Department of Neurosurgery, University of Florida, Gainesville, Florida Neurosurgery 31; 783-786, 1992 ABSTRACT: A CASE OF a 60-year-old man with metastatic adenocarcinoma of the colon who developed bilateral ophthalmoplegia acutely is presented. Bilateral cavernous sinus metastasis was suggested by magnetic resonance imaging and proven by endoscopic sphenoidal biopsy. The patient was treated with radiotherapy (2000 cGy) with near resolution of his symptoms. This patient represents the first reported case of acute bilateral ophthalmoplegia resulting from hematogenous metastasis to both cavernous sinuses, diagnosed antemortem. A review of the more common causes of acute bilateral ophthalmoplegia is presented. KEY WORDS: Adenocarcinoma; Cavernous sinus; Ophthalmoplegia Metastatic disease of the cavernous sinus is not an uncommon cause of parasellar syndromes. It accounted for 23% of 102 parasellar lesions reviewed by Thomas and Yoss (18). Its presentation, however, occurs almost exclusively as a unilateral, rapidly progressive, painful ophthalmoplegia (1,11). Occasionally, trigeminal nerve function is spared with lesions situated anteriorly within the cavernous sinus (15). The diagnosis should be considered strongly in patients with unilateral cavernous sinus syndrome. We report the case of a man with adenocarcinoma of the colon with bilateral cavernous sinus metastasis. The diagnosis was made antemortem, which, to our knowledge, is the first documented case. Additionally, a review of the more common causes of acute bilateral ophthalmoplegia is presented. CASE HISTORY A 60-year-old white man underwent a right hemicolectomy for an abdominal mass 2 weeks before presentation. A diagnosis of adenocarcinoma of the colon with metastasis to the liver was made at the time of surgery. The patient did well and was discharged 1 week after his procedure. Within 10 days, the patient developed right ptosis acutely with associated diplopia. His symptoms rapidly progressed during the next 72 hours, resulting in an inability to open both of his eyes. He denied any headache, numbness, weakness, fever, or night sweats. He also denied any history of diabetes mellitus or ethanol abuse. A physical examination revealed an elderly man
DISCUSSION Although reports of bilateral cavernous sinus metastasis can be found in the literature (12,14), it is an
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AUTHOR(S): Supler, Mitchell L., M.D.; Friedman, William A., M.D.
with bilateral ptosis. His blood pressure was 112/80 mm Hg, and he was afebrile. No meningismus was noted. His higher cortical functions were intact. A cranial nerve evaluation demonstrated complete external ophthalmoplegia on the left. His left pupil measured 3 mm in diameter and had intact direct and consensual light reflexes. His right pupil measured 6 mm and was poorly responsive to light. Further examination of the right eye revealed third and fourth nerve palsies, with normal lateral rectus function. His visual acuity was 20/20, corrected bilaterally. Corneal reflexes were present, and his facial sensation was unaffected. The remainder of his neurological examination was unremarkable. A cerebral magnetic resonance imaging scan with MAGNEVIST was performed. No abnormal pial or dural enhancement was noted. Changes within the sphenoid sinus were thought to be consistent with sinusitis. Also, both cavernous sinuses demonstrated unusual dense enhancement suggesting a diffuse process (Fig. 1). Neither cavernous sinus appeared to be enlarged. A lumbar puncture was performed revealing an opening pressure of 160 mm H2O, a total protein level of 74 mg/dL, and a glucose level of 79 mg/dL. The cerebrospinal fluid contained six nucleated cells, 83% monocytes, and 17% polymorphonuclear neutrophils. Stains for bacteria, fungi, and acid fast organisms were negative. Cerebrospinal fluid cytology was similarly negative for any neoplastic cells. The patient was given thiamine without any changes in his symptoms. A tensilon test was also performed, which had no effect on his extraocular muscle palsies. Because of the abnormal appearance of the sphenoid and cavernous sinuses, the possibility of a neoplastic process affecting these areas was highly considered. The patient then underwent an endoscopic sphenoid sinus biopsy. Mucoperiosteal changes of the sphenoid without obvious tumor or infection were noted at the time of surgery. A frozen section diagnosis revealed nasal mucosa with fibrosis. No tumor was seen. Additional biopsies were taken for permanent section evaluation. Despite the initial lack of pathological evidence, metastatic disease of both cavernous sinuses was still considered the most likely cause. The patient was then given a trial treatment with dexamethasone with only mild improvement of his symptoms. Radiotherapy consisting of 2000 cGy given in 5 fractions was also initiated. On the third day of treatment, the final pathological diagnosis revealed poorly differentiated adenocarcinoma (Fig. 2). At the completion of radiotherapy, the patient experienced marked improvement of his symptoms. His third and fourth nerve palsies in both eyes had resolved. Only a residual left sixth nerve paresis remained. The patient was discharged to the care of his family; he died of systemic disease 6 weeks later.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 October 1992, Volume 31, Number 4 783 Acute Bilateral Ophthalmoplegia Secondary to Cavernous Sinus Metastasis: A Case Report Case Report
Received, August 29, 1991. Accepted, March 11, 1992. Reprint requests: William A. Friedman, M.D.,
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
vision can be spared when a calcified diagphragma sella limits the superior extent of an expanding sella (19) . Evidence for acute or chronic endocrinopathies and radiological abnormality of the sellar region suggest pituitary disease (2). Occlusive basilar-vertebral artery disease occasionally produces bilateral ophthalmoplegia. In a series of 12 patients with basilar-vertebral disease, Masucci (10) reported six cases of bilateral ocular muscle palsies of which two had complete ophthalmoplegia. Shutt et al. (16) have noted that the ophthalmoplegia is associated with fluctuating states of wakefulness, gait disturbance, hyperreflexia, and dysarthria, which aids localization within the neuraxis. No discussion of bilateral ocular palsies would be complete without mentioning Wernicke's encephalopathy. Patients classically present with bilateral abducens palsies, nystagmus, confabulation, and a history of ethanol abuse. Ptosis is not present. Improvement after the administration of thiamine is noted. Infectious processes can also produce symptoms through infiltration at the base of the skull. Mucormycosis (4) and tuberculous meningitis (20) have both been reported to cause progressive, painful, bilateral ophthalmoplegia. Evaluation of the cerebrospinal fluid for pleocytosis and evidence for the offending organism facilitate the diagnosis. Leptomeningeal seeding by metastatic cancer can exhibit multiple neurological deficits including ocular muscle dysfunction. In a series of 50 patients with meningeal carcinomatosis, Olson et al. (13) reported combined third and sixth nerve palsies in 12 patients. Ophthalmoplegia eventually resulted in 35 cases (13). Other nonspecific symptoms included headache, leg pains, back pain, and unsteadiness of gait (9). Cranial nerve involvement was usually a late manifestation (9). The most common malignancies reported were breast, lymphoma, lung, and melanoma (9,13) . Lumbar puncture is an essential tool in making the diagnosis, and several spinal taps are often necessary to demonstrate malignant cells through cytocentrifuge techniques. Other rare causes of acute bilateral ophthalmoplegia include basal sarcoidosis (20), diabetic neuropathy (15), and clivus chordoma (5). Hematogenous metastasis to both cavernous sinuses producing bilateral ophthalmoplegia can also be considered a rare phenomenon. Only one case has been previously reported. Mills et al. (11) noted the development of total bilateral ophthalmoplegia in a 59-year-old man after burr hole drainage of a subdural hematoma. Metastatic lung carcinoma was eventually diagnosed at necropsy. Our case, diagnosed antemortem, demonstrates that prompt recognition of this rare occurrence by neurosurgeons can enable the cancer patient to receive treatment that may reverse neurological deficits and improve quality of life.
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extremely rare cause among diseases producing bilateral ophthalmoplegia. There are a number of more common etiologies that should be considered when evaluating patients with this constellation of symptoms. Postinfectious polyneuritis (Guillain-Barré syndrome) can often produce acute bilateral ophthalmoplegia. The syndrome consists of a rapid, symmetric, ascending polyneuropathy frequently occurring several weeks after an acute infection. Fisher's syndrome, a variant that predominantly affects cranial nerves, produces ophthalmoplegia and bilateral facial paresis, with only mild limb weakness (3) . The paralysis may progress for 10 days before stabilizing. Among 60 patients with acute bilateral ophthalmoplegia, Keane (7) reported Guillain-Barré and Fisher's syndromes as the offending cause in 25 patients. He recommended observation for several days to document ophthalmoplegia, limb weakness, and loss of deep tendon reflexes. A demonstrated rise in cerebrospinal fluid protein also helps to ensure the diagnosis (7). Disorders of neuromuscular junction frequently produce bilateral ophthalmoplegia. Ocular muscle dysfunction is the initial presenting symptom in 75% of patients with myasthenia gravis (5). Diplopia and ptosis are the most common features that normally develop toward the end of the day and are most profound at night. The ophthalmoplegia produced is usually painless and invariably spares pupillary function (15). Intravenous edrophonium can alleviate ocular palsies and can easily confirm the diagnosis. Botulinum neurotoxin may also produce bilateral ophthalmoplegia and is difficult to discern from myasthenia gravis and Guillain-Barré syndrome. A history of ingestion of tainted food is crucial. Evidence for circulating neurotoxin and an absence of cerebrospinal fluid protein elevation facilitate the diagnosis (15). Additionally, botulism can present with accommodative paresis producing blurred vision. An examination may reveal mydriasis with poorly reactive pupils in addition to ptosis and extraocular muscle paresis (6,8,11,17). Dysthyroid myopathy commonly produces diplopia. However, ptosis is extremely rare, and the pupils are always spared (5). Evidence for lid retraction, proptosis, and a positive forced duction test leads one to the diagnosis. Chronic ocular myopathy, however, produces a symmetric, progressive immobility of the eyes (5). Ptosis is common, and the ophthalmoplegia produced is painless. Diplopia, interestingly, is infrequent. The course is usually slow without fluctuations or remissions, and a family history may be present (5). Pituitary apoplexy can produce bilateral ophthalmoplegia acutely. Symptoms result from hemorrhage into a pituitary adenoma that has extended into the cavernous sinuses. Bilateral total ophthalmoplegia resulting from this syndrome was first reported by Walsh in 1949 (19). More recently, Keane (7) reported 5 cases among his series of 60 patients. Typically, the onset is abrupt, with associated severe headache. Vision is often affected resulting in varying degrees of blindness. However,
REFERENCES: (1-20) 1.
3.
4. 5. 6.
7. 8. 9. 10. 11. 12.
13.
14.
15.
16.
18. 19.
20.
COMMENTS The cavernous sinus syndrome is characterized by ocular motor nerve palsies involving the third, fourth, and sixth cranial nerves as well as the first and second divisions of the fifth cranial sensory nerve (1). Involvement of the trigeminal nerve leads to the painful ophthalmoplegia commonly seen in this syndrome (3). When involvement of the fifth cranial nerve is absent or appears late in the course of this syndrome, an extrinsic process compressing the cavernous sinus is suggested (4). Additionally, the optic nerve may be affected intracranially, causing visual loss. Numerous causes for the cavernous sinus syndrome have been reported and fall into categories of tumors, aneurysms, and inflammatory lesions as well as vascular lesions (3). Metastatic lesions are frequently seen in the cavernous sinus syndrome but bilateral involvement is rarely associated with metastatic disease (2). In their case review, Supler et al. describe a rare presentation of bilateral hematogenous metastasis to the cavernous sinuses. They have also provided a thorough review of the literature on the subject of bilateral ophthalmoplegia in their discussion of differential diagnosis. Craig E. Geist Washington, District of Columbia REFERENCES: (1-4) 1.
2. 3.
4.
Bartholow R: Aneurysms of the arteries at the base of the brain: Their symptomatology, diagnosis and treatment. Am J Met Sci 64:373386, 1972. Keane JR: Acute bilateral ophthalmoplegia: 60 cases. Neurology 36:279-281, 1986. Miller NR: Topical diagnosis of neuropathic ocular motility disorders, in Walsh and Hoyt's Clinical Neuro-Ophthalmology. Baltimore, Williams & Wilkins, ed 4, 1985. Mills RP, Insalaco SJ, Joseph A: Bilateral cavernous sinus metastasis and ophthalmoplegia. J Neurosurg 55:463-466, 1981.
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/31/4/783/2752100 by Universitaetsbibliothek Muenchen user on 04 February 2019
2.
Ahmad K, Yamokoshi C, Kim Y, Post MJ, Fayos JV: Involvement of cavernous sinus region by malignant neoplasm: Report of five cases. J Am Optom Assoc 87:504-508, 1987. David NJ, Gargano FP, Glaser JS: Pituitary apoplexy in clinical perspective, in Glaser JS, Smith JL (eds): Neuro-Ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. St. Louis, CV Mosby, 1975, vol 8, pp 140-165. Fisher M: An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia, and areflexia) N Engl J Med 225:57-65, 1956. Gass JDM: Ocular manifestations of acute mucormycosis. Arch Ophthalmol 65:226-237, 1961. Glaser JS: Neuro-ophthalmology. Hagerstown, Harper and Row, 1978, pp 263281. Hedges TR, Jones A, Stark L, Hoyt WF: Botulin ophthalmoplegia: Clinical and oculographic observations. Arch Ophthalmol 101:211-213, 1983. Keane JR: Acute bilateral ophthalmoplegia: 60 cases. Neurology 36:279-281, 1986. Konig H, Gassman HB, Jenzer G: Ocular involvement in benign botulism B. Am J Ophthalmol 80:430-432, 1975. Little JR, Dale AJD, Okazaki H: Meningeal carcinomatosis: Clinical manifestations. Arch Neurol 30:138-143, 1974. Masucci EF: Bilateral ophthalmoplegia in basilar- vertebral artery disease. Brain 88:97106, 1965. Miller NR, Moses H: Occular involvement in wound botulism. Arch Ophthalmol 95:17881789, 1977. Mills RP, Insalaco SJ, Joseph A: Bilateral cavernous sinus metastasis and ophthalmoplegia. J Neurosurg 55:463-466, 1981. Olson ME, Chernik NL, Posner JB: Infiltration of the leptomeninges by systemic cancer: A clinical and pathologic study. Arch Neurol 30:122-137, 1974. Post MJ, Mendez DR, Kline LB, Acker JD, Glaser JS: Metastatic disease to the cavernous sinus: Clinical syndrome and CT diagnosis. J Comput Assist Tomogr 9:115-120, 1985. Sergott RC, Glaser JS, Berger LJ: Simultaneous, bilateral diabetic ophthalmoplegia: Report of two cases and discussion of differential diagnosis. Ophthalmology 91:18-21, 1984. Shutt HKR, David MJ, Smith JL: Complete bilateral III and IV nerve palsies due to basilar artery disease, in Smith JL (ed): NeuroOphthalmology: Symposium of the University
17.
of Miami and the Bascom Palmer Eye Institute. Hallandale, FL, Huffman, 1971, vol 5, pp 356-362. Terranova W, Palumbo JN, Breman JG: Ocular findings in botulism type B. JAMA 241:475-477, 1979. Thomas JE, Yoss RE: The parasellar syndrome: Problems in determining etiology. Mayo Clin Proc 45:617-623, 1970. Walsh FB: Bilateral total ophthalmoplegia with adenoma of the two pituitary glands: Report of two cases: An anatomic study. Arch Ophthalmol 42:646-654, 1949. Walsh FB, Hoyt WF: Clinical Neuro-Ophthalmology. Baltimore, Williams & Wilkins, 1969, ed 3, pp 1434-1440.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Department of Neurosurgery, Box J-265, JHMHC, University of Florida, Gainesville, FL 32610.
REFERENCES: (1) 1.
Richard BW, Jones FR, Younge BR: Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol 113:489496, 1992.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Brian R. Younge Rochester, Minnesota
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This article describes a patient with bilateral ophthalmoplegia that was caused by metastatic tumor to the cavernous sinus from adenocarcinoma of the colon. The proof of this was obtained antemortem by endoscopic sphenoidal biopsy and led to the patient being treated by irradiation, with near resolution of his symptoms. As pointed out by the authors, total bilateral ophthalmoplegia is not very common, and if we can assume the pupils were not involved, this makes for a very important exception to the general rule about third-nerve palsies and compressive lesions that spare the pupils. It is not entirely clear, however, that both pupils were totally uninvolved, and I would point out that such sparing might be overestimated in a bilateral involvement of third nerves, especially because the sympathetic pathways may well have not dilated the pupils much if they, too, were involved. The article provides a thorough review of multiple ocular motor palsies, specifically bilateral palsies, and I would point out that by far the majority of such cases imply a serious cause, most often tumor (1). In patients afflicted with multiple cranial nerve palsies, either uni- or bilateral ophthalmoplegia, a workup for the conditions mentioned in this article are mandatory. Recovery from such palsies is dependent on the ultimate cause, and as pointed out by the authors, palliative treatment is certainly worthy of consideration, especially in the more radiosensitive metastatic lesions, such as the adenocarcinomas of the breast or bowel.
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Figure 1. MAGNEVIST enhanced coronal T1weighted magnetic resonance imaging scan. Enhancement of both cavernous sinuses suggested a diffuse process. Enhancement within the sphenoid sinus was also noted.
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Figure 2. Photomicrograph of the sphenoid sinus lesion reveals poorly differentiated adenocarcinoma (hematoxylin and eosin, ×500).
DISCUSSION Although reports of bilateral cavernous sinus metastasis can be found in the literature (12,14), it is an
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AUTHOR(S): Supler, Mitchell L., M.D.; Friedman, William A., M.D.
with bilateral ptosis. His blood pressure was 112/80 mm Hg, and he was afebrile. No meningismus was noted. His higher cortical functions were intact. A cranial nerve evaluation demonstrated complete external ophthalmoplegia on the left. His left pupil measured 3 mm in diameter and had intact direct and consensual light reflexes. His right pupil measured 6 mm and was poorly responsive to light. Further examination of the right eye revealed third and fourth nerve palsies, with normal lateral rectus function. His visual acuity was 20/20, corrected bilaterally. Corneal reflexes were present, and his facial sensation was unaffected. The remainder of his neurological examination was unremarkable. A cerebral magnetic resonance imaging scan with MAGNEVIST was performed. No abnormal pial or dural enhancement was noted. Changes within the sphenoid sinus were thought to be consistent with sinusitis. Also, both cavernous sinuses demonstrated unusual dense enhancement suggesting a diffuse process (Fig. 1). Neither cavernous sinus appeared to be enlarged. A lumbar puncture was performed revealing an opening pressure of 160 mm H2O, a total protein level of 74 mg/dL, and a glucose level of 79 mg/dL. The cerebrospinal fluid contained six nucleated cells, 83% monocytes, and 17% polymorphonuclear neutrophils. Stains for bacteria, fungi, and acid fast organisms were negative. Cerebrospinal fluid cytology was similarly negative for any neoplastic cells. The patient was given thiamine without any changes in his symptoms. A tensilon test was also performed, which had no effect on his extraocular muscle palsies. Because of the abnormal appearance of the sphenoid and cavernous sinuses, the possibility of a neoplastic process affecting these areas was highly considered. The patient then underwent an endoscopic sphenoid sinus biopsy. Mucoperiosteal changes of the sphenoid without obvious tumor or infection were noted at the time of surgery. A frozen section diagnosis revealed nasal mucosa with fibrosis. No tumor was seen. Additional biopsies were taken for permanent section evaluation. Despite the initial lack of pathological evidence, metastatic disease of both cavernous sinuses was still considered the most likely cause. The patient was then given a trial treatment with dexamethasone with only mild improvement of his symptoms. Radiotherapy consisting of 2000 cGy given in 5 fractions was also initiated. On the third day of treatment, the final pathological diagnosis revealed poorly differentiated adenocarcinoma (Fig. 2). At the completion of radiotherapy, the patient experienced marked improvement of his symptoms. His third and fourth nerve palsies in both eyes had resolved. Only a residual left sixth nerve paresis remained. The patient was discharged to the care of his family; he died of systemic disease 6 weeks later.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 October 1992, Volume 31, Number 4 783 Acute Bilateral Ophthalmoplegia Secondary to Cavernous Sinus Metastasis: A Case Report Case Report
Received, August 29, 1991. Accepted, March 11, 1992. Reprint requests: William A. Friedman, M.D.,
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
vision can be spared when a calcified diagphragma sella limits the superior extent of an expanding sella (19) . Evidence for acute or chronic endocrinopathies and radiological abnormality of the sellar region suggest pituitary disease (2). Occlusive basilar-vertebral artery disease occasionally produces bilateral ophthalmoplegia. In a series of 12 patients with basilar-vertebral disease, Masucci (10) reported six cases of bilateral ocular muscle palsies of which two had complete ophthalmoplegia. Shutt et al. (16) have noted that the ophthalmoplegia is associated with fluctuating states of wakefulness, gait disturbance, hyperreflexia, and dysarthria, which aids localization within the neuraxis. No discussion of bilateral ocular palsies would be complete without mentioning Wernicke's encephalopathy. Patients classically present with bilateral abducens palsies, nystagmus, confabulation, and a history of ethanol abuse. Ptosis is not present. Improvement after the administration of thiamine is noted. Infectious processes can also produce symptoms through infiltration at the base of the skull. Mucormycosis (4) and tuberculous meningitis (20) have both been reported to cause progressive, painful, bilateral ophthalmoplegia. Evaluation of the cerebrospinal fluid for pleocytosis and evidence for the offending organism facilitate the diagnosis. Leptomeningeal seeding by metastatic cancer can exhibit multiple neurological deficits including ocular muscle dysfunction. In a series of 50 patients with meningeal carcinomatosis, Olson et al. (13) reported combined third and sixth nerve palsies in 12 patients. Ophthalmoplegia eventually resulted in 35 cases (13). Other nonspecific symptoms included headache, leg pains, back pain, and unsteadiness of gait (9). Cranial nerve involvement was usually a late manifestation (9). The most common malignancies reported were breast, lymphoma, lung, and melanoma (9,13) . Lumbar puncture is an essential tool in making the diagnosis, and several spinal taps are often necessary to demonstrate malignant cells through cytocentrifuge techniques. Other rare causes of acute bilateral ophthalmoplegia include basal sarcoidosis (20), diabetic neuropathy (15), and clivus chordoma (5). Hematogenous metastasis to both cavernous sinuses producing bilateral ophthalmoplegia can also be considered a rare phenomenon. Only one case has been previously reported. Mills et al. (11) noted the development of total bilateral ophthalmoplegia in a 59-year-old man after burr hole drainage of a subdural hematoma. Metastatic lung carcinoma was eventually diagnosed at necropsy. Our case, diagnosed antemortem, demonstrates that prompt recognition of this rare occurrence by neurosurgeons can enable the cancer patient to receive treatment that may reverse neurological deficits and improve quality of life.
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extremely rare cause among diseases producing bilateral ophthalmoplegia. There are a number of more common etiologies that should be considered when evaluating patients with this constellation of symptoms. Postinfectious polyneuritis (Guillain-Barré syndrome) can often produce acute bilateral ophthalmoplegia. The syndrome consists of a rapid, symmetric, ascending polyneuropathy frequently occurring several weeks after an acute infection. Fisher's syndrome, a variant that predominantly affects cranial nerves, produces ophthalmoplegia and bilateral facial paresis, with only mild limb weakness (3) . The paralysis may progress for 10 days before stabilizing. Among 60 patients with acute bilateral ophthalmoplegia, Keane (7) reported Guillain-Barré and Fisher's syndromes as the offending cause in 25 patients. He recommended observation for several days to document ophthalmoplegia, limb weakness, and loss of deep tendon reflexes. A demonstrated rise in cerebrospinal fluid protein also helps to ensure the diagnosis (7). Disorders of neuromuscular junction frequently produce bilateral ophthalmoplegia. Ocular muscle dysfunction is the initial presenting symptom in 75% of patients with myasthenia gravis (5). Diplopia and ptosis are the most common features that normally develop toward the end of the day and are most profound at night. The ophthalmoplegia produced is usually painless and invariably spares pupillary function (15). Intravenous edrophonium can alleviate ocular palsies and can easily confirm the diagnosis. Botulinum neurotoxin may also produce bilateral ophthalmoplegia and is difficult to discern from myasthenia gravis and Guillain-Barré syndrome. A history of ingestion of tainted food is crucial. Evidence for circulating neurotoxin and an absence of cerebrospinal fluid protein elevation facilitate the diagnosis (15). Additionally, botulism can present with accommodative paresis producing blurred vision. An examination may reveal mydriasis with poorly reactive pupils in addition to ptosis and extraocular muscle paresis (6,8,11,17). Dysthyroid myopathy commonly produces diplopia. However, ptosis is extremely rare, and the pupils are always spared (5). Evidence for lid retraction, proptosis, and a positive forced duction test leads one to the diagnosis. Chronic ocular myopathy, however, produces a symmetric, progressive immobility of the eyes (5). Ptosis is common, and the ophthalmoplegia produced is painless. Diplopia, interestingly, is infrequent. The course is usually slow without fluctuations or remissions, and a family history may be present (5). Pituitary apoplexy can produce bilateral ophthalmoplegia acutely. Symptoms result from hemorrhage into a pituitary adenoma that has extended into the cavernous sinuses. Bilateral total ophthalmoplegia resulting from this syndrome was first reported by Walsh in 1949 (19). More recently, Keane (7) reported 5 cases among his series of 60 patients. Typically, the onset is abrupt, with associated severe headache. Vision is often affected resulting in varying degrees of blindness. However,
REFERENCES: (1-20) 1.
3.
4. 5. 6.
7. 8. 9. 10. 11. 12.
13.
14.
15.
16.
18. 19.
20.
COMMENTS The cavernous sinus syndrome is characterized by ocular motor nerve palsies involving the third, fourth, and sixth cranial nerves as well as the first and second divisions of the fifth cranial sensory nerve (1). Involvement of the trigeminal nerve leads to the painful ophthalmoplegia commonly seen in this syndrome (3). When involvement of the fifth cranial nerve is absent or appears late in the course of this syndrome, an extrinsic process compressing the cavernous sinus is suggested (4). Additionally, the optic nerve may be affected intracranially, causing visual loss. Numerous causes for the cavernous sinus syndrome have been reported and fall into categories of tumors, aneurysms, and inflammatory lesions as well as vascular lesions (3). Metastatic lesions are frequently seen in the cavernous sinus syndrome but bilateral involvement is rarely associated with metastatic disease (2). In their case review, Supler et al. describe a rare presentation of bilateral hematogenous metastasis to the cavernous sinuses. They have also provided a thorough review of the literature on the subject of bilateral ophthalmoplegia in their discussion of differential diagnosis. Craig E. Geist Washington, District of Columbia REFERENCES: (1-4) 1.
2. 3.
4.
Bartholow R: Aneurysms of the arteries at the base of the brain: Their symptomatology, diagnosis and treatment. Am J Met Sci 64:373386, 1972. Keane JR: Acute bilateral ophthalmoplegia: 60 cases. Neurology 36:279-281, 1986. Miller NR: Topical diagnosis of neuropathic ocular motility disorders, in Walsh and Hoyt's Clinical Neuro-Ophthalmology. Baltimore, Williams & Wilkins, ed 4, 1985. Mills RP, Insalaco SJ, Joseph A: Bilateral cavernous sinus metastasis and ophthalmoplegia. J Neurosurg 55:463-466, 1981.
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/31/4/783/2752100 by Universitaetsbibliothek Muenchen user on 04 February 2019
2.
Ahmad K, Yamokoshi C, Kim Y, Post MJ, Fayos JV: Involvement of cavernous sinus region by malignant neoplasm: Report of five cases. J Am Optom Assoc 87:504-508, 1987. David NJ, Gargano FP, Glaser JS: Pituitary apoplexy in clinical perspective, in Glaser JS, Smith JL (eds): Neuro-Ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. St. Louis, CV Mosby, 1975, vol 8, pp 140-165. Fisher M: An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia, and areflexia) N Engl J Med 225:57-65, 1956. Gass JDM: Ocular manifestations of acute mucormycosis. Arch Ophthalmol 65:226-237, 1961. Glaser JS: Neuro-ophthalmology. Hagerstown, Harper and Row, 1978, pp 263281. Hedges TR, Jones A, Stark L, Hoyt WF: Botulin ophthalmoplegia: Clinical and oculographic observations. Arch Ophthalmol 101:211-213, 1983. Keane JR: Acute bilateral ophthalmoplegia: 60 cases. Neurology 36:279-281, 1986. Konig H, Gassman HB, Jenzer G: Ocular involvement in benign botulism B. Am J Ophthalmol 80:430-432, 1975. Little JR, Dale AJD, Okazaki H: Meningeal carcinomatosis: Clinical manifestations. Arch Neurol 30:138-143, 1974. Masucci EF: Bilateral ophthalmoplegia in basilar- vertebral artery disease. Brain 88:97106, 1965. Miller NR, Moses H: Occular involvement in wound botulism. Arch Ophthalmol 95:17881789, 1977. Mills RP, Insalaco SJ, Joseph A: Bilateral cavernous sinus metastasis and ophthalmoplegia. J Neurosurg 55:463-466, 1981. Olson ME, Chernik NL, Posner JB: Infiltration of the leptomeninges by systemic cancer: A clinical and pathologic study. Arch Neurol 30:122-137, 1974. Post MJ, Mendez DR, Kline LB, Acker JD, Glaser JS: Metastatic disease to the cavernous sinus: Clinical syndrome and CT diagnosis. J Comput Assist Tomogr 9:115-120, 1985. Sergott RC, Glaser JS, Berger LJ: Simultaneous, bilateral diabetic ophthalmoplegia: Report of two cases and discussion of differential diagnosis. Ophthalmology 91:18-21, 1984. Shutt HKR, David MJ, Smith JL: Complete bilateral III and IV nerve palsies due to basilar artery disease, in Smith JL (ed): NeuroOphthalmology: Symposium of the University
17.
of Miami and the Bascom Palmer Eye Institute. Hallandale, FL, Huffman, 1971, vol 5, pp 356-362. Terranova W, Palumbo JN, Breman JG: Ocular findings in botulism type B. JAMA 241:475-477, 1979. Thomas JE, Yoss RE: The parasellar syndrome: Problems in determining etiology. Mayo Clin Proc 45:617-623, 1970. Walsh FB: Bilateral total ophthalmoplegia with adenoma of the two pituitary glands: Report of two cases: An anatomic study. Arch Ophthalmol 42:646-654, 1949. Walsh FB, Hoyt WF: Clinical Neuro-Ophthalmology. Baltimore, Williams & Wilkins, 1969, ed 3, pp 1434-1440.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Department of Neurosurgery, Box J-265, JHMHC, University of Florida, Gainesville, FL 32610.
REFERENCES: (1) 1.
Richard BW, Jones FR, Younge BR: Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol 113:489496, 1992.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Brian R. Younge Rochester, Minnesota
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This article describes a patient with bilateral ophthalmoplegia that was caused by metastatic tumor to the cavernous sinus from adenocarcinoma of the colon. The proof of this was obtained antemortem by endoscopic sphenoidal biopsy and led to the patient being treated by irradiation, with near resolution of his symptoms. As pointed out by the authors, total bilateral ophthalmoplegia is not very common, and if we can assume the pupils were not involved, this makes for a very important exception to the general rule about third-nerve palsies and compressive lesions that spare the pupils. It is not entirely clear, however, that both pupils were totally uninvolved, and I would point out that such sparing might be overestimated in a bilateral involvement of third nerves, especially because the sympathetic pathways may well have not dilated the pupils much if they, too, were involved. The article provides a thorough review of multiple ocular motor palsies, specifically bilateral palsies, and I would point out that by far the majority of such cases imply a serious cause, most often tumor (1). In patients afflicted with multiple cranial nerve palsies, either uni- or bilateral ophthalmoplegia, a workup for the conditions mentioned in this article are mandatory. Recovery from such palsies is dependent on the ultimate cause, and as pointed out by the authors, palliative treatment is certainly worthy of consideration, especially in the more radiosensitive metastatic lesions, such as the adenocarcinomas of the breast or bowel.
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Figure 1. MAGNEVIST enhanced coronal T1weighted magnetic resonance imaging scan. Enhancement of both cavernous sinuses suggested a diffuse process. Enhancement within the sphenoid sinus was also noted.
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Figure 2. Photomicrograph of the sphenoid sinus lesion reveals poorly differentiated adenocarcinoma (hematoxylin and eosin, ×500).
