Neuroradiology (1992) 34:417-419
Neuro--
radiology 9 Springer-Verlag 1992
Accessory nerve neuroma presenting as recurrent jugular foramen syndrome H. Sawada 1, F. Udaka 1, M. Kameyama 1, K. Iwasaki 2, and A. Kondou 2 1Department of Neurology, Sumitomo Hospital and 2Department of Neurosurgery, Kitano Hospital, Osaka, Japan Received: 24 December 1991
Summary. We report a patient with spontaneous recovery and recurrence of a jugular foramen syndrome secondary to an accessory nerve neuroma. He showed sudden onset of unilateral palsy of the ninth, tenth and eleventh cranial nerves in 1982. H e recovered almost fully and in 1990 the palsies recurred. MRI revealed a small mass in the right jugular foramen. The tumour was resected via suboccipital craniectomy, and diagnosed as a neuroma of the eleventh cranial nerve.
Key words: Neuroma - Jugular foramen syndrome - Vernet syndrome
The jugular foramen syndrome (JFS) is unilateral palsy of the ninth, tenth and eleventh cranial nerves, which exit through the jugular foramen. Primary and metastatic tumours in the jugular foramen, trauma and inflammation at the base of the skull have resulted in this syndrome [1-4]. JFS with spontaneous recovery is thought to be caused by benign inflammatory cranial polyneuropathy [5]. We report a patient who presented with spontaneous recovery and recurrence of a JFS resulting from an accessory nerve neuroma.
Case report In 1982, a 40-year-old man developed sudden hoarseness and difficulty in swallowing and in raising his right arm. He was found to have palsies of the right ninth and tenth cranial nerves. Skull base radiography was normal. Cerebrospinal fluid examination revealed no abnormal findings. The hoarseness and dysphagia disappeared after a few months, and the weakness of his right arm recovered almost completely. On 24 January 1990, the patient experienced an itching sensation in the right external auditory meatus. On 28 January he felt a tingling sensation in the throat, and on 29
January developed hoarseness and aspiration and was admitted to hospital. Examination on admission was as follows. The pupils were equal and the light reflex was brisk and complete. Ocular movements were smooth and showed a full range. He had no blepharoptosis. Homer's sign was negative. Motor and sensory functions of the trigeminal nerves were intact. The muscular strength of eye closure and mouth closure was full. Hearing acuity was normal, and Weber's and Rinne's tests were negative. The right soft palate moved poorly and showed tactile hypoaesthesia. His voice was hoarse. Tactile sensation was intact in both external auditory meatus. The right sternocleidomastoid and trapezius muscles showed atrophy with reduced strength of 3/5 or 4/5 according to manual muscle testing. The patient found it difficult to raise his right arm because of weakness of the right trapezius (Fig. 1). Manual muscle testing showed normal strength, except for elevation of the right arm. Deep tendon reflexes of the limbs were normal and no pathological reflexes were present. Sensation and coordination were normal. Blood cell counts and serum laboratory investigations were normal. The lumbar spinal fluid contained 2 mononuclear cells/ml, and its protein concentration was 27.7 mg/dl. Needle electromyography performed on the middle lobe of the right trapezius showed long duration polyphasic units with a reduction in the number of units. The right deltoid was normal. Plain films of the skull base were normal. X-ray CT showed no abnormality except for equivocal enlargement of the right jugular foramen. Magnetic resonance imaging of the head revealed a mass, whose rim enhanced with gadolinium, at the right jugular foramen (Fig. 2 a, b). At surgery, the tumour was found to extend from the eleventh cranial nerve in the jugular foramen into the posterior cranial fossa (Fig. 3). The tumour was resected, and histologically diagnosed as a neuroma (Fig.4). Microscopic examination showed no a hemorrhage or cellular infiltration.
418
Fig. 1. The patient with both arms raised. On the right, the supraspinatus (S) contracted strongly, while the trapezius (T) was atrophic
foramen. The tumour grew from the eleventh cranial nerve, and compressed the adjacent ninth and tenth nerves
Fig.2. T2-weighted NRI (a) and coronal Tl-weighted image enhanced with gadolinium (b). The tumour lies between the internal jugular vein and vertebral artery (arrows)
Fig. 4. Light microscopy shows a neuroma with a pallisading pattern with no microhaemorrhage or haemosiderin deposition (haematoxylin and eosin, original magnification, x 100)
Fig,3. Suboccipital craniectomy. A posterolateral view of the right ninth tenth and eleventh cranial nerves emerging through the jugular
After the operation, weakness and atrophy of the right trapezius recovered and the patient could lift his right arm without difficulty.
Discussion M o t o r and sensory disturbance of the soft palate, itching in the external auditory meatus, and tingling in the throat indicated a ninth cranial nerve lesion. Hoarseness resulted from vocal cord dysfunction due to a tenth cranial nerve palsy while weakness and atrophy of the sternocleidomastoid and trapezius muscles indicated an accessory nerve lesion. These clinical features established the clinical diagnosis of a JFS [6]. The JFS of Vernet has two variants; the lacerocondylar syndrome of Collet-Sicard consists of unilateral palsy of cranial nerves I X to X I I without H o m e r ' s sign, whereas the retropharyngeal syndrome of Villaret includes
H o m e r ' s sign. The most c o m m o n a etiology of JFS (Vernet) is reported to be not tumour but an idiopathic disorder, whereas in the lacerocondylar and retropharyngeal syndromes, primary or metastatic tumours are the most c o m m o n cause [7]. In a JFS resulting from idiopathic benign disorders the cranial nerve palsies occur suddenly and recover fully or partially over several months. Plain films or CT of the skull base fail to demonstrate any abnormal findings [8, 9]. Thus, on the basis of clinical and laboratory findings, the patient could have b e e n diagnosed as having idiopathic benign JFS. However, M R I revealed a n e u r o m a of the eleventh cranial nerve. Although the tumour was so small that we remain uncertain whether it contributed to his first episode of JFS, the recurrent JFS was attributable to the t u m o r b e c a u s e surgical resection resulted in resolution of the clinical manifestations. The possible mechanisms of the recurrence of JFS include (1) m i c r o h a e m o r r h a g e into the tumour, (2) coin-
419 cidental inflammation at the jugular f o r a m e n , (3) microinfarction of the nerve, or (4) mechanical compression. T h e cerebrospinal fluid s h o w e d no i n f l a m m a t o r y changes and histological examination of the t u m o u r revealed no evidence of m i c r o h a e m o r r h a g e or acute inflammation. T h e precise m e c h a n i s m is therefore uncertain. In patients with JFS, even in those with s p o n t a n e o u s recovery, we m u s t consider the possibility of a n e u r o m a and we r e c o m m e n d m a g n e t i c r e s o n a n c e imaging of the skull base.
References 1. Svien HJ, Baker HL, Rivers MH (1963) Jugular foramen syndrome and allied syndromes. Neurology 13:797-89 2. Lee SH, Osborn MA, Buchheit WA (1976) Glioma of the jugular foramen. J Neurosurg 44:493-495 3. Boileau MA, Grotta JC, Borit A, Linden C van der, Nath A, Ostrow R Kopaniky D (1987) Metastatic renal cell carcinoma simulating glumus jugulare tumor. J Surg Onco135:201-203
4. Wilson H, Johnson DH (1984) Jugular foramen syndrome as a comlication of metastatic cancer of the prostate. South Med J 77: 92-93 5. Font JH (1952) The jugular foramen syndrome - evidence that transient cases may be of viral origin. AMA Arch Otolaryngo156: 134-141 6. Robbins KT, Fenton RS (1980) Jugular foramen syndrome. J Otolaryngol 9:505-516 7. Tanaka M, lsaka K, Morimatsu M, Hirai S (1983) Jugular foramen syndrome. Neurology 32:119-120 8. Fukui T, Ohta K, Hoshino M, Kobayashi I, Maruyama S (1983) A case of Villaret syndrome of unknown origin with benign course. Clin Neurol (Tokyo) 23:744-748 9. Tanaka M, Hirai S, Okamoto K, Morimatsu M, Yamaguchi H (1987) Benign and idiopathic jugular foramen syndrome - an etiological consideration. Clin Neurol (Tokyo) 27:329-333 Dr. H. Sawada Department of Neurology Sumitomo Hospital 5-2-2 Nakanoshima, Kita-ku Osaka, Japan
Neuro--
radiology 9 Springer-Verlag 1992
Accessory nerve neuroma presenting as recurrent jugular foramen syndrome H. Sawada 1, F. Udaka 1, M. Kameyama 1, K. Iwasaki 2, and A. Kondou 2 1Department of Neurology, Sumitomo Hospital and 2Department of Neurosurgery, Kitano Hospital, Osaka, Japan Received: 24 December 1991
Summary. We report a patient with spontaneous recovery and recurrence of a jugular foramen syndrome secondary to an accessory nerve neuroma. He showed sudden onset of unilateral palsy of the ninth, tenth and eleventh cranial nerves in 1982. H e recovered almost fully and in 1990 the palsies recurred. MRI revealed a small mass in the right jugular foramen. The tumour was resected via suboccipital craniectomy, and diagnosed as a neuroma of the eleventh cranial nerve.
Key words: Neuroma - Jugular foramen syndrome - Vernet syndrome
The jugular foramen syndrome (JFS) is unilateral palsy of the ninth, tenth and eleventh cranial nerves, which exit through the jugular foramen. Primary and metastatic tumours in the jugular foramen, trauma and inflammation at the base of the skull have resulted in this syndrome [1-4]. JFS with spontaneous recovery is thought to be caused by benign inflammatory cranial polyneuropathy [5]. We report a patient who presented with spontaneous recovery and recurrence of a JFS resulting from an accessory nerve neuroma.
Case report In 1982, a 40-year-old man developed sudden hoarseness and difficulty in swallowing and in raising his right arm. He was found to have palsies of the right ninth and tenth cranial nerves. Skull base radiography was normal. Cerebrospinal fluid examination revealed no abnormal findings. The hoarseness and dysphagia disappeared after a few months, and the weakness of his right arm recovered almost completely. On 24 January 1990, the patient experienced an itching sensation in the right external auditory meatus. On 28 January he felt a tingling sensation in the throat, and on 29
January developed hoarseness and aspiration and was admitted to hospital. Examination on admission was as follows. The pupils were equal and the light reflex was brisk and complete. Ocular movements were smooth and showed a full range. He had no blepharoptosis. Homer's sign was negative. Motor and sensory functions of the trigeminal nerves were intact. The muscular strength of eye closure and mouth closure was full. Hearing acuity was normal, and Weber's and Rinne's tests were negative. The right soft palate moved poorly and showed tactile hypoaesthesia. His voice was hoarse. Tactile sensation was intact in both external auditory meatus. The right sternocleidomastoid and trapezius muscles showed atrophy with reduced strength of 3/5 or 4/5 according to manual muscle testing. The patient found it difficult to raise his right arm because of weakness of the right trapezius (Fig. 1). Manual muscle testing showed normal strength, except for elevation of the right arm. Deep tendon reflexes of the limbs were normal and no pathological reflexes were present. Sensation and coordination were normal. Blood cell counts and serum laboratory investigations were normal. The lumbar spinal fluid contained 2 mononuclear cells/ml, and its protein concentration was 27.7 mg/dl. Needle electromyography performed on the middle lobe of the right trapezius showed long duration polyphasic units with a reduction in the number of units. The right deltoid was normal. Plain films of the skull base were normal. X-ray CT showed no abnormality except for equivocal enlargement of the right jugular foramen. Magnetic resonance imaging of the head revealed a mass, whose rim enhanced with gadolinium, at the right jugular foramen (Fig. 2 a, b). At surgery, the tumour was found to extend from the eleventh cranial nerve in the jugular foramen into the posterior cranial fossa (Fig. 3). The tumour was resected, and histologically diagnosed as a neuroma (Fig.4). Microscopic examination showed no a hemorrhage or cellular infiltration.
418
Fig. 1. The patient with both arms raised. On the right, the supraspinatus (S) contracted strongly, while the trapezius (T) was atrophic
foramen. The tumour grew from the eleventh cranial nerve, and compressed the adjacent ninth and tenth nerves
Fig.2. T2-weighted NRI (a) and coronal Tl-weighted image enhanced with gadolinium (b). The tumour lies between the internal jugular vein and vertebral artery (arrows)
Fig. 4. Light microscopy shows a neuroma with a pallisading pattern with no microhaemorrhage or haemosiderin deposition (haematoxylin and eosin, original magnification, x 100)
Fig,3. Suboccipital craniectomy. A posterolateral view of the right ninth tenth and eleventh cranial nerves emerging through the jugular
After the operation, weakness and atrophy of the right trapezius recovered and the patient could lift his right arm without difficulty.
Discussion M o t o r and sensory disturbance of the soft palate, itching in the external auditory meatus, and tingling in the throat indicated a ninth cranial nerve lesion. Hoarseness resulted from vocal cord dysfunction due to a tenth cranial nerve palsy while weakness and atrophy of the sternocleidomastoid and trapezius muscles indicated an accessory nerve lesion. These clinical features established the clinical diagnosis of a JFS [6]. The JFS of Vernet has two variants; the lacerocondylar syndrome of Collet-Sicard consists of unilateral palsy of cranial nerves I X to X I I without H o m e r ' s sign, whereas the retropharyngeal syndrome of Villaret includes
H o m e r ' s sign. The most c o m m o n a etiology of JFS (Vernet) is reported to be not tumour but an idiopathic disorder, whereas in the lacerocondylar and retropharyngeal syndromes, primary or metastatic tumours are the most c o m m o n cause [7]. In a JFS resulting from idiopathic benign disorders the cranial nerve palsies occur suddenly and recover fully or partially over several months. Plain films or CT of the skull base fail to demonstrate any abnormal findings [8, 9]. Thus, on the basis of clinical and laboratory findings, the patient could have b e e n diagnosed as having idiopathic benign JFS. However, M R I revealed a n e u r o m a of the eleventh cranial nerve. Although the tumour was so small that we remain uncertain whether it contributed to his first episode of JFS, the recurrent JFS was attributable to the t u m o r b e c a u s e surgical resection resulted in resolution of the clinical manifestations. The possible mechanisms of the recurrence of JFS include (1) m i c r o h a e m o r r h a g e into the tumour, (2) coin-
419 cidental inflammation at the jugular f o r a m e n , (3) microinfarction of the nerve, or (4) mechanical compression. T h e cerebrospinal fluid s h o w e d no i n f l a m m a t o r y changes and histological examination of the t u m o u r revealed no evidence of m i c r o h a e m o r r h a g e or acute inflammation. T h e precise m e c h a n i s m is therefore uncertain. In patients with JFS, even in those with s p o n t a n e o u s recovery, we m u s t consider the possibility of a n e u r o m a and we r e c o m m e n d m a g n e t i c r e s o n a n c e imaging of the skull base.
References 1. Svien HJ, Baker HL, Rivers MH (1963) Jugular foramen syndrome and allied syndromes. Neurology 13:797-89 2. Lee SH, Osborn MA, Buchheit WA (1976) Glioma of the jugular foramen. J Neurosurg 44:493-495 3. Boileau MA, Grotta JC, Borit A, Linden C van der, Nath A, Ostrow R Kopaniky D (1987) Metastatic renal cell carcinoma simulating glumus jugulare tumor. J Surg Onco135:201-203
4. Wilson H, Johnson DH (1984) Jugular foramen syndrome as a comlication of metastatic cancer of the prostate. South Med J 77: 92-93 5. Font JH (1952) The jugular foramen syndrome - evidence that transient cases may be of viral origin. AMA Arch Otolaryngo156: 134-141 6. Robbins KT, Fenton RS (1980) Jugular foramen syndrome. J Otolaryngol 9:505-516 7. Tanaka M, lsaka K, Morimatsu M, Hirai S (1983) Jugular foramen syndrome. Neurology 32:119-120 8. Fukui T, Ohta K, Hoshino M, Kobayashi I, Maruyama S (1983) A case of Villaret syndrome of unknown origin with benign course. Clin Neurol (Tokyo) 23:744-748 9. Tanaka M, Hirai S, Okamoto K, Morimatsu M, Yamaguchi H (1987) Benign and idiopathic jugular foramen syndrome - an etiological consideration. Clin Neurol (Tokyo) 27:329-333 Dr. H. Sawada Department of Neurology Sumitomo Hospital 5-2-2 Nakanoshima, Kita-ku Osaka, Japan
