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Original Article
Adolescent-onset idiopathic hemifacial spasm Jianfeng Liang, Zhuangli Guo1, Li Zhang, Yanbing Yu Department of Neurosurgery, China-Japan Friendship Hospital, Beijing, 1Department of Emergency Neurology, Affiliated Hospital of Medical College, Qingdao University, Qingdao, Shandong Province, People’s Republic of China
Abstract Aims: To study the clinical manifestations, illness-related factors and microsurgical management of adolescent-onset idiopathic hemifacial spasm. Materials and Methods: Of the 1221 microvascular decompression procedures performed for idiopathic hemifacial spasm between March 2001 and July 2007, 16 (1.3%) were in adolescent age (≤18). Results: Clinical manifestations in the adolescent-onset were typical, but milder when compared with late-onset. Gender, thickening and adhesion of the arachnoid membrane at the cerebellopontine angle and a small volume of posterior cranial fossa might be important illness-related factors for adolescent-onset idiopathic hemifacial spasm. Arterial compression was the main cause. Typical compression of offending arteries was observed in 14 patients. Compression of petrous bone crest was found in one patient. Immediate effective rate was 100%, and curative rate was 75%. The curative rate, effective rate and delayed resolution rate during the follow-up period (mean: 22.9 months) were 87.5%, 100% and 12.5%, respectively. There was no recurrence. The postoperative complications (2; 12.5%) included: One patient had transient moderate facial palsy and decreased hearing and one patient had transient decreased hearing and tinnitus. Conclusions: This suggests that microvascular decompression is effective in adolescent-onset idiopathic hemifacial spasm.
Address for correspondence: Dr. Yanbing Yu, Department of Neurosurgery, China-Japan Friendship Hospital, 2 Yinghua East Road, Chaoyang, Beijing-100029, People’s Republic of China. E-mail: [email protected] Dr. Li Zhang, Department of Neurosurgery, China-Japan Friendship Hospital, 2 Yinghua East Road, Chaoyang, Beijing-100029, People’s Republic of China. E-mail: [email protected] Received : 23-12-2013 Review completed : 07-02-2014 Accepted : 06-04-2014
Key words: Adolescent-onset, hemifacial spasm, microvascular decompression
Introduction Hemifacial spasm (HFS) is characterized by unilateral involuntary contractions of muscles innervated by the ipsilateral facial nerve and is a progressive disease and generally affects middle-aged and elderly people.[1,2] Adolescent-onset (≤18 years) HFS is extremely rare.[3,4] Earlier studies suggest the thickening and adhesion of the arachnoid membrane, and artery compression as the causes in young-onset idiopathic HFS. This reports Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.132367
Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
the clinical characteristics, illness-related factors and microsurgical management in young-onset idiopathic HFS.
Materials and Methods Of the 1221 microvascular decompression (MVD) procedures performed for idiopathic hemifacial spasm between March 2001 and July 2007, 16 (1.3%) were in adolescent age (≤18). There were 4 males and 12 females and mean age was 24.4 years (range 13-36 years) and the age of onset was 11 to 18 years (mean 16.3 years old). The mean duration of symptoms was 8.2 years (range 1-18 years). Symptoms were on right side in 9 and left side in 7 patients. The symptoms were typical, but milder when compared to later-onset. Ten patients were treated by acupuncture for 1 to 3 months (mean 1.5 months) with no response. The other 6 patients, were treated with botulinum toxin 175
[Downloaded free from http://www.neurologyindia.com on Thursday, May 15, 2014, IP: 202.177.173.189] || Click here to download free Android application for this journal Liang, et al.: Adolescent-onset idiopathic hemifacial spasm
type A injection, but with recurrence symptoms within 3 to 4 months. All the 16 patients had MVD treatment. Surgical procedure and intra-operative observations Exploration of the cerebellopontine angle (CPA) was done through suboccipital retrosigmoidal key hole approach. Intraoperatively, small volume of posterior cranial fossas (basilar invagination or flat skull base) was found in 9 (56.3%) patients. Thickening and adhesion of arachnoid membrane were found in 13 (81.3%) [Figure 1] patients. Typical compression of offending arteries was observed at the root exit zone (REZ) of the facial nerve in 14 patients: Anterior inferior cerebellar artery (AICA) compression (4), posterior inferior cerebellar artery (PICA) compression (6), and multiple arteries (4). Teflon felt was placed between the offending arteries and the brainstem so as to transpose the course of the arteries away from the REZ of cranial nerves. Petrous bone crest compression was found in 1 patient [Figures 2 and 3], and the teflon felt was interposed between the bone crest and REZ. Thickening and
adhesion of the arachnoid membrane without vascular compression was observed in one patient, and the teflon felt was placed around the REZ to protect the facial nerve [Figure 4]. Vein compression was not found in our group.
Results The immediate curative rate was 75% and effective rate was 100%. The range of the postoperative follow-up period was 1 to 44 months (mean 22.9 months). The curative rate, effective rate and delayed resolution rate during the follow-up period were 87.5%, 100% and 12.5%, respectively. There was no recurrence. The postoperative complications (2; 12.5%) included: One patient had transient moderate facial palsy and decreased hearing, and one patient had transient decreased hearing and tinnitus. During the follow-up period these complications had improved.
Figure 2: The petrous bone crests were obvious on a pre-operative CT scan of a left HFS patient Figure 1: Thickening and adhesions of the arachnoid membrane
Figure 3: The petrous bone crest compression was found during the operation of the left HFS patient in Figure 2
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Figure 4: The teflon felt was placed around the REZ to protect the facial nerve in a left HFS patient without vascular compression but with thickening and adhesions of the arachnoid membrane
Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
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Discussion The incidence rate of HFS is approximately 11/100000. It usually affects the left side, and it is more common in women than men[5] HFS is more common in the elderly, and is extremely rare in the adolescent.[3,4] The pathogenesis of HFS is due to facial nerve demyelination induced by artery compression at REZ, which results in the short circuit between the incoming and outgoing nerve fibers. Aging, hypertension and arterial atherosclerosis are considered as the predisposing factors of HFS, and thus the disease is more common in the elderly.[1,2] Adolescent-onset idiopathic HSF is extremely rare and accounted for 1.3% of the total cohort of patients with HFS. The symptoms of adolescent-onset HFS patients in our group were relatively milder compared with the late-onset HFS. Due to severe psychological stress and failed medical treatment, these 16 patients had chosen the surgical treatment. Of the ten patients (two adolescents and eight adults) with HFS reported by Jho et al. [5] in 1987, arterial compression was found in all the ten patients intra-operatively. During the 7.3-year follow-up, the curative rate was 75%. The rate of complications was 50%; four patients with temporary mild paralysis and one patient with mild hearing loss. The authors suggested that MVD is the preferred method of treatment for patients with adolescent-onset HFS. In the 12 patients with adolescent-onset (accounted for 1.2% of all patients with HSF) reported by Levy et al.,[6] intra-operatively the compression was as a result of veins and AICA branches. The curative rate was 67% at 10.4-year follow-up. However, these two studies had not studied the clinical characteristics of adolescent idiopathic HFS, and above this, the two reports did not study the relevant factors regarding the prevalence of adolescent-onset idiopathic HFS. In our study, 75% of patients were females and the side involved was right side in 56.3% of patients. Clinical manifestations were typical, but milder than those with elder-onset. Intra-operative findings included: Small volume posterior cranial fossa due to basilar invagination and/or flat skull base (9; 56.3%), thickening and adhesion of the arachnoid membrane (13; 81.3%).
Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
In the present study, the typical compression of offending arteries was observed at the REZ of facial nerves in four patients. There was no offending vessel in two patients: Petrous bone crest compression was found in one, and thickening and adhesions of the arachnoid membrane in the other. There was no veinous compression at the REZ. Similar to adult-onset HFS patients, MVD was also found effective for adolescent-onset patients. To the best of our knowledge there are no objective criteria to define small and “crowded” posterior fossa, a finding that merits evaluation in future studies. The incidence of facial and vestibulocochlear nerve complications after MVD in our cases may be high than those in adult patients. However, this study included a small number of patients. The data were consistent with the report of Jho et al.[5] The surgeon should pay more attention to adolescent-onset HFS patients during MVD to lower the possibility of postoperative complications involving the cranial nerves, which may result in enormous long-term implications for young patients.
Acknowledgement The authors wish to acknowledge the help received from all the members in the department of Neurosurgery, China-Japan Friendship Hospital.
References 1. 2. 3. 4. 5. 6.
Campos-Benitez M, Kaufmann AM. Neurovascular compression findings in hemifacial spasm. J Neurosurg 2008;109:416-20. Miller LE, Miller VM. Safety and effectiveness of microvascular decompression for treatment of hemifacial spasm: A systematic review. Br J Neurosurg 2012;26:438-44. Chang JW, Chang JH, Park YG, Chung SS. Microvascular decompression of the facial nerve for hemifacial spasm in youth. Childs Nerv Syst 2001;17:309-12. Tan EK, Chan LL. Young onset hemifacial spasm. Acta Neurol Scand 2006;114:59-62. Tan NC, Chan LL, Tan EK. Hemifacial spasm and involuntary facial movements. QJM 2002;95:493-500. Levy EI, Resnick DK, Jannetta PJ, Lovely T, Bissonette DJ. Pediatric hemifacial spasm: The efficacy of microvascular decompression. Pediatr Neurosurg 1997;27:238-41.
How to cite this article: Liang J, Guo Z, Zhang L, Yu Y. Adolescentonset idiopathic hemifacial spasm. Neurol India 2014;62:175-7. Source of Support: The National Natural Science Fundation of China 81173424 and 81271448, Conflict of Interest: None declared.
177
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Original Article
Adolescent-onset idiopathic hemifacial spasm Jianfeng Liang, Zhuangli Guo1, Li Zhang, Yanbing Yu Department of Neurosurgery, China-Japan Friendship Hospital, Beijing, 1Department of Emergency Neurology, Affiliated Hospital of Medical College, Qingdao University, Qingdao, Shandong Province, People’s Republic of China
Abstract Aims: To study the clinical manifestations, illness-related factors and microsurgical management of adolescent-onset idiopathic hemifacial spasm. Materials and Methods: Of the 1221 microvascular decompression procedures performed for idiopathic hemifacial spasm between March 2001 and July 2007, 16 (1.3%) were in adolescent age (≤18). Results: Clinical manifestations in the adolescent-onset were typical, but milder when compared with late-onset. Gender, thickening and adhesion of the arachnoid membrane at the cerebellopontine angle and a small volume of posterior cranial fossa might be important illness-related factors for adolescent-onset idiopathic hemifacial spasm. Arterial compression was the main cause. Typical compression of offending arteries was observed in 14 patients. Compression of petrous bone crest was found in one patient. Immediate effective rate was 100%, and curative rate was 75%. The curative rate, effective rate and delayed resolution rate during the follow-up period (mean: 22.9 months) were 87.5%, 100% and 12.5%, respectively. There was no recurrence. The postoperative complications (2; 12.5%) included: One patient had transient moderate facial palsy and decreased hearing and one patient had transient decreased hearing and tinnitus. Conclusions: This suggests that microvascular decompression is effective in adolescent-onset idiopathic hemifacial spasm.
Address for correspondence: Dr. Yanbing Yu, Department of Neurosurgery, China-Japan Friendship Hospital, 2 Yinghua East Road, Chaoyang, Beijing-100029, People’s Republic of China. E-mail: [email protected] Dr. Li Zhang, Department of Neurosurgery, China-Japan Friendship Hospital, 2 Yinghua East Road, Chaoyang, Beijing-100029, People’s Republic of China. E-mail: [email protected] Received : 23-12-2013 Review completed : 07-02-2014 Accepted : 06-04-2014
Key words: Adolescent-onset, hemifacial spasm, microvascular decompression
Introduction Hemifacial spasm (HFS) is characterized by unilateral involuntary contractions of muscles innervated by the ipsilateral facial nerve and is a progressive disease and generally affects middle-aged and elderly people.[1,2] Adolescent-onset (≤18 years) HFS is extremely rare.[3,4] Earlier studies suggest the thickening and adhesion of the arachnoid membrane, and artery compression as the causes in young-onset idiopathic HFS. This reports Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.132367
Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
the clinical characteristics, illness-related factors and microsurgical management in young-onset idiopathic HFS.
Materials and Methods Of the 1221 microvascular decompression (MVD) procedures performed for idiopathic hemifacial spasm between March 2001 and July 2007, 16 (1.3%) were in adolescent age (≤18). There were 4 males and 12 females and mean age was 24.4 years (range 13-36 years) and the age of onset was 11 to 18 years (mean 16.3 years old). The mean duration of symptoms was 8.2 years (range 1-18 years). Symptoms were on right side in 9 and left side in 7 patients. The symptoms were typical, but milder when compared to later-onset. Ten patients were treated by acupuncture for 1 to 3 months (mean 1.5 months) with no response. The other 6 patients, were treated with botulinum toxin 175
[Downloaded free from http://www.neurologyindia.com on Thursday, May 15, 2014, IP: 202.177.173.189] || Click here to download free Android application for this journal Liang, et al.: Adolescent-onset idiopathic hemifacial spasm
type A injection, but with recurrence symptoms within 3 to 4 months. All the 16 patients had MVD treatment. Surgical procedure and intra-operative observations Exploration of the cerebellopontine angle (CPA) was done through suboccipital retrosigmoidal key hole approach. Intraoperatively, small volume of posterior cranial fossas (basilar invagination or flat skull base) was found in 9 (56.3%) patients. Thickening and adhesion of arachnoid membrane were found in 13 (81.3%) [Figure 1] patients. Typical compression of offending arteries was observed at the root exit zone (REZ) of the facial nerve in 14 patients: Anterior inferior cerebellar artery (AICA) compression (4), posterior inferior cerebellar artery (PICA) compression (6), and multiple arteries (4). Teflon felt was placed between the offending arteries and the brainstem so as to transpose the course of the arteries away from the REZ of cranial nerves. Petrous bone crest compression was found in 1 patient [Figures 2 and 3], and the teflon felt was interposed between the bone crest and REZ. Thickening and
adhesion of the arachnoid membrane without vascular compression was observed in one patient, and the teflon felt was placed around the REZ to protect the facial nerve [Figure 4]. Vein compression was not found in our group.
Results The immediate curative rate was 75% and effective rate was 100%. The range of the postoperative follow-up period was 1 to 44 months (mean 22.9 months). The curative rate, effective rate and delayed resolution rate during the follow-up period were 87.5%, 100% and 12.5%, respectively. There was no recurrence. The postoperative complications (2; 12.5%) included: One patient had transient moderate facial palsy and decreased hearing, and one patient had transient decreased hearing and tinnitus. During the follow-up period these complications had improved.
Figure 2: The petrous bone crests were obvious on a pre-operative CT scan of a left HFS patient Figure 1: Thickening and adhesions of the arachnoid membrane
Figure 3: The petrous bone crest compression was found during the operation of the left HFS patient in Figure 2
176
Figure 4: The teflon felt was placed around the REZ to protect the facial nerve in a left HFS patient without vascular compression but with thickening and adhesions of the arachnoid membrane
Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
[Downloaded free from http://www.neurologyindia.com on Thursday, May 15, 2014, IP: 202.177.173.189] || Click here to download free Android application for this journal Liang, et al.: Adolescent-onset idiopathic hemifacial spasm
Discussion The incidence rate of HFS is approximately 11/100000. It usually affects the left side, and it is more common in women than men[5] HFS is more common in the elderly, and is extremely rare in the adolescent.[3,4] The pathogenesis of HFS is due to facial nerve demyelination induced by artery compression at REZ, which results in the short circuit between the incoming and outgoing nerve fibers. Aging, hypertension and arterial atherosclerosis are considered as the predisposing factors of HFS, and thus the disease is more common in the elderly.[1,2] Adolescent-onset idiopathic HSF is extremely rare and accounted for 1.3% of the total cohort of patients with HFS. The symptoms of adolescent-onset HFS patients in our group were relatively milder compared with the late-onset HFS. Due to severe psychological stress and failed medical treatment, these 16 patients had chosen the surgical treatment. Of the ten patients (two adolescents and eight adults) with HFS reported by Jho et al. [5] in 1987, arterial compression was found in all the ten patients intra-operatively. During the 7.3-year follow-up, the curative rate was 75%. The rate of complications was 50%; four patients with temporary mild paralysis and one patient with mild hearing loss. The authors suggested that MVD is the preferred method of treatment for patients with adolescent-onset HFS. In the 12 patients with adolescent-onset (accounted for 1.2% of all patients with HSF) reported by Levy et al.,[6] intra-operatively the compression was as a result of veins and AICA branches. The curative rate was 67% at 10.4-year follow-up. However, these two studies had not studied the clinical characteristics of adolescent idiopathic HFS, and above this, the two reports did not study the relevant factors regarding the prevalence of adolescent-onset idiopathic HFS. In our study, 75% of patients were females and the side involved was right side in 56.3% of patients. Clinical manifestations were typical, but milder than those with elder-onset. Intra-operative findings included: Small volume posterior cranial fossa due to basilar invagination and/or flat skull base (9; 56.3%), thickening and adhesion of the arachnoid membrane (13; 81.3%).
Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
In the present study, the typical compression of offending arteries was observed at the REZ of facial nerves in four patients. There was no offending vessel in two patients: Petrous bone crest compression was found in one, and thickening and adhesions of the arachnoid membrane in the other. There was no veinous compression at the REZ. Similar to adult-onset HFS patients, MVD was also found effective for adolescent-onset patients. To the best of our knowledge there are no objective criteria to define small and “crowded” posterior fossa, a finding that merits evaluation in future studies. The incidence of facial and vestibulocochlear nerve complications after MVD in our cases may be high than those in adult patients. However, this study included a small number of patients. The data were consistent with the report of Jho et al.[5] The surgeon should pay more attention to adolescent-onset HFS patients during MVD to lower the possibility of postoperative complications involving the cranial nerves, which may result in enormous long-term implications for young patients.
Acknowledgement The authors wish to acknowledge the help received from all the members in the department of Neurosurgery, China-Japan Friendship Hospital.
References 1. 2. 3. 4. 5. 6.
Campos-Benitez M, Kaufmann AM. Neurovascular compression findings in hemifacial spasm. J Neurosurg 2008;109:416-20. Miller LE, Miller VM. Safety and effectiveness of microvascular decompression for treatment of hemifacial spasm: A systematic review. Br J Neurosurg 2012;26:438-44. Chang JW, Chang JH, Park YG, Chung SS. Microvascular decompression of the facial nerve for hemifacial spasm in youth. Childs Nerv Syst 2001;17:309-12. Tan EK, Chan LL. Young onset hemifacial spasm. Acta Neurol Scand 2006;114:59-62. Tan NC, Chan LL, Tan EK. Hemifacial spasm and involuntary facial movements. QJM 2002;95:493-500. Levy EI, Resnick DK, Jannetta PJ, Lovely T, Bissonette DJ. Pediatric hemifacial spasm: The efficacy of microvascular decompression. Pediatr Neurosurg 1997;27:238-41.
How to cite this article: Liang J, Guo Z, Zhang L, Yu Y. Adolescentonset idiopathic hemifacial spasm. Neurol India 2014;62:175-7. Source of Support: The National Natural Science Fundation of China 81173424 and 81271448, Conflict of Interest: None declared.
177
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
