Clinical Neurology and Neurosurgery 119 (2014) 121–124
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Resolution of hemifacial spasm after successful treatment of posterior fossa arteriovenous malformation by gamma knife radiosurgery Yas¸ar Bayri ∗ , Bahattin Tanrıkulu, Ulas¸ Yener, As¸kın S¸eker, Türker Kılıc¸ Marmara University, Faculty of Medicine, Department of Neurosurgery, Selimiye M. Tibbiye C. No. 38, Haydarpasa, Istanbul 34668, Turkey
a r t i c l e
i n f o
Article history: Received 29 July 2013 Received in revised form 28 December 2013 Accepted 19 January 2014 Available online 27 January 2014 Keywords: Arteriovenous malformation Gamma-knife radiosurgery Hemifacial spasm
1. Introduction Hemifacial spasm (HFS) is a condition manifested by involuntary unilateral contraction of the facial muscles. Most of symptoms associated with HFS result from compression of facial nerve at the root exit zone. Such compression is usually caused by nearby vessels. Hemifacial spasm may also develop from other pathologies that involve cerebellopontine angle (CPA), such as tumors, arteriovenous malformations (AVMs), or aneurysms that impact the root exit zone of the facial nerve. Microvascular decompression (MVD) is preferred surgical treatment for most patients with HFS [1]. We report a case of HFS caused by an AVM in CPA in which the patient was treated with gamma knife radiosurgery.
angiography confirmed this and permitted definitive diagnosis of Spetzler-Martin Grade 3 AVM (Fig. 1). The patient refused surgery and was treated with Leksell gamma knife radiosurgery (Leksell Gamma Knife, Model B, Gamma Plan 5. 32) instead, with a prescribed dose of 12 Gy to 50% isodose line (Fig. 2). The volume of nidus was 7.23 cm3 . By 6 months of follow-up, the HFS had regressed such that it was localized to right periocular area. By 15 months, the spasm had completely resolved. Repeat MRI and digital subtraction angiography at 24 months after gamma-knife radiosurgery showed that the size of the AVM had decreased (Fig. 3).
3. Discussion 2. Case report A 55-year-old man presented to our clinic with right HFS of 3 years’ duration. His initial symptom was involuntary facial contractions in periorbital area. The patient had no history of drug therapy or other treatments to address facial contractions. A neurological examination was unremarkable except for right HFS involving right periocular and perioral regions. Magnetic resonance imaging (MRI) was performed, and T2-weighted images showed lesion in right CPA that was suspected to be an AVM. Cerebral digital subtraction
∗ Corresponding author at: Acıbadem Emin bey sokak Atalı apt No. 11/3, Kadıköy, I˙ stanbul, Turkey. Tel.: +90 5325633714; fax: +90 2165451048. E-mail address: [email protected] (Y. Bayri). 0303-8467/$ – see front matter © 2014 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.clineuro.2014.01.014
The incidence of HFS of any origin is 0.84 per 100,000 for females and 0.74 per 100,000 for males, with corresponding prevalence of 14.5 and 7.4 per 100,000 [2]. The peak age range of HFS occurrence is 50–60 years and magnetic resonance imaging is in the diagnostic tool of choice for ruling out primary pathologies that may cause HFS, such as tumor or other brainstem lesion [2]. The method of choice for treating HFS also depends on the specific cause identified. In cases of gross pathology, such as a CPA mass lesion (e.g., tumor or AVM) which accounts for 0.02% of all HFS [3], the treatment options are surgery or Leksell gamma knife radiosurgery, and embolization for AVMs only [4]. In our case, the cause of HFS was an AVM in the CPA and there is only 6 previous reports of such cases in literature [1,5–8] (Table 1). The management for these patients included complete excision of AVM, observation alone or no intervention.
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Fig. 1. (a) T2-weighted MRI revealed a hypointense, signal void lesion in the right cerebellopontine angle, suggesting a possible arteriovenous malformation. (b) Cerebral digital subtraction angiography identified the lesion as an arteriovenous malformation.
Leksell gamma knife radiosurgery is an effective treatment modality for AVMs; however, ours is the first documented case in which this technique has been used to treat HFS caused by an AVM in the CPA. The cause of the HFS in our patient was most likely direct vascular compression related to arterialized veins around the facial
nerve. And HFS began to resolve after 6 months. It would be very optimistic if we talk about the total closure of AVM by 6 months since the optimal time for AVM closure after gamma knife radiosurgery is around 2 years [9]. One might think that HFS revealed because of gamma knife effect on facial nerve. In presented case we use 12 Gy radiation to 50% isodose line for AVM. This was the
Fig. 2. The arteriovenous malformation was treated with Leksell gamma-knife radiosurgery.
Y. Bayri et al. / Clinical Neurology and Neurosurgery 119 (2014) 121–124
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Fig. 3. At 24 months after the gamma-knife procedure, MRI and digital subtraction angiography revealed that the arteriovenous malformation had diminished in size.
Table 1 Summary of case reports about HFS caused by posterior fossa AVM. Case no.
Authors/year of publication
Age (y)/sex
Side
Duration
Intervention
Outcome
1
Janetta et al. (1977) [1]
44 F
L
3y
MVD
2 3 4 5
Dujovni et al. (1979) [5] Pierry et al. (1979) [6] Kim et al. (1991) [7] Nagata et al. (1992) [8]
24 21 64 66
L R R R
6 mo 1y 6y 7y
6
Nagata et al. (1992) [8]
28 F
L
8y
No intervention MVD MVD MVD and clipping of feeding artery AVM resection
Complete resolution just after surgery/4 years later recurrence Persistence of symptoms Complete resolution just after surgery Complete resolution just after surgery Complete resolution just after surgery
M F F M
Complete resolution just after surgery
M: Male, F: Female, L: Left, R: Right, y: Year, mo: Month.
optimal dose in this case to spare peripheral tissues from adverse effects of radiation especially cochlea and brain stem which should not take more than 4 Gy and 8 Gy radiation respectively [10]. There is limited literature about direct gamma knife irradiation of facial nerve for HFS but there are lots of publications about gamma knife treatment of trigeminal neuralgia, which is another cranial nerve hyperactive dysfunction syndrome (HDS). Effective radiation dose for trigeminal neuralgia is reported to be 40 Gy to 50% isodose line (80 Gy maximal dose) [3]. If we assume that facial nerve was affected by radiation in presented case, the dose of radiation taken by facial nerve would be much more less than 12 Gy, which might not be an effective dose to treat a HDS such as HFS. On the other hand there is one case report about direct gamma knife irradiation of VII-VIII nerve bundle for idiopathic HFS in a patient with severe hypoacusia and there are two cases of idiopathic HFS with no auditory problems which were treated with hypofractioned stereotactic radiotherapy, with 15 Gy in 5 fractions of 3 Gy each, and all of three patients had improvement of their symptoms with no auditory complications [11]. We suppose that slowly shrinkage and changes in hemodynamics of the AVM after gamma knife radiosurgery gradually relieved vascular compression around facial nerve and the patient’s symptoms. And also in light of these preliminary findings about gamma
knife irradiation of facial nerve for HFS, low dose radiation also may help improvement of symptoms in our patient. 4. Conclusion Hemifacial spasm caused by an AVM is rare. Our success with gamma knife treatment in this patient with an AVM in the CPA suggests that this method is safe and effective for this patient group. Conflict of interest: The authors had no conflicts of interest. References [1] Jannetta PJ, Abbasy M, Maroon JC, Ramos FM, Albin MS. Etiology and definitive microsurgical treatment of hemifacial spasm. Operative techniques and results in 47 patients. Journal of neurosurgery 1977;47:321–8. [2] Mooij JJA. Cranial Nerve Compression Syndromes. In: Lumenta CB, Di Rocco C, Haase J, Mooij JJA, editors. Neurosurgery. Berlin, Heidelberg: Springer; 2010. p. 475–9. [3] Kobata H, Kondo A, Iwasaki K, Nishioka T. Combined hyperactive dysfunction syndrome of the cranial nerves: trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia: 11-year experience and review. Neurosurgery 1998;43:1351–61 [discussion 1361–1362]. [4] Park H, Hwang SC, Kim BT, Shin WH. Hemifacial spasm caused by a huge tentorial meningioma. J Korean Neurosurg Soc 2009;46:269–72. [5] Dujovny M, Osgood CP, Faille R, Bennett MH, Kerber C. Posterior fossa AVM producing hemifacial spasm: a case report. Angiology 1979;30:425–32.
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[6] Pierry A, Cameron M. Clonic hemifacial spasm from posterior fossa arteriovenous malformation. J Neurol Neurosurg Psychiatry 1979;42: 670–2. [7] Yoshiaki Kim AT, Kimura M, Yoshinaga S, Tomonaga M. Arteriovenous malformation in the cerebellopontine angle presenting as hemifacial spasm. Neurol Med Chir (Tokyo) 1991;31:109–12. [8] Nagata S, Matsushima T, Fujii K, Fukui M, Kuromatsu C. Hemifacial spasm due to tumor, aneurysm, or arteriovenous malformation. Surg Neurol 1992;38: 204–9.
[9] Lunsford LD, Kondziolka D, Flickinger JC, Bissonette DJ, Jungreis CA, Maitz AH, et al. Stereotactic radiosurgery for arteriovenous malformations of the brain. J Neurosurg 1991;75:512–24. [10] Petti PL, Larson DA, Kunwar S. Use of hybrid shots in planning perfexion gamma knife treatments for lesions close to critical structures. J Neurosurg 2008;109(Suppl):34–40. [11] Fraioli MF, Moschettoni L, Fraioli C, Strigari L. Treatment of idiopathic hemifacial spasm with radiosurgery or hypofractionated stereotactic radiotherapy: preliminary results. Minim Invasive Neurosurg: MIN 2010;53:34–6.
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Resolution of hemifacial spasm after successful treatment of posterior fossa arteriovenous malformation by gamma knife radiosurgery Yas¸ar Bayri ∗ , Bahattin Tanrıkulu, Ulas¸ Yener, As¸kın S¸eker, Türker Kılıc¸ Marmara University, Faculty of Medicine, Department of Neurosurgery, Selimiye M. Tibbiye C. No. 38, Haydarpasa, Istanbul 34668, Turkey
a r t i c l e
i n f o
Article history: Received 29 July 2013 Received in revised form 28 December 2013 Accepted 19 January 2014 Available online 27 January 2014 Keywords: Arteriovenous malformation Gamma-knife radiosurgery Hemifacial spasm
1. Introduction Hemifacial spasm (HFS) is a condition manifested by involuntary unilateral contraction of the facial muscles. Most of symptoms associated with HFS result from compression of facial nerve at the root exit zone. Such compression is usually caused by nearby vessels. Hemifacial spasm may also develop from other pathologies that involve cerebellopontine angle (CPA), such as tumors, arteriovenous malformations (AVMs), or aneurysms that impact the root exit zone of the facial nerve. Microvascular decompression (MVD) is preferred surgical treatment for most patients with HFS [1]. We report a case of HFS caused by an AVM in CPA in which the patient was treated with gamma knife radiosurgery.
angiography confirmed this and permitted definitive diagnosis of Spetzler-Martin Grade 3 AVM (Fig. 1). The patient refused surgery and was treated with Leksell gamma knife radiosurgery (Leksell Gamma Knife, Model B, Gamma Plan 5. 32) instead, with a prescribed dose of 12 Gy to 50% isodose line (Fig. 2). The volume of nidus was 7.23 cm3 . By 6 months of follow-up, the HFS had regressed such that it was localized to right periocular area. By 15 months, the spasm had completely resolved. Repeat MRI and digital subtraction angiography at 24 months after gamma-knife radiosurgery showed that the size of the AVM had decreased (Fig. 3).
3. Discussion 2. Case report A 55-year-old man presented to our clinic with right HFS of 3 years’ duration. His initial symptom was involuntary facial contractions in periorbital area. The patient had no history of drug therapy or other treatments to address facial contractions. A neurological examination was unremarkable except for right HFS involving right periocular and perioral regions. Magnetic resonance imaging (MRI) was performed, and T2-weighted images showed lesion in right CPA that was suspected to be an AVM. Cerebral digital subtraction
∗ Corresponding author at: Acıbadem Emin bey sokak Atalı apt No. 11/3, Kadıköy, I˙ stanbul, Turkey. Tel.: +90 5325633714; fax: +90 2165451048. E-mail address: [email protected] (Y. Bayri). 0303-8467/$ – see front matter © 2014 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.clineuro.2014.01.014
The incidence of HFS of any origin is 0.84 per 100,000 for females and 0.74 per 100,000 for males, with corresponding prevalence of 14.5 and 7.4 per 100,000 [2]. The peak age range of HFS occurrence is 50–60 years and magnetic resonance imaging is in the diagnostic tool of choice for ruling out primary pathologies that may cause HFS, such as tumor or other brainstem lesion [2]. The method of choice for treating HFS also depends on the specific cause identified. In cases of gross pathology, such as a CPA mass lesion (e.g., tumor or AVM) which accounts for 0.02% of all HFS [3], the treatment options are surgery or Leksell gamma knife radiosurgery, and embolization for AVMs only [4]. In our case, the cause of HFS was an AVM in the CPA and there is only 6 previous reports of such cases in literature [1,5–8] (Table 1). The management for these patients included complete excision of AVM, observation alone or no intervention.
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Y. Bayri et al. / Clinical Neurology and Neurosurgery 119 (2014) 121–124
Fig. 1. (a) T2-weighted MRI revealed a hypointense, signal void lesion in the right cerebellopontine angle, suggesting a possible arteriovenous malformation. (b) Cerebral digital subtraction angiography identified the lesion as an arteriovenous malformation.
Leksell gamma knife radiosurgery is an effective treatment modality for AVMs; however, ours is the first documented case in which this technique has been used to treat HFS caused by an AVM in the CPA. The cause of the HFS in our patient was most likely direct vascular compression related to arterialized veins around the facial
nerve. And HFS began to resolve after 6 months. It would be very optimistic if we talk about the total closure of AVM by 6 months since the optimal time for AVM closure after gamma knife radiosurgery is around 2 years [9]. One might think that HFS revealed because of gamma knife effect on facial nerve. In presented case we use 12 Gy radiation to 50% isodose line for AVM. This was the
Fig. 2. The arteriovenous malformation was treated with Leksell gamma-knife radiosurgery.
Y. Bayri et al. / Clinical Neurology and Neurosurgery 119 (2014) 121–124
123
Fig. 3. At 24 months after the gamma-knife procedure, MRI and digital subtraction angiography revealed that the arteriovenous malformation had diminished in size.
Table 1 Summary of case reports about HFS caused by posterior fossa AVM. Case no.
Authors/year of publication
Age (y)/sex
Side
Duration
Intervention
Outcome
1
Janetta et al. (1977) [1]
44 F
L
3y
MVD
2 3 4 5
Dujovni et al. (1979) [5] Pierry et al. (1979) [6] Kim et al. (1991) [7] Nagata et al. (1992) [8]
24 21 64 66
L R R R
6 mo 1y 6y 7y
6
Nagata et al. (1992) [8]
28 F
L
8y
No intervention MVD MVD MVD and clipping of feeding artery AVM resection
Complete resolution just after surgery/4 years later recurrence Persistence of symptoms Complete resolution just after surgery Complete resolution just after surgery Complete resolution just after surgery
M F F M
Complete resolution just after surgery
M: Male, F: Female, L: Left, R: Right, y: Year, mo: Month.
optimal dose in this case to spare peripheral tissues from adverse effects of radiation especially cochlea and brain stem which should not take more than 4 Gy and 8 Gy radiation respectively [10]. There is limited literature about direct gamma knife irradiation of facial nerve for HFS but there are lots of publications about gamma knife treatment of trigeminal neuralgia, which is another cranial nerve hyperactive dysfunction syndrome (HDS). Effective radiation dose for trigeminal neuralgia is reported to be 40 Gy to 50% isodose line (80 Gy maximal dose) [3]. If we assume that facial nerve was affected by radiation in presented case, the dose of radiation taken by facial nerve would be much more less than 12 Gy, which might not be an effective dose to treat a HDS such as HFS. On the other hand there is one case report about direct gamma knife irradiation of VII-VIII nerve bundle for idiopathic HFS in a patient with severe hypoacusia and there are two cases of idiopathic HFS with no auditory problems which were treated with hypofractioned stereotactic radiotherapy, with 15 Gy in 5 fractions of 3 Gy each, and all of three patients had improvement of their symptoms with no auditory complications [11]. We suppose that slowly shrinkage and changes in hemodynamics of the AVM after gamma knife radiosurgery gradually relieved vascular compression around facial nerve and the patient’s symptoms. And also in light of these preliminary findings about gamma
knife irradiation of facial nerve for HFS, low dose radiation also may help improvement of symptoms in our patient. 4. Conclusion Hemifacial spasm caused by an AVM is rare. Our success with gamma knife treatment in this patient with an AVM in the CPA suggests that this method is safe and effective for this patient group. Conflict of interest: The authors had no conflicts of interest. References [1] Jannetta PJ, Abbasy M, Maroon JC, Ramos FM, Albin MS. Etiology and definitive microsurgical treatment of hemifacial spasm. Operative techniques and results in 47 patients. Journal of neurosurgery 1977;47:321–8. [2] Mooij JJA. Cranial Nerve Compression Syndromes. In: Lumenta CB, Di Rocco C, Haase J, Mooij JJA, editors. Neurosurgery. Berlin, Heidelberg: Springer; 2010. p. 475–9. [3] Kobata H, Kondo A, Iwasaki K, Nishioka T. Combined hyperactive dysfunction syndrome of the cranial nerves: trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia: 11-year experience and review. Neurosurgery 1998;43:1351–61 [discussion 1361–1362]. [4] Park H, Hwang SC, Kim BT, Shin WH. Hemifacial spasm caused by a huge tentorial meningioma. J Korean Neurosurg Soc 2009;46:269–72. [5] Dujovny M, Osgood CP, Faille R, Bennett MH, Kerber C. Posterior fossa AVM producing hemifacial spasm: a case report. Angiology 1979;30:425–32.
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Y. Bayri et al. / Clinical Neurology and Neurosurgery 119 (2014) 121–124
[6] Pierry A, Cameron M. Clonic hemifacial spasm from posterior fossa arteriovenous malformation. J Neurol Neurosurg Psychiatry 1979;42: 670–2. [7] Yoshiaki Kim AT, Kimura M, Yoshinaga S, Tomonaga M. Arteriovenous malformation in the cerebellopontine angle presenting as hemifacial spasm. Neurol Med Chir (Tokyo) 1991;31:109–12. [8] Nagata S, Matsushima T, Fujii K, Fukui M, Kuromatsu C. Hemifacial spasm due to tumor, aneurysm, or arteriovenous malformation. Surg Neurol 1992;38: 204–9.
[9] Lunsford LD, Kondziolka D, Flickinger JC, Bissonette DJ, Jungreis CA, Maitz AH, et al. Stereotactic radiosurgery for arteriovenous malformations of the brain. J Neurosurg 1991;75:512–24. [10] Petti PL, Larson DA, Kunwar S. Use of hybrid shots in planning perfexion gamma knife treatments for lesions close to critical structures. J Neurosurg 2008;109(Suppl):34–40. [11] Fraioli MF, Moschettoni L, Fraioli C, Strigari L. Treatment of idiopathic hemifacial spasm with radiosurgery or hypofractionated stereotactic radiotherapy: preliminary results. Minim Invasive Neurosurg: MIN 2010;53:34–6.
