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Show Journal of Clmlcal Neuro- 0l'hthalmology 12( 3): 158- 162, 1992. Papilledema and Intraspinal Lumbar Paraganglioma David R. Hardten, M. D., Dennis Y. Wen, M. B., B. S., Jonathan D. Wirtschafter, M. D., Joo H. Sung, M. D., and Donald L. Erickson, M. D. © 1992 Raven Press, Ltd., New York Optic nervehead swelling is most frequently caused by ocular or intracranial lesions. The case presented here demonstrates that the spinal subarachnoid space must also be considered as a potential site for a lesion causing optic nervehead swelling. A 56- year- old man is presented with an intraspinal lumbar paraganglioma associated with increased cerebrospinal fluid protein, papilledema, transient obscurations of vision, and back pain. This may be the first reported case of a paraganglioma associated with optic nervehead swelling. Magnetic resonance imaging of the lumbosacral region revealed the lesion noninvasively. The papilledema, transient obscurations of vision, and back pain resolved after resection of the tumor. The mechanisms are not defined for optic nervehead swelling in association with spinal tumors in general and paraganglioma in particular. The measured abnormal elevation of cerebrospinal fluid protein may have resulted in increased intracranial pressure and papilledema. Key Words: Optic nervehead swelling- Paraganglioma- Spinal cord lesion- Papilledema- Magnetic resonance imaging ( MRI). From the Departments of Ophthalmology ( D. R. H., J. D. W.), Neurosurgery ( D. Y. W., J. D. W., D. L. E.), and Neuropathology ( J. H. 5.), University of Minnesota Hospital and Clinics, Minneapolis, Minnesota, U. S. A. This work was supported in part by grants from the Minnesota Lions and Lionesses and by an unrestricted grant from Research to Prevent Blindness, Inc. Address reprint requests to Dr. Jonathan D. Wirtschafter, Department of Ophthalmology, University of Minnesota, UMHC Box 493, Harvard Street at East River Road, Minneapolis, MN 55455, U. S. A. 158 Optic nervehead swelling usually is a direct result of ocular and intracranial causes, although it has occasionally been associated with spinal cord tumors. The mechanisms by which spinal cord tumors produce optic nervehead swelling have not been clearly established. We describe what we believe to be the first reported case of an intraspinal lumbar paraganglioma that resulted in optic nervehead swelling. Magnetic resonance imaging ( MRI) of the spine was the optimal technology to make the diagnosis of the intradural lesion. CASE HISTORY The patient was a 56- year- old male who presented with a 1V2- year history of intermittent dimming of his vision, which occurred several times each day. He had experienced mild headaches for several years. Back pain had been present for more than 30 years since a minor injury while in military service, but had worsened over the last 6 years. He had noted radicular pain and numbness down his right leg in the last 2 years. He was unable to lie comfortably for more than 2 hours at a time because of the pain. Past medical history was remarkable for hypertension, atrial fibrillation, and nephrolithiasis. Medications were nicardipine, quinidine, hydrochlorothiazide/ triamterene, digoxin, bumetanide, naproxen, and aspirin. In the year prior to referral to our center, optic nervehead swelling was noted, and evaluation included normal brain and lumbar spine com~ uted tomography. Electromyelography revealed bl1ateral borderline conduction velocities of lumbar motor and sensory nerves, and dener- PAPILLEDEMA AND PARAGANGLIOMA 159 vation potentials in the L4- 5 distribution on the right suggestive of a radiculopathy. Lumbar puncture performed at the L3- 4 level revealed 6,400 RBC/ mm3 , 200 WBC/ mm3 , and a protein of 2,022 mg/ dl. Upon referral to our institution, physical examination revealed deep tendon reflexes to be symmetric but reduced in the lower extremities. Motor strength and muscle bulk was normal, and straight leg raising was negative. Visual acuity in the right eye was 20/ 20, and in the left eye was 20/ 15. Con-trast sensitivity testing with wall- mounted plates ( Vistech, Dayton, OH) revealed decreased sensitivity in both eyes at higher spatial frequencies, with the right eye showing poorer responses than the left. Goldmann visual field testing showed bilateral enlargement of the physiologic blind spot. Fundus examination demonstrated bilateral optic nervehead swelling and nerve fiber layer hemorrhages ( Fig. 1). Magnetic resonance imaging of the head revealed multiple nonspecific punctate signal aber- A B FIG. 1. ( A) Right optic nervehead showing nerve fiber layer hemorrhages and axonal swelling. ( B) Left optic nervehead showing nerve fiber layer hemorrhages and axonal swelling. I Clin Neuro- ophthalmol, Vol. 12. No. 3, 1992 160 D. R. HARDTEN ET AL. rations in the white matter ( Fig. 2). Because of the association of back pain with the optic nervehead swelling a lumbar spine MRI was performed. Spine MRI revealed an enhancing intradural mass at Ll- 2 ( Fig. 3). The patient underwent lumbar laminectomy and total excision of a wellencapsulated, but rather adherent intradural mass, arising from a lumbar nerve root, which measured 4 x 2 em. Cerebrospinal fluid ( CSF) was removed rostral to the tumor during surgery revealing 0- 2 RBClmm3 and a protein of 182 mg/ dl. The biopsied mass was histologically characteristic for paraganglioma. The tumor was cellular and strikingly lobulated by delicate vascular stroma ( Fig. 4). The tumor cells were uniform and epithelioid, having round or oval nuclei, and watery or pale granular cytoplasm in hematoxylin and eosin stain. With immunoperoxidase stain of the paraffin section with antiserum against chromogranin, a majority of the tumor cells exhibited strong reactivity. Two months postoperatively the visual acuity was 20/ 15 in each eye, and there was resolution of the transient visual obscurations, headaches, and radicular back pain. The papilledema was markedly improved, and small golden dots were seen in FIG. 2. Magnetic resonance imaging of the brain and optic nerve sheaths. ( Axial view, 1.5 tesla, TR 500 msec, TE 20 msec.) : ':":,,,,' 1 \' 01. 12. No. 3. 1992 FIG. 3. Intraspinal paraganglioma as shown by magnetic resonance imaging at the L1- 2 level after infusion of gadolinium. ( Sagittal view, 1.5 tesla, TR 2,500 msec, TE 90 msec.) the region of the papillomacular bundle compatible with resolving papilledema. DISCUSSION Although optic nervehead swelling most commonly occurs in optic neuritis, anterior ischemic optic neuropathy, benign intracranial hypertension, and intracranial mass lesions, spinal neoplasms have occasionally been associated with papilledema ( 1- 14). In this patient an unusual lesion, an intradural lumbar paraganglioma, caused visual symptoms and optic nervehead swelling, which resolved after removal of the lesion. Despite several reports of spinal cord tumors associated with increased intracranial pressure and papilledema, the pathophysiology remains poorly understood. A wide range of mechanisms have been described to account for this phenomenon of increased intracranial pressure and papilledema secondary to a spinal cord tumor. The majority of mechanisms concentrate on the effects of the tu~ or or tum~ r products on the normal CSF resorption mechamsms. Paraganglioma is a relatively unusual tumor in the spinal column, with about 80 cases reported in the literature, but it has not been previously associated with optic nervehead swelling ( 15- 17). PAPILLEDEMA AND PARAGANGLIOMA FIG. 4. Paraganglioma showing clusters of tumor cells separated by delicate vascular stroma. ( Hematoxylin and eosin, x 180.) 161 Elevated CSF protein in intradural tumors may arise from several sources. Recurrent spontaneous bleeding into the subarachnoid space may occur in ependymomas, which are the predominant tumors associated with this phenomenon ( 14). Tumor material may also break down, or the tumor may spread throughout the extra and intracranial subarachnoid space, blocking the CSF drainage channels. Such tumor spread is frequent in ependymomas ( 9). Increased protein may result from active secretion of protein by the tumor or transudation from leaky tumor vessels. A localized basal arachnoiditis from substances foreign to the normal CSF may also occur, causing obstruction to CSF drainage ( 2,11). In spinal column tumors increased CSF protein appears to be a major factor causing increased intracranial pressure and papilledema, frequently through the development of a communicating hydrocephalus. Elevated CSF protein probably also plays a role in the Guillain- Barre syndrome and poliomyelitis ( 5). Experimentally, serum protein and artificially viscous solutions placed in the CSF have been shown to decrease the rate of fluid absorption from the subarachnoid space ( 5). Cerebrospinal fluid viscosity and osmotic tension themselves seem to be less important, as their effect should be stable over time. Impaired CSF absorption in a patient with hydrocephalus from a lumbar neurofibroma was demonstrated using CSF infu-sion studies ( 8), supporting these experimental studies. This case illustrates the syndrome of papilledema associated with an intradural tumor, in this instance a cauda equina paraganglioma. The visual symptoms and papilledema responded well to resection of the lesion. The increased intracranial pressure is possibly caused by blockage of the outflow channels by increased protein in the CSF, although hydrocephalus was not present. Magnetic resonance imaging of the lumbosacral region now allows this diagnosis to be recognized with little risk to the patient. REFERENCES 1. Ammerman Bj, Smith DR. Papilledema and spinal cord tumors. Surg Neuro/ 1975; 3: 55- 7. 2. Arseni C, Maretsis M. Tumors of the lower spinal cord associated with increased intracranial pressure and papilledema. , Neurosurg 1967; 27: 105- 10. 3. Borgesen SE, Sorensen SC, Olesen L Gjerris F. Spinal tumours associated with increased intracranial pressure. Acta Neurol Scand 1977; 56: 263- 8. 4. Farmilo RW, McAuley DL. Osborne DRS. Papilloedema and spinal cord tumors. 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