314
Surg Neurol 1992;38:309-14
24. Nicola GC, Nizzoli V. Increased intracranial pressure and papilloedema associated with spinal tumors. Neurochirurgia (Stuttg) 1969;12:138-44. 25. Nishida K, Ueda S, Matsumoto K, Kusaka K, Takeuchi R. Cauda equina neurinomaassociated with normal pressure hydrocephalus. Neurol Med Chit (Tokyo) 1990;30:258-62. 26. Norstom CW, Kernohan JW, Love JG. One hundred primary caudal tumors. JAMA 1961;178:1071-7. 27. Pardatscher K, Iraci G, Cappellotto P, Rigobello L, Pellone M, Fiore D. Multiple intramedullary neurinomas of the spinal cord. J Neurosurg 1979;50:817-22.
Oikawa et al
28. Rath S, Mishra M. Papilloedema caused by spinal tumour in a case of optic nerve glioma. Indian J Ophthalmol 1984;32:175-6. 29. Ridsdale L, Moseley I. Thoracolumber intraspinal tumours presenting features of raised intracranial pressure. J Neurol Neurosurg Psychiatry 1978;41:737-45. 30. Schijiman E, Zficcaro G, MongesJA. Spinal tumor and hydrocephalus. Childs Brain 1981;8:401-5. 31. Ucar S, Fl6rez G, GarciaJ. Increased intracranial pressure associated with spinal cord tumours. Neurochirurgia (Stuttg) 1976;19:265-8.
