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ORIGINAL ARTICLE
Year : 2014  |  Volume : 62  |  Issue : 2  |  Page : 175--177

Adolescent-onset idiopathic hemifacial spasm

Jianfeng Liang1, Zhuangli Guo2, Li Zhang1, Yanbing Yu1,  
1 Department of Neurosurgery, China-Japan Friendship Hospital, Beijing, China
2 Department of Emergency Neurology, Affiliated Hospital of Medical College, Qingdao University, Qingdao, Shandong Province, China

Correspondence Address:
Li Zhang
Department of Neurosurgery, China Japan Friendship Hospital, 2 Yinghua East Road, Chaoyang, Beijing-100029
China
Yanbing Yu
Department of Neurosurgery, China Japan Friendship Hospital, 2 Yinghua East Road, Chaoyang, Beijing-100029
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.



How to cite this article:
Liang J, Guo Z, Zhang L, Yu Y. Adolescent-onset idiopathic hemifacial spasm.Neurol India 2014;62:175-177


How to cite this URL:
Liang J, Guo Z, Zhang L, Yu Y. Adolescent-onset idiopathic hemifacial spasm. Neurol India [serial online] 2014 [cited 2017 Nov 23 ];62:175-177
Available from: http://www.neurologyindia.com/text.asp?2014/62/2/175/132367


Full Text

 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 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 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 [Figure 2] and [Figure 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.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

 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.

 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%).

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

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