- Email: [email protected]
Diagnostic Principles in the Jugular Foramen Syndrome
Diagnostic Principles in the Jugular Foramen Syndrome MORRIS H. RIVERS, M.D. HENDRIK J. SVIEN, M.D. HILLIER L. BAKER. JR., M.D.
THE JUGULAR FORAMEN SYNDROME refers to the neurologic deficit produced when the function of nerves traversing the jugular foramen has been disturbed. Passing through the jugular foramen are, from anterior to posterior, the petrosal sinus; the glossopharyngeal, vagus, and spinal accessory nerves; the transverse sinus; and some meningeal branches from the occipital and ascending pharyngeal arteries. The hypoglossal nerve (cranial nerve XII) passes through the condylar foramen (hypoglossal canal), whose intracranial orifice is within 8 mm. of the posteromedial border of the jugular foramen; and consequently, a mass originating at the jugular foramen may involve the twelfth nerve even though it is relatively small. The extracranial end of the condylar foramen is separated from the extracranial end of the jugular foramen by only a thin edge of bone, usually less than a millimeter thick; consequently, an extracranial mass encroaching upon the jugular foramen, or a mass that passes through the jugular foramen, is very likely to involve the twelfth nerve as it emerges from the condylar foramen into the extracranial space. Also, extracranial masses near the jugular foramen may involve the cervical sympathetic chain, which is close to the last four cranial nerves at the base of the skull. Clinically, a true jugular foramen syndrome (also called Vernet's syndrome) consists of unilateral loss of taste on the posterior third of the tongue (glossopharyngeal nerve, cranial nerve IX) and paralysis of the vocal cord and palate (vagus nerve, cranial nerve X) and of the trapezius and sternocleidomastoid muscles (spinal accessory nerve, cranial nerve XI). Many other related syndromes, with involvement of one or more of the last four cranial nerves and the cervical sympathetic system with or without long-tract signs, are described in textbooks of neurology. Pertinent to this discussion are the posterior retropharyngeal syndrome (Villaret's syndrome) with involvement of cranial nerves IX, X, XI, and 1129
1130
M. H.
RIVERS,
H.
J. SVIEN,
H. L.
BAKER, JR.
XII, plus the cervical sympathetic system; posterior lacerocondylar syndrome (Collet-Sicard syndrome) with involvement of cranial nerves IX, X, XI, and XII; and Jackson's syndrome, which is produced by a unilateral nuclear or radicular lesion of cranial nerves X, XI, and XII. ETIOLOGY
The etiologic factors which may produce the syndromes described above are: tumors arising at the jugular foramen; extracranial tumors at the base of the skull; vascular lesions of the brain stem, which always produce long-tract signs as well as involvement of the cranial nerves; meningitis at the base of the skull; syphilitic involvement; tumors of the cerebellopontine angle with extension to the jugular foramen; abscess of the retropharyngeal space; aneurysm of the carotid artery at the base of the skull; and skull fracture in this region. Neurinomas of the last four cranial nerves might be expected to be one of the major causes of these syndromes; however, this type of tumor is not common. Columella and associates found only seven cases of neurinoma of the twelfth cranial nerve and seven cases of neurinoma of the ninth, tenth and eleventh cranial-nerve complex reported in the literature up to 1959. Williams and Fox in 1962 had similar findings. Neurinomas of the acoustic nerve (cranial nerve VIII) may involve also the ninth, tenth, eleventh, and twelfth nerves; but signs of involvement of these nerves always appear later than clinical manifestations of involvement of the eighth nerve and usually of the facial nerve (cranial nerve VII) as well. Pool and Pava, in discussing 122 cases of neurinoma of the acoustic nerve, reported involvement of the ninth and tenth cranial nerves in 28 cases, of the eleventh cranial nerve in four cases, and of the twelfth cranial nerve in seven cases-but only after involvement of the eighth cranial nerve in all cases. An intracranial epidermoid tumor that affected the tenth, eleventh, and twelfth cranial nerves was described by Love in 1951. Meningiomas arising near the jugular foramen also may produce the syndrome. Extracranial tumors that damage the last four cranial nerves may be primary or secondary. A frequent primary tumor in this region is one of the glomus jugulare. Siekert reviewed a series of 33 such tumors and found involvement of the ninth, tenth, eleventh, and twelfth cranial nerves in about a third of the cases (but never as the first sign). Other cranial nerves also were involved in all cases. Extracranially, metastatic carcinoma is much more common than any primary tumor, although Winborn and associates found approximately 50 cases of extracranial neurinoma of the parapharyngeal space reported in the literature. Many times it is difficult or impossible to arrive at a distinct etiologic diagnosis of the jugular foramen syndrome. Vascular lesions often can be diagnosed after a careful history and examination. Because of the
Diagnostic Principles in the Jugular Foramen Syndrome
1131
proximity of the cranial-nerve nuclei to long tracts running through the medulla, pons, and brain stem, it is almost impossible to conceive of a vascular lesion in this location producing isolated lower cranial-nerve palsies without producing long-tract signs as well. Therefore the presence of lower cranial-nerve signs and symptoms in association with long-tract signs indicates that the patient has an intramedullary tumor or vascular lesion in the brain stem or a large mass in the posterior fossa compressing the long tracts. In the latter instance, it is more than likely that the seventh and eighth, and possibly the fifth, cranial nerves would be involved as well as the last four cranial nerves. Syphilis has been implicated as a cause of this syndrome. Although unproved, it is possible also that the manifestations of this syndrome may be the result of diabetic involvement of the nerves in question. DIAGNOSTIC METHODS
The history of the patient's condition is often of extreme importance. An intracranial mass at the jugular foramen frequently produces signs of progressive involvement of first one nerve, then another, and finally the complete syndrome. A vascular lesion of the brain stem is usually of much more rapid onset and invariably is accompanied by long-tract signs. Resolution of this type of lesion is usually slow; the patient may not recover for months. Inflammatory lesions ordinarily are accompanied by other symptoms of meningitis at the onset. A general physical examination, as well as a careful neurologic examination, should be performed. This general examination should be complete and should stress search for a distant malignant lesion, cervicalnode enlargement, retropharyngeal tumor, and abnormalities of the nasopharynx. (Primary nasopharyngeal malignant lesions are not rare.) The presence of a retropharyngeal mass, Horner's syndrome, or both, helps to localize the cause of the jugular foramen syndrome to the extracranial region. The neurologic examination must be complete in order to rule out cerebellar signs, long-tract signs, and signs of involvement of adjacent cranial nerves (VIII, VII, V, and so forth) which would suggest a source other than the jugular foramen or an extension from this location of the etiologic factor. The presence or absence of papilledema is extremely important, inasmuch as papilledema and signs of increased intracranial pressure are rare in cases of tumor limited to the last four cranial nerves. Complete urinalysis, routine blood examinations, and chest and skull roentgenograms including basal views (for the presence or absence of erosion of the jugular foramen and evidence suggesting an extracranial mass) should be made in each case. The presence of a retropharyngeal or cervical mass may dictate the next step in evaluation of the patient. Direct surgical attack on such a
1132
M. H.
RIVERS,
H.
J. SVIEN,
H. L.
BAKER, JR.
mass may be indicated for diagnosis or treatment. A meningioma originating in the posterior fossa has been known to present as a mass in the neck, so the surgical team may have to consist of a neurosurgeon and a general surgeon experienced in operating in the cervical retropharyngeal space. In the event that a retropharyngeal mass produces bulging into the nasopharynx, a biopsy specimen can be obtained through the nasopharyngeal wall. Carotid angiography is useful in demonstrating extracranial masses, including a rare extracranial carotid aneurysm. When an extracranial retropharyngeal mass is present, the carotid artery usually is displaced from its normal location in the neck; and a picture showing this may be of great help to the surgeon in locating the mass. Intracranial masses producing the jugular foramen syndrome may be much more difficult to diagnose, particularly when of small size. The method of contrast study most commonly used is fractional pneumoencephalography, in which films are taken of the posterior fossa as air is introduced into the lumbar subarachnoid space. Shift or deformity of the normal structures of the posterior fossa may be seen, or the tumor may be outlined by air in the basal subarachnoid space and cistern. The use of positive contrast media such as iophendylate (Pantopaque) in the subarachnoid space seems to offer a good chance of successful demonstration of intracranial lesions at the jugular foramen. In some cases it is impossible to arrive at a definite etiologic diagnosis; especially in these cases any systemic disease such as diabetes should be treated vigorously, because it is possible that the disease may be an etiologic factor. Neurologic recheck examinations at regular intervals should be advised. SUMMARY
The jugular foramen syndrome is produced by involvement of the ninth, tenth, and eleventh cranial nerves unilaterally. Symptoms of the syndrome are unilateral loss of taste on the posterior third of the tongue and paralysis of the vocal cord, palate, and trapezius and sternocleidomastoid muscles. Etiologic factors in the production of this and related symptoms are: thrombosis of the jugular bulb at the jugular foramen; primary or metastatic intracranial or extracranial tumors; trauma; meningitis; vascular lesions, abscess, and infections of the retropharyngeal space; aneurysms of the carotid artery; and, possibly, diabetic neuritis. Evaluation of a patient with the jugular foramen syndrome should begin with a complete physical and neurologic examination, routine laboratory tests, and skull roentgenograms including basal views. Extracranial masses may be quite accessible to surgical biopsy and removal; they often are well outlined by carotid angiography. Investigation of intracranial lesions in the posterior fossa may consist
Diagnostic Principles in the Jugular Foramen Syndrome
1133
of pneumoencephalography, contrast roentgenography with iophendylate, or direct exploration of the posterior fossa. Patients in whom a positive etiologic diagnosis cannot be made should have frequent re-evaluation. REFERENCES 1. Columella, F., Delzanno, G. B. and Nicola, G. C.: Les neurinomes des quatre derniers nerfs craniens. Neurochirurgie. 5: 280-295, 1959. 2. Love, J. G.: Jugular foramen syndrome (Jackson's syndrome) due to intracranial epidermoid tumor: Successful surgical treatment. Proc. Staff Meet., Mttyo Clin. 26: 252-256 (June 20) 1951. 3. Pool, J. L. and Pava, A. A.: Early Diagnosis and Treatment of Acoustic Nerve Tumors. Springfield, Illinois, Charles C Thomas, Publisher, 1957, pp. 36-38. 4. Siekert, R. G.: Neurologic manifestations of tumors of glomus jugulare: Chemodectoma, nonchromaffin paraganglioma or carotid-body-like tumor. A.M.A. Arch. Neurol. & Psychiat. 76: 1-13 (July) 1956. 5. Williams, J. M. and Fox, J. L.: Neurinoma of intracranial portion of hypoglossal nerve: Review and case report. J. Neurosurg. 19: 248-250 (March) 1962. 6. Winborn, C. D., Martinez, D. M., Holding, B. F., Jr. and Hutcheson, J. B.: Neurilemmoma of hypoglossal nerve: Report of case. A.M.A. Arch. Otolaryng. 69: 583-588 (May) 1959.
THE JUGULAR FORAMEN SYNDROME refers to the neurologic deficit produced when the function of nerves traversing the jugular foramen has been disturbed. Passing through the jugular foramen are, from anterior to posterior, the petrosal sinus; the glossopharyngeal, vagus, and spinal accessory nerves; the transverse sinus; and some meningeal branches from the occipital and ascending pharyngeal arteries. The hypoglossal nerve (cranial nerve XII) passes through the condylar foramen (hypoglossal canal), whose intracranial orifice is within 8 mm. of the posteromedial border of the jugular foramen; and consequently, a mass originating at the jugular foramen may involve the twelfth nerve even though it is relatively small. The extracranial end of the condylar foramen is separated from the extracranial end of the jugular foramen by only a thin edge of bone, usually less than a millimeter thick; consequently, an extracranial mass encroaching upon the jugular foramen, or a mass that passes through the jugular foramen, is very likely to involve the twelfth nerve as it emerges from the condylar foramen into the extracranial space. Also, extracranial masses near the jugular foramen may involve the cervical sympathetic chain, which is close to the last four cranial nerves at the base of the skull. Clinically, a true jugular foramen syndrome (also called Vernet's syndrome) consists of unilateral loss of taste on the posterior third of the tongue (glossopharyngeal nerve, cranial nerve IX) and paralysis of the vocal cord and palate (vagus nerve, cranial nerve X) and of the trapezius and sternocleidomastoid muscles (spinal accessory nerve, cranial nerve XI). Many other related syndromes, with involvement of one or more of the last four cranial nerves and the cervical sympathetic system with or without long-tract signs, are described in textbooks of neurology. Pertinent to this discussion are the posterior retropharyngeal syndrome (Villaret's syndrome) with involvement of cranial nerves IX, X, XI, and 1129
1130
M. H.
RIVERS,
H.
J. SVIEN,
H. L.
BAKER, JR.
XII, plus the cervical sympathetic system; posterior lacerocondylar syndrome (Collet-Sicard syndrome) with involvement of cranial nerves IX, X, XI, and XII; and Jackson's syndrome, which is produced by a unilateral nuclear or radicular lesion of cranial nerves X, XI, and XII. ETIOLOGY
The etiologic factors which may produce the syndromes described above are: tumors arising at the jugular foramen; extracranial tumors at the base of the skull; vascular lesions of the brain stem, which always produce long-tract signs as well as involvement of the cranial nerves; meningitis at the base of the skull; syphilitic involvement; tumors of the cerebellopontine angle with extension to the jugular foramen; abscess of the retropharyngeal space; aneurysm of the carotid artery at the base of the skull; and skull fracture in this region. Neurinomas of the last four cranial nerves might be expected to be one of the major causes of these syndromes; however, this type of tumor is not common. Columella and associates found only seven cases of neurinoma of the twelfth cranial nerve and seven cases of neurinoma of the ninth, tenth and eleventh cranial-nerve complex reported in the literature up to 1959. Williams and Fox in 1962 had similar findings. Neurinomas of the acoustic nerve (cranial nerve VIII) may involve also the ninth, tenth, eleventh, and twelfth nerves; but signs of involvement of these nerves always appear later than clinical manifestations of involvement of the eighth nerve and usually of the facial nerve (cranial nerve VII) as well. Pool and Pava, in discussing 122 cases of neurinoma of the acoustic nerve, reported involvement of the ninth and tenth cranial nerves in 28 cases, of the eleventh cranial nerve in four cases, and of the twelfth cranial nerve in seven cases-but only after involvement of the eighth cranial nerve in all cases. An intracranial epidermoid tumor that affected the tenth, eleventh, and twelfth cranial nerves was described by Love in 1951. Meningiomas arising near the jugular foramen also may produce the syndrome. Extracranial tumors that damage the last four cranial nerves may be primary or secondary. A frequent primary tumor in this region is one of the glomus jugulare. Siekert reviewed a series of 33 such tumors and found involvement of the ninth, tenth, eleventh, and twelfth cranial nerves in about a third of the cases (but never as the first sign). Other cranial nerves also were involved in all cases. Extracranially, metastatic carcinoma is much more common than any primary tumor, although Winborn and associates found approximately 50 cases of extracranial neurinoma of the parapharyngeal space reported in the literature. Many times it is difficult or impossible to arrive at a distinct etiologic diagnosis of the jugular foramen syndrome. Vascular lesions often can be diagnosed after a careful history and examination. Because of the
Diagnostic Principles in the Jugular Foramen Syndrome
1131
proximity of the cranial-nerve nuclei to long tracts running through the medulla, pons, and brain stem, it is almost impossible to conceive of a vascular lesion in this location producing isolated lower cranial-nerve palsies without producing long-tract signs as well. Therefore the presence of lower cranial-nerve signs and symptoms in association with long-tract signs indicates that the patient has an intramedullary tumor or vascular lesion in the brain stem or a large mass in the posterior fossa compressing the long tracts. In the latter instance, it is more than likely that the seventh and eighth, and possibly the fifth, cranial nerves would be involved as well as the last four cranial nerves. Syphilis has been implicated as a cause of this syndrome. Although unproved, it is possible also that the manifestations of this syndrome may be the result of diabetic involvement of the nerves in question. DIAGNOSTIC METHODS
The history of the patient's condition is often of extreme importance. An intracranial mass at the jugular foramen frequently produces signs of progressive involvement of first one nerve, then another, and finally the complete syndrome. A vascular lesion of the brain stem is usually of much more rapid onset and invariably is accompanied by long-tract signs. Resolution of this type of lesion is usually slow; the patient may not recover for months. Inflammatory lesions ordinarily are accompanied by other symptoms of meningitis at the onset. A general physical examination, as well as a careful neurologic examination, should be performed. This general examination should be complete and should stress search for a distant malignant lesion, cervicalnode enlargement, retropharyngeal tumor, and abnormalities of the nasopharynx. (Primary nasopharyngeal malignant lesions are not rare.) The presence of a retropharyngeal mass, Horner's syndrome, or both, helps to localize the cause of the jugular foramen syndrome to the extracranial region. The neurologic examination must be complete in order to rule out cerebellar signs, long-tract signs, and signs of involvement of adjacent cranial nerves (VIII, VII, V, and so forth) which would suggest a source other than the jugular foramen or an extension from this location of the etiologic factor. The presence or absence of papilledema is extremely important, inasmuch as papilledema and signs of increased intracranial pressure are rare in cases of tumor limited to the last four cranial nerves. Complete urinalysis, routine blood examinations, and chest and skull roentgenograms including basal views (for the presence or absence of erosion of the jugular foramen and evidence suggesting an extracranial mass) should be made in each case. The presence of a retropharyngeal or cervical mass may dictate the next step in evaluation of the patient. Direct surgical attack on such a
1132
M. H.
RIVERS,
H.
J. SVIEN,
H. L.
BAKER, JR.
mass may be indicated for diagnosis or treatment. A meningioma originating in the posterior fossa has been known to present as a mass in the neck, so the surgical team may have to consist of a neurosurgeon and a general surgeon experienced in operating in the cervical retropharyngeal space. In the event that a retropharyngeal mass produces bulging into the nasopharynx, a biopsy specimen can be obtained through the nasopharyngeal wall. Carotid angiography is useful in demonstrating extracranial masses, including a rare extracranial carotid aneurysm. When an extracranial retropharyngeal mass is present, the carotid artery usually is displaced from its normal location in the neck; and a picture showing this may be of great help to the surgeon in locating the mass. Intracranial masses producing the jugular foramen syndrome may be much more difficult to diagnose, particularly when of small size. The method of contrast study most commonly used is fractional pneumoencephalography, in which films are taken of the posterior fossa as air is introduced into the lumbar subarachnoid space. Shift or deformity of the normal structures of the posterior fossa may be seen, or the tumor may be outlined by air in the basal subarachnoid space and cistern. The use of positive contrast media such as iophendylate (Pantopaque) in the subarachnoid space seems to offer a good chance of successful demonstration of intracranial lesions at the jugular foramen. In some cases it is impossible to arrive at a definite etiologic diagnosis; especially in these cases any systemic disease such as diabetes should be treated vigorously, because it is possible that the disease may be an etiologic factor. Neurologic recheck examinations at regular intervals should be advised. SUMMARY
The jugular foramen syndrome is produced by involvement of the ninth, tenth, and eleventh cranial nerves unilaterally. Symptoms of the syndrome are unilateral loss of taste on the posterior third of the tongue and paralysis of the vocal cord, palate, and trapezius and sternocleidomastoid muscles. Etiologic factors in the production of this and related symptoms are: thrombosis of the jugular bulb at the jugular foramen; primary or metastatic intracranial or extracranial tumors; trauma; meningitis; vascular lesions, abscess, and infections of the retropharyngeal space; aneurysms of the carotid artery; and, possibly, diabetic neuritis. Evaluation of a patient with the jugular foramen syndrome should begin with a complete physical and neurologic examination, routine laboratory tests, and skull roentgenograms including basal views. Extracranial masses may be quite accessible to surgical biopsy and removal; they often are well outlined by carotid angiography. Investigation of intracranial lesions in the posterior fossa may consist
Diagnostic Principles in the Jugular Foramen Syndrome
1133
of pneumoencephalography, contrast roentgenography with iophendylate, or direct exploration of the posterior fossa. Patients in whom a positive etiologic diagnosis cannot be made should have frequent re-evaluation. REFERENCES 1. Columella, F., Delzanno, G. B. and Nicola, G. C.: Les neurinomes des quatre derniers nerfs craniens. Neurochirurgie. 5: 280-295, 1959. 2. Love, J. G.: Jugular foramen syndrome (Jackson's syndrome) due to intracranial epidermoid tumor: Successful surgical treatment. Proc. Staff Meet., Mttyo Clin. 26: 252-256 (June 20) 1951. 3. Pool, J. L. and Pava, A. A.: Early Diagnosis and Treatment of Acoustic Nerve Tumors. Springfield, Illinois, Charles C Thomas, Publisher, 1957, pp. 36-38. 4. Siekert, R. G.: Neurologic manifestations of tumors of glomus jugulare: Chemodectoma, nonchromaffin paraganglioma or carotid-body-like tumor. A.M.A. Arch. Neurol. & Psychiat. 76: 1-13 (July) 1956. 5. Williams, J. M. and Fox, J. L.: Neurinoma of intracranial portion of hypoglossal nerve: Review and case report. J. Neurosurg. 19: 248-250 (March) 1962. 6. Winborn, C. D., Martinez, D. M., Holding, B. F., Jr. and Hutcheson, J. B.: Neurilemmoma of hypoglossal nerve: Report of case. A.M.A. Arch. Otolaryng. 69: 583-588 (May) 1959.