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An intraneural ganglion cyst causing unilateral hypoglossal nerve palsy
Journal of Clinical Neuroscience 18 (2011) 1114–1115
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Reports
An intraneural ganglion cyst causing unilateral hypoglossal nerve palsy Shanu Gambhir ⇑, Asim Mujic, Andrew Hunn Department of Neurosurgery, Royal Hobart Hospital, 48 Liverpool Street, Hobart, Tasmania 7000, Australia
a r t i c l e
i n f o
Article history: Received 3 December 2010 Accepted 21 December 2010
Keywords: Hypoglossal nerve Intraneural ganglion Palsy Synovial cyst
a b s t r a c t Intracranial ganglion cysts are rare. We report a patient with a rare unilateral hypoglossal nerve palsy caused by an intraneural ganglion cyst. To our knowledge, there are only four reports of ganglion/synovial cysts causing unilateral hypoglossal nerve palsy. Our aim is to present the fifth report, and to compare our findings with the others. Crown Copyright Ó 2011 Published by Elsevier Ltd. All rights reserved.
1. Introduction We report a patient with a rare unilateral hypoglossal nerve palsy caused by an intraneural ganglion cyst. Most intraneural ganglion cysts are found in peripheral nerves near joints or tendon sheaths.1 The most common location of intraneural ganglion cysts is in the peroneal nerve behind the fibular head.2–4 These cysts can become symptomatic due to compression and invasion of adjacent nerves. They are rarely found within cranial nerves. To our knowledge, there are only four reports of ganglion/synovial cysts causing unilateral hypoglossal nerve palsy.5–8 Our aim is to present the fifth report, and to compare our findings with the others.
enhancement and was best seen on T2-weighted MRI, where it was hyperintense and well demarcated. A stereotactic suboccipital craniotomy was performed. A bluish lesion was visualised between the cerebellum and medulla (compressing the medulla), which, when opened, extruded a clear jelly-like substance (Fig. 1b). Once debulked, the cyst was carefully dissected from the hypoglossal nerve, vertebral artery and left C1 nerve root to achieve complete macroscopic excision (Fig. 2b). The patient had an uneventful postoperative recovery. He was discharged on postoperative day 5 with a subjective feeling of increased tongue movement. At the week 6 follow-up, the patient still had left-sided hemiatrophy and tongue deviation, but his slurred speech had completely resolved. His postoperative MRI at 6 weeks did not show any residual cyst (Fig. 2a).
2. Case report 3. Discussion A 70-year-old male presented with a history of progressive left hypoglossal nerve palsy and mildly slurred speech. He had a previous history of left Bell’s palsy in 1989, which resolved completely. He developed mild left-tongue deviation in 2002, but brain MRI at that time did not show any lesions. He presented because of progressive worsening of his symptoms; he had developed mildly slurred speech and also left-tongue haemiatrophy. On examination, there was left-sided tongue deviation and moderate atrophy. The rest of the neurological examination was unremarkable, including the lower cranial nerves. MRI of the patient’s brain (Fig. 1a) showed a 16 mm by 14 mm by 18 mm mass adjacent to the left side of the medulla that had compressed and displaced the medulla and extended into the left hypoglossal canal. It was isointense on T1-weighted MRI with no ⇑ Corresponding author. Postal address: 7/1 Collins Street, Hobart, Tasmania 7000, Australia. Tel.: +61 3 6222 8308. E-mail address: [email protected] (S. Gambhir).
The aim of this report was to present a patient with a rare, isolated hypoglossal nerve palsy due to an intraneuronal ganglion cyst. To our knowledge, this is the 5th patient to be reported at the time of submission, and the 2nd from Royal Hobart Hospital, Tasmania, Australia. Hypoglossal nerve palsy usually occurs in association with involvement of lower cranial nerves (CN IX, X and XI). Isolated hypoglossal nerve paralysis is rare but has been reported to be caused by hypoglossal nerve schwannomas, dural arteriovenous fistulas, enlarged emissary veins of the hypoglossal canal, aneurysms of the stump of a persistent hypoglossal artery, occipital condyle fractures, arachnoid cysts, metastatic lesions to the skull base, and internal carotid and vertebral artery dissections. It has also followed neck surgery and has occurred with no apparent cause.7 A cranial nerve ganglion cyst, however, is uncommon. It is most commonly located in the peripheral nerves, which travel near joints
Case Reports / Journal of Clinical Neuroscience 18 (2011) 1114–1115
1115
Fig. 1. (a) Axial T2-weighted MRI showing a left intraneural ganglion cyst causing compression of the medulla, and (b) an intraoperative photograph of a bluish intraneural ganglion cyst. (This figure is available in colour at www.sciencedirect.com).
Fig. 2. (a) Axial T2-weighted postoperative MRI showing no recurrence at the 6-week follow-up, and (b) an intraoperative photograph showing complete macroscopic excision of the intraneural ganglion cyst. (The figure is available in colour at www.sciencedirect.com).
and tendon sheaths.2–4 Cysts can become symptomatic if they compress or invade adjacent nerves. To our knowledge, only four similar cases have been described previously.5–8 The first report, in 2003 by Mujic et al.,5 described an atlantooccipital synovial cyst causing ipsilateral hypoglossal nerve palsy. They called it a synovial cyst due to the presence of connective tissue with myxoid change and the absence of any neural tissue. Following this, Baldauf et al.6 reported a similar case as an intraneural ganglion cyst due to the presence of neural tissue affecting the left hypoglossal nerve. The other two case reports are by Elhammady et al.7 and Nonaka et al.,8 who described an atlantooccipital juxtafacet cyst and an intraneural ganglion cyst, respectively. These reports have been summarised in Supplementary Table 1 and compared to the current patient. In the present patient, the intraneural cyst was a well-demarcated lesion compressing the hypoglossal nerve upon opening the dura. The lesion was abutting the ipsilateral vertebral artery and C1 nerve root but was not causing significant compression. Histopathological findings were consistent with benign connective tissue with advanced myxoid change. Immunohistochemical stains for neurofilament protein and S-100 in the cyst wall were positive for the presence of neural filaments. doi:10.1016/j.jocn.2010.12.030
Appendix A. Supplementary material Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2010.12.030. References 1. Harbaugh KS, Tiel RL, Kline DG. Ganglion cyst involvement of peripheral nerves. J Neurosurg 1997;87:403–8. 2. Spinner RJ, Amrami KK, Kliot M, et al. Suprascapular intraneural ganglia and glenohumeral joint connections. J Neurosurg 2006;104:551–7. 3. Spinner RJ, Atkinson JLD, Tiel RL. Peroneal intraneural ganglia: the importance of the articular branch. A unifying theory. J Neurosurg 2003;99:330–43. 4. Spinner RJ, Carmichael SW, Atkinson JLD. Intraneural ganglion cyst. J Neurosurg 2006;104:990–2. 5. Mujic A, Hunn A, Liddell J, et al. Isolated unilateral hypoglossal nerve paralysis caused by an atlanto-occipital joint synovial cyst. J Clin Neurosci 2003;10:492–5. 6. Baldauf J, Junghans D, Schroeder HW. Endoscope-assisted microsurgical resection of an intraneural ganglion cyst of the hypoglossal nerve. J Neurosurg 2005;103:920–2. 7. Elhammady MS, Farhat H, Aziz-Sultan MA, et al. Isolated unilateral hypoglossal nerve palsy secondary to an atlantooccipital joint juxtafacet synovial cyst. J Neurosurg Spine 2009;10:234–9. 8. Nonaka Y, Grossi PM, Filomena CA, et al. Unilateral hypoglossal nerve palsy caused by an intraneural Ganglion cyst. J Neurosurg 2010;113:380–3.
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Reports
An intraneural ganglion cyst causing unilateral hypoglossal nerve palsy Shanu Gambhir ⇑, Asim Mujic, Andrew Hunn Department of Neurosurgery, Royal Hobart Hospital, 48 Liverpool Street, Hobart, Tasmania 7000, Australia
a r t i c l e
i n f o
Article history: Received 3 December 2010 Accepted 21 December 2010
Keywords: Hypoglossal nerve Intraneural ganglion Palsy Synovial cyst
a b s t r a c t Intracranial ganglion cysts are rare. We report a patient with a rare unilateral hypoglossal nerve palsy caused by an intraneural ganglion cyst. To our knowledge, there are only four reports of ganglion/synovial cysts causing unilateral hypoglossal nerve palsy. Our aim is to present the fifth report, and to compare our findings with the others. Crown Copyright Ó 2011 Published by Elsevier Ltd. All rights reserved.
1. Introduction We report a patient with a rare unilateral hypoglossal nerve palsy caused by an intraneural ganglion cyst. Most intraneural ganglion cysts are found in peripheral nerves near joints or tendon sheaths.1 The most common location of intraneural ganglion cysts is in the peroneal nerve behind the fibular head.2–4 These cysts can become symptomatic due to compression and invasion of adjacent nerves. They are rarely found within cranial nerves. To our knowledge, there are only four reports of ganglion/synovial cysts causing unilateral hypoglossal nerve palsy.5–8 Our aim is to present the fifth report, and to compare our findings with the others.
enhancement and was best seen on T2-weighted MRI, where it was hyperintense and well demarcated. A stereotactic suboccipital craniotomy was performed. A bluish lesion was visualised between the cerebellum and medulla (compressing the medulla), which, when opened, extruded a clear jelly-like substance (Fig. 1b). Once debulked, the cyst was carefully dissected from the hypoglossal nerve, vertebral artery and left C1 nerve root to achieve complete macroscopic excision (Fig. 2b). The patient had an uneventful postoperative recovery. He was discharged on postoperative day 5 with a subjective feeling of increased tongue movement. At the week 6 follow-up, the patient still had left-sided hemiatrophy and tongue deviation, but his slurred speech had completely resolved. His postoperative MRI at 6 weeks did not show any residual cyst (Fig. 2a).
2. Case report 3. Discussion A 70-year-old male presented with a history of progressive left hypoglossal nerve palsy and mildly slurred speech. He had a previous history of left Bell’s palsy in 1989, which resolved completely. He developed mild left-tongue deviation in 2002, but brain MRI at that time did not show any lesions. He presented because of progressive worsening of his symptoms; he had developed mildly slurred speech and also left-tongue haemiatrophy. On examination, there was left-sided tongue deviation and moderate atrophy. The rest of the neurological examination was unremarkable, including the lower cranial nerves. MRI of the patient’s brain (Fig. 1a) showed a 16 mm by 14 mm by 18 mm mass adjacent to the left side of the medulla that had compressed and displaced the medulla and extended into the left hypoglossal canal. It was isointense on T1-weighted MRI with no ⇑ Corresponding author. Postal address: 7/1 Collins Street, Hobart, Tasmania 7000, Australia. Tel.: +61 3 6222 8308. E-mail address: [email protected] (S. Gambhir).
The aim of this report was to present a patient with a rare, isolated hypoglossal nerve palsy due to an intraneuronal ganglion cyst. To our knowledge, this is the 5th patient to be reported at the time of submission, and the 2nd from Royal Hobart Hospital, Tasmania, Australia. Hypoglossal nerve palsy usually occurs in association with involvement of lower cranial nerves (CN IX, X and XI). Isolated hypoglossal nerve paralysis is rare but has been reported to be caused by hypoglossal nerve schwannomas, dural arteriovenous fistulas, enlarged emissary veins of the hypoglossal canal, aneurysms of the stump of a persistent hypoglossal artery, occipital condyle fractures, arachnoid cysts, metastatic lesions to the skull base, and internal carotid and vertebral artery dissections. It has also followed neck surgery and has occurred with no apparent cause.7 A cranial nerve ganglion cyst, however, is uncommon. It is most commonly located in the peripheral nerves, which travel near joints
Case Reports / Journal of Clinical Neuroscience 18 (2011) 1114–1115
1115
Fig. 1. (a) Axial T2-weighted MRI showing a left intraneural ganglion cyst causing compression of the medulla, and (b) an intraoperative photograph of a bluish intraneural ganglion cyst. (This figure is available in colour at www.sciencedirect.com).
Fig. 2. (a) Axial T2-weighted postoperative MRI showing no recurrence at the 6-week follow-up, and (b) an intraoperative photograph showing complete macroscopic excision of the intraneural ganglion cyst. (The figure is available in colour at www.sciencedirect.com).
and tendon sheaths.2–4 Cysts can become symptomatic if they compress or invade adjacent nerves. To our knowledge, only four similar cases have been described previously.5–8 The first report, in 2003 by Mujic et al.,5 described an atlantooccipital synovial cyst causing ipsilateral hypoglossal nerve palsy. They called it a synovial cyst due to the presence of connective tissue with myxoid change and the absence of any neural tissue. Following this, Baldauf et al.6 reported a similar case as an intraneural ganglion cyst due to the presence of neural tissue affecting the left hypoglossal nerve. The other two case reports are by Elhammady et al.7 and Nonaka et al.,8 who described an atlantooccipital juxtafacet cyst and an intraneural ganglion cyst, respectively. These reports have been summarised in Supplementary Table 1 and compared to the current patient. In the present patient, the intraneural cyst was a well-demarcated lesion compressing the hypoglossal nerve upon opening the dura. The lesion was abutting the ipsilateral vertebral artery and C1 nerve root but was not causing significant compression. Histopathological findings were consistent with benign connective tissue with advanced myxoid change. Immunohistochemical stains for neurofilament protein and S-100 in the cyst wall were positive for the presence of neural filaments. doi:10.1016/j.jocn.2010.12.030
Appendix A. Supplementary material Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2010.12.030. References 1. Harbaugh KS, Tiel RL, Kline DG. Ganglion cyst involvement of peripheral nerves. J Neurosurg 1997;87:403–8. 2. Spinner RJ, Amrami KK, Kliot M, et al. Suprascapular intraneural ganglia and glenohumeral joint connections. J Neurosurg 2006;104:551–7. 3. Spinner RJ, Atkinson JLD, Tiel RL. Peroneal intraneural ganglia: the importance of the articular branch. A unifying theory. J Neurosurg 2003;99:330–43. 4. Spinner RJ, Carmichael SW, Atkinson JLD. Intraneural ganglion cyst. J Neurosurg 2006;104:990–2. 5. Mujic A, Hunn A, Liddell J, et al. Isolated unilateral hypoglossal nerve paralysis caused by an atlanto-occipital joint synovial cyst. J Clin Neurosci 2003;10:492–5. 6. Baldauf J, Junghans D, Schroeder HW. Endoscope-assisted microsurgical resection of an intraneural ganglion cyst of the hypoglossal nerve. J Neurosurg 2005;103:920–2. 7. Elhammady MS, Farhat H, Aziz-Sultan MA, et al. Isolated unilateral hypoglossal nerve palsy secondary to an atlantooccipital joint juxtafacet synovial cyst. J Neurosurg Spine 2009;10:234–9. 8. Nonaka Y, Grossi PM, Filomena CA, et al. Unilateral hypoglossal nerve palsy caused by an intraneural Ganglion cyst. J Neurosurg 2010;113:380–3.