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顔面筋の麻痺

著者: 黒田岳志1) 昭和大学 医学部内科学講座 脳神経内科学部門

著者: 河村満2) 奥沢病院

監修: 永山正雄 国際医療福祉大学大学院医学研究科 脳神経内科学

著者校正/監修レビュー済:2021/10/13
参考ガイドライン:
  1. 日本顔面神経研究会:顔面神経麻痺診療の手引-Bell麻痺とHunt症候群- 2011年版
  1. 日本神経治療学会:標準的神経治療:Bell麻痺 2019
患者向け説明資料

概要・推奨   

  1. 末梢性顔面神経麻痺で造影頭部MRIを施行することは腫瘍性疾患やその他の頭蓋内病変に伴う麻痺との鑑別に有用である(推奨度1)。
  1. Bell麻痺やRamsay Hunt症候群の治療において、発症早期の副腎皮質ホルモン療法が奨励される(推奨度1)。
  1. Ramsay Hunt症候群に対しては麻痺の重症度にかかわらず、副腎皮質ホルモン療法と抗ウイルス薬の併用が奨励される(推奨度1)
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
黒田岳志 : 特に申告事項無し[2021年]
河村満 : 特に申告事項無し[2021年]
監修:永山正雄 : 未申告[2021年]

改訂のポイント:
  1. 定期レビューを行い、必要箇所を加筆修正し、鑑別疾患にIgG4関連疾患を追加した。

病態・疫学・診察

疫学情報・病態・注意事項  
  1. 顔面筋の麻痺は大きく「顔面麻痺」と「顔面神経麻痺」に分けられる。「顔面麻痺」は筋および神経筋接合部疾患に伴う「筋性顔面麻痺」と核上性の障害による「中枢性顔面麻痺」に分けられ、「顔面神経麻痺」は核性の障害によるものを「中枢性顔面神経麻痺」、核下性の障害によるものを「末梢性顔面神経麻痺」と呼ぶ。
 
顔面筋の麻痺(分類)

  1. 顔面筋の麻痺は大きく「顔面麻痺」と「顔面神経麻痺」に分けられる。
  1. 「顔面麻痺」は筋および神経筋接合部疾患に伴う「筋性顔面麻痺」と核上性の障害による「中枢性顔面麻痺」に分けられる。
  1. 「顔面神経麻痺」は核性の障害によるものを「中枢性顔面神経麻痺」、核下性の障害によるものを「末梢性顔面神経麻痺」と呼ぶ。

出典

img1:  著者提供
 
 
 
顔面筋の麻痺(分類)

出典

 
  1. 顔面神経麻痺の原因で最も多いのはBell麻痺である。発病率は20~30/10万人[1]で、性差はなく、40歳代にピークを持つ[2]。顔面神経麻痺の約53%(末梢性顔面神経麻痺の60~70%)を占め、多くは一側性で急性発症である[3]。次いでRamsay Hunt症候群が14.1%(末梢性顔面神経麻痺の10~15%)を占める[3]
 
顔面神経麻痺患者の原因と頻度

 
  1. Bell麻痺の原因として単純ヘルペスウイルス1型(HSV-1)の膝神経節での再活性化が指摘されており、発症のきっかけとして免疫力の低下や疲労、ストレスなどの背景を有することが多い。神経の炎症と浮腫が病態の主体であり、浮腫は顔面神経管内で神経の絞扼と虚血を生じ、さらに二次的な神経損傷をもたらす。
  1. Ramsay Hunt症候群は帯状疱疹ウイルス(VZV)の膝神経節での再活性化が原因となり、一般的には耳介・外耳道や舌、口腔粘膜に皮疹が出現する。皮疹の出現しない無疱疹性帯状疱疹(Zoster Sine Herpete:ZSH)の場合もあり、Bell麻痺と診断される10~20%がZSHであると考えられている[4]
  1. そのほかの末梢性顔面神経麻痺として、発症日が不明確で進行性の場合や増悪と寛解を繰り返す場合は腫瘤による圧迫や腫瘍浸潤に伴う顔面神経麻痺を疑う。外傷歴がある場合は外傷性顔面神経麻痺を、鼓膜に発赤や耳下腺に腫張・疼痛がある場合は真珠腫性中耳炎や耳下腺炎などの炎症性耳疾患を疑う。
  1. 中枢性顔面麻痺の原因としては急性発症の場合は脳血管障害の頻度が高い。また、若年であれば多発性硬化症などの脱髄疾患の可能性がある。慢性進行性の経過であれば腫瘍を疑う。中枢性顔面麻痺は顔面麻痺以外の症状を伴うことが多い。
 
問診・診察のポイント  
 
  1. 日常診療のなかで遭遇する顔面筋の麻痺で、頻度の多いものはBell麻痺とRamsay Hunt症候群であり、合わせて全体の約70%を占める。重要な点は2点あり、1つはその他の30%を見逃さないこと、もう1つはBell麻痺とRamsay Hunt症候群をしっかり鑑別することである。

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文献 

著者: W A Hauser, W E Karnes, J Annis, L T Kurland
雑誌名: Mayo Clin Proc. 1971 Apr;46(4):258-64.
Abstract/Text
PMID 5573820  Mayo Clin Proc. 1971 Apr;46(4):258-64.
著者: K K Adour, F M Byl, R L Hilsinger, Z M Kahn, M I Sheldon
雑誌名: Laryngoscope. 1978 May;88(5):787-801.
Abstract/Text In a series of 1502 patients seen in our Facial Paralysis Research Clinic 1048 were diagnosed as having Bell's palsy. Review of clinical, epidemiologic, and laboratory data, plus review of the literature, has led to the conclusion that Bell's palsy is an acute benign cranial polyneuritis probably caused by reactivation of the herpes-simplex virus, and the dysfunction of the motor cranial nerves (V, VII, X) may represent inflammation and demyelinization rather than ischemic compression. Spinal fluid analysis suggests that the disease is a phenomenon of the central nervous system with secondary peripheral neural manifestations. With our presently available information, treatment of a viral disease with an anti-inflammatory agent is rational. Prednisone treatment started within the first week of the disease can restore better function to the paralyzed face than is achieved without such therapy, and facial nerve decompression has been unnecessary.

PMID 642672  Laryngoscope. 1978 May;88(5):787-801.
著者: N Kukimoto, M Ikeda, K Yamada, M Tanaka, M Tsurumachi, H Tomita
雑誌名: Acta Otolaryngol Suppl. 1988;446:17-22.
Abstract/Text The degree of participation and regional specificity of virus infection in relation to atraumatic acute peripheral facial palsy was studied, placing particular emphasis on change in the CF titre of varicella zoster virus (VZV), herpes simplex virus (HSV) and adenovirus (adeno). The subjects of the study were 91 patients with Hunt's syndrome and 396 patients with Bell's palsy treated at 17 institutions all over Japan in the period between April 1985 and November 1986. Among the cases of Hunt's syndrome, the positive conversion rate of CF antibody titre of VZV was 81%. In Bell's palsy cases, virus participation was detectable in 8% with VZV, 4% with HSV and 4% with adeno. With regard to the age distribution, Bell's palsy cases with possible virus involvement tended to be observed in younger patients than those without that possibility. As to regional specificity, the incidence of Bell's palsy with possible virus involvement tended to be higher in densely populated areas. With regard to the main cause of acute peripheral facial palsy, virus infection has been implicated, as well as insufficient blood circulation (ischemia). Even in cases of acute peripheral facial palsy, in which herpes zoster oticus is not observed, the participation of varicella zoster virus (VZV) as a cause of paralysis has been pointed out in some cases (zoster sine herpete). Furthermore, it is known that the serum antibody titres of various viruses such as herpes simplex virus (HSV) change significantly in some cases of Bell's palsy (2, 5-13).(ABSTRACT TRUNCATED AT 250 WORDS)

PMID 2844057  Acta Otolaryngol Suppl. 1988;446:17-22.
著者: Andrew L Thompson, Richard I Aviv, Joseph M Chen, Julian M Nedzelski, Heng-Wai Yuen, Allan J Fox, Aditya Bharatha, Eric S Bartlett, Sean P Symons
雑誌名: Laryngoscope. 2009 Dec;119(12):2428-36. doi: 10.1002/lary.20644.
Abstract/Text OBJECTIVES/HYPOTHESIS: This study characterizes the magnetic resonance (MR) appearances of facial nerve schwannoma (FNS). We hypothesize that the extent of FNS demonstrated on MR will be greater compared to prior computed tomography studies, that geniculate involvement will be most common, and that cerebellar pontine angle (CPA) and internal auditory canal (IAC) involvement will more frequently result in sensorineural hearing loss (SNHL).
STUDY DESIGN: Retrospective study.
METHODS: Clinical, pathologic, and enhanced MR imaging records of 30 patients with FNS were analyzed. Morphologic characteristics and extent of segmental facial nerve involvement were documented.
RESULTS: Median age at initial imaging was 51 years (range, 28-76 years). Pathologic confirmation was obtained in 14 patients (47%), and the diagnosis reached in the remainder by identification of a mass, thickening, and enhancement along the course of the facial nerve. All 30 lesions involved two or more contiguous segments of the facial nerve, with 28 (93%) involving three or more segments. The median segments involved per lesion was 4, mean of 3.83. Geniculate involvement was most common, in 29 patients (97%). CPA (P = .001) and IAC (P = .02) involvement was significantly related to SNHL. Seventeen patients (57%) presented with facial nerve dysfunction, manifesting in 12 patients as facial nerve weakness or paralysis, and/or in eight with involuntary movements of the facial musculature.
CONCLUSIONS: This study highlights the morphologic heterogeneity and typical multisegment involvement of FNS. Enhanced MR is the imaging modality of choice for FNS. The neuroradiologist must accurately diagnose and characterize this lesion, and thus facilitate optimal preoperative planning and counseling.

PMID 19780031  Laryngoscope. 2009 Dec;119(12):2428-36. doi: 10.1002/la・・・
著者: D Dumitru, N E Walsh, L D Porter
雑誌名: Am J Phys Med Rehabil. 1988 Aug;67(4):137-44.
Abstract/Text Facial nerve paralysis is the most common mononeuropathy and idiopathic facial paralysis (Bell's palsy) the most common seventh nerve disease electromyographers may be asked to evaluate. The electrophysiologic method of choice to assess the facial nerve is side-to-side evoked amplitude comparison with the affected side expressed as a percentage of the nonaffected side. This examination should be performed on days 3, 5, 7, 9, 11 and 13 after onset of paralysis. If the percentage of surviving axons falls below 10% within the first 14 days, an incomplete recovery is suggested. Electromyography may assist in prognosticating a functional return, determining neural conduction across the site of injury and following reinervation in the recovery period. The persistence or early return of an absent R1 component of the blink reflex may qualitatively suggest a satisfactory functional outcome in facial paralysis. Supramaximally exciting the facial nerve at the stylomastoid foramen and comparing the clinical response on the affected and nonaffected side, maximum stimulation test, can also predict eventual seventh nerve return. Observing a minimal twitch, utilizing the nerve excitability test or measuring the facial nerve latency have yielded poor correlations with functional return and are of limited usefulness in the prognostication of acute facial palsies. Trigeminal somatosensory evoked potentials can be employed to evaluate the status of the trigeminal nerve as approximately 50% of patients with Bell's palsy also have lesions involving the fifth nerve. Side-to-side amplitude comparison and electromyography are the two most valuable electrophysiologic methods of assessing facial nerve functioning.

PMID 3041998  Am J Phys Med Rehabil. 1988 Aug;67(4):137-44.
著者: Donald H Gilden
雑誌名: N Engl J Med. 2004 Sep 23;351(13):1323-31. doi: 10.1056/NEJMcp041120.
Abstract/Text
PMID 15385659  N Engl J Med. 2004 Sep 23;351(13):1323-31. doi: 10.1056・・・
著者: N Julian Holland, Graeme M Weiner
雑誌名: BMJ. 2004 Sep 4;329(7465):553-7. doi: 10.1136/bmj.329.7465.553.
Abstract/Text
PMID 15345630  BMJ. 2004 Sep 4;329(7465):553-7. doi: 10.1136/bmj.329.7・・・
著者: Scott Shannon, Susan Meadow, Stephen H Horowitz
雑誌名: J Fam Pract. 2003 Feb;52(2):156, 159.
Abstract/Text
PMID 12585996  J Fam Pract. 2003 Feb;52(2):156, 159.
著者: M J Ramsey, R DerSimonian, M R Holtel, L P Burgess
雑誌名: Laryngoscope. 2000 Mar;110(3 Pt 1):335-41. doi: 10.1097/00005537-200003000-00001.
Abstract/Text OBJECTIVE: A meta-analysis was designed to evaluate facial recovery in patients with complete idiopathic facial nerve paralysis (IFNP) by comparing outcomes of those treated with corticosteroid therapy with outcomes of those treated with placebo or no treatment.
STUDY DESIGN: Meta-analysis of prospective trials evaluating corticosteroid therapy for idiopathic facial nerve paralysis.
METHODS: A protocol was followed outlining methods for trial selection, data extraction, and statistical analysis. A MEDLINE search of the English language literature was performed to identify clinical trials evaluating steroid treatment of IFNP. Three independent observers used an eight-point analysis to determine inclusion criteria. Data analysis was limited to individuals with clinically complete IFNP. The endpoints measured were clinically complete or incomplete facial motor recovery. Effect magnitude and significance were evaluated by calculating the rate difference and Fisher's Exact Test P value. Pooled analysis was performed with a random effects model.
RESULTS: Forty-seven trials were identified. Of those, 27 were prospective and 20 retrospective. Three prospective trials met the inclusion criteria Tests of heterogeneity indicate the trial with the smallest sample size (RD = -0.19; 95% CI, -0.58-0.20), to be an outlier. It was excluded from the final analysis. Analyses of data from the remaining two studies indicate corticosteroid treatment improves complete facial motor recovery for individuals with complete IFNP. Rate difference demonstrates a 17% (990% CI, 0.01-0.32) improvement in clinically complete recovery for the treatment group based on the random effects model.
CONCLUSIONS: Corticosteroid treatment provides a clinically and statistically significant improvement in recovery of function in complete IFNP.

PMID 10718415  Laryngoscope. 2000 Mar;110(3 Pt 1):335-41. doi: 10.1097・・・
著者: J R Austin, S P Peskind, S G Austin, D H Rice
雑誌名: Laryngoscope. 1993 Dec;103(12):1326-33. doi: 10.1288/00005537-199312000-00002.
Abstract/Text Idiopathic facial nerve paralysis (IFNP) is a common malady. Because its etiology is unclear, there are a variety of treatment options. Studies to date have not clearly established the benefits of treatment with oral steroids (prednisone). The authors performed a randomized double-blind controlled study comparing the use of placebo versus prednisone which shows that prednisone-treated patients benefit from early treatment. Seventy-six patients met inclusion criteria and completed follow-up until recovery; 35 patients received prednisone and 41 received placebo. Their mean age was 36.8 years. Facial nerve function was assessed using the House-Brackmann facial nerve grading scale, as well as a variety of other measures. Patients were evaluated pretreatment, regularly post-treatment until judged recovered (return of facial function to a grade III or better), and at 6 months after recovery. Difference in mean time to resolution for the prednisone (51.4 days) and placebo (69.3 days) groups was not statistically significant. There was a significant difference in grade at recovery, with the placebo group having a higher proportion of grade III results (P < .03). Eight of 10 patients with electroneurography (ENOG) evidence of denervation were in the placebo group and accounted for 6 of the 7 grade III results. However, the difference in proportion of patients with evidence of denervation for the prednisone (5.7%) and placebo (19.5%) groups did not achieve statistical significance. This study shows that patients treated with prednisone have less denervation than placebo-treated patients. They also have a significant improvement in facial grade at recovery compared to placebo-treated patients. Therefore, the authors recommend that all patients at risk for developing denervation receive prednisone treatment.

PMID 8246650  Laryngoscope. 1993 Dec;103(12):1326-33. doi: 10.1288/00・・・
著者: Minoru Ikeda, Yuzuru Abiko, Nobuo Kukimoto, Hideo Omori, Hidehisa Nakazato, Kyoko Ikeda
雑誌名: Laryngoscope. 2005 May;115(5):855-60. doi: 10.1097/01.MLG.0000157694.57872.82.
Abstract/Text OBJECTIVE: To show the significance of various factors when predicting the outcome of facial nerve paralysis.
DESIGN: Retrospective chart review.
SETTING: Nihon University Itabashi Hospital in Tokyo.
SUBJECTS: Four hundred sixty-seven patients with facial paralysis who visited the hospital within 14 days of disease onset.
METHODS: The failure rate of complete recovery was studied for each of these nine factors: sex, age, varicella-zoster virus (VZV) infection as the cause of paralysis, initial severity of paralysis, number of days from onset of paralysis to the beginning of medical treatment, nerve excitability test (NET), stapedial reflex, lacrimal secretion, and severity of facial paralysis 1 month after onset. These factors were analyzed by logistic regression.
RESULTS: Logistic regression clarified that age, VZV infection, NET response, loss of stapedial reflex, and the state of paralysis 1 month after the onset had statistical significance for the prognosis of facial paralysis. The poor recovery rate was greater than 50% in the patients who exhibited abnormal responses on NET or failed to attain recovery to grade III or better during the 1-month period after the onset of paralysis. These findings were therefore considered as high risk factors for the prognosis. The poor recovery rate was between 25% and 50% in patients who were 50 years or older or whose initial grading of paralysis was V or worse. These findings were classified as moderate risk factors. Patients with VZV-caused paralysis and loss of stapedial reflex had poor recovery rates of below 25%, and these were classified as low risk factors.
CONCLUSION: It is possible to predict the prognosis of facial paralysis on the basis of several clinical findings. NET response, severe initial paralysis, age 50 years or older, and, as a second-stage factor, severity of facial paralysis 1 month after the onset were found to be especially important factors for predicting the prognosis of facial paralysis.

PMID 15867653  Laryngoscope. 2005 May;115(5):855-60. doi: 10.1097/01.M・・・
著者: J R Keane
雑誌名: Neurology. 1994 Jul;44(7):1198-202.
Abstract/Text Among inpatients with facial diplegia, one-half (22 patients) had benign, self-limited causes, including Bell's palsy (10), Guillain-Barré syndrome (5), multiple idiopathic cranial neuropathies (3), brainstem encephalitis (2), Miller Fisher syndrome (1), and association with benign intracranial hypertension (1). Nine patients had tumors: four meningeal, three prepontine, and two intrapontine. Syphilis (2 patients), Hansen's disease (1), cryptococcal meningitis with acquired immunodeficiency syndrome (1), and tuberculous meningitis (1) constituted those with an infectious etiology, while miscellaneous causes included one patient each with diabetes, sarcoidosis, head trauma, pontine tegmental hemorrhage, undiagnosed Möbius syndrome in an adult, systemic lupus erythematosus with severe neuropathy, and slowly progressive degeneration--possibly bulbospinal neuronopathy. Bilaterality makes facial neuropathy a more ominous sign with widely varying causes that requires prompt investigation.

PMID 8035915  Neurology. 1994 Jul;44(7):1198-202.
著者: R J Barohn, J T Kissel, J R Warmolts, J R Mendell
雑誌名: Arch Neurol. 1989 Aug;46(8):878-84.
Abstract/Text Over a 10-year period, we followed up 60 patients (35 men and 25 women) with chronic inflammatory demyelinating polyradiculoneuropathy. Diagnosis was based on previously outlined criteria. Patients were treated in a uniform manner and the overwhelming majority, 56 (94.9%) of 59 treated patients, initially responded to immunosuppressive therapy. The time for initial improvement was 1.9 +/- 3.6 months while the time to reach a clinical plateau was 6.6 +/- 5.4 months. The course was monophasic in 32 patients (53.3%) and relapsing in 28 (46.6%). Despite the initial responsiveness, only 24 (40%) of 60 patients are in partial or complete remission, receiving no medication. Two patients died. We were unable to identify specific clinical or laboratory features at the time of diagnosis that predicted outcome. Our data analysis, along with previous reports, suggests that chronic inflammatory demyelinating polyradiculoneuropathy may be more heterogeneous than previously emphasized. In this light, we have proposed diagnostic criteria that allow for the heterogeneity but at the same time provide for a more consistent approach to better establish the natural history of this condition.

PMID 2757528  Arch Neurol. 1989 Aug;46(8):878-84.
著者: E D Shapiro, M A Gerber
雑誌名: Clin Infect Dis. 2000 Aug;31(2):533-42. doi: 10.1086/313982. Epub 2000 Sep 14.
Abstract/Text Lyme disease, which is caused by Borrelia burgdorferi and transmitted in the United States primarily by Ixodes scapularis (the deer tick), is the most common vector borne disease in the United States. Its most frequent manifestation, a characteristic, expanding annular rash (erythema migrans), sometimes accompanied by myalgia, arthralgia, and malaise, occurs in nearly 90% of persons with symptomatic infection. Other manifestations of Lyme disease include seventh cranial nerve palsy, aseptic meningitis, and arthritis. Extensive coverage in the press about the serious effects of Lyme disease has led to widespread anxiety about this illness that is far out of proportion to the actual morbidity that it causes. This problem is exacerbated by the frequent use of serological tests to eliminate the possible diagnosis of Lyme disease in persons with only nonspecific symptoms (such as arthralgia or fatigue) who have a very low probability that Lyme disease is the cause of their symptoms. Consequently, misdiagnosis is frequent and is the most common cause of failure of treatment. The prognosis for most persons with Lyme disease is excellent.

PMID 10987718  Clin Infect Dis. 2000 Aug;31(2):533-42. doi: 10.1086/31・・・
著者: L S Newman, C S Rose, L A Maier
雑誌名: N Engl J Med. 1997 Apr 24;336(17):1224-34. doi: 10.1056/NEJM199704243361706.
Abstract/Text
PMID 9110911  N Engl J Med. 1997 Apr 24;336(17):1224-34. doi: 10.1056・・・
著者: K K Adour, J Wingerd
雑誌名: Neurology. 1974 Dec;24(12):1112-6.
Abstract/Text
PMID 4475380  Neurology. 1974 Dec;24(12):1112-6.
著者: K K Adour, J Wingerd, D N Bell, J J Manning, J P Hurley
雑誌名: N Engl J Med. 1972 Dec 21;287(25):1268-72. doi: 10.1056/NEJM197212212872503.
Abstract/Text
PMID 4344326  N Engl J Med. 1972 Dec 21;287(25):1268-72. doi: 10.1056・・・
著者: Kazuhiro Kawaguchi, Hiroo Inamura, Yasuhiro Abe, Hidehiro Koshu, Emi Takashita, Yasushi Muraki, Yoko Matsuzaki, Hidekazu Nishimura, Hitoshi Ishikawa, Akira Fukao, Seiji Hongo, Masaru Aoyagi
雑誌名: Laryngoscope. 2007 Jan;117(1):147-56. doi: 10.1097/01.mlg.0000248737.65607.9e.
Abstract/Text OBJECTIVES: To determine whether reactivation of herpes simplex virus (HSV) type 1 or varicella-zoster virus (VZV) is the main cause of Bell's palsy and whether antiviral drugs bring about recovery from Bell's palsy.
STUDY DESIGN: Randomized, multicenter, controlled study.
METHODS: One hundred fifty patients with Bell's palsy were enrolled in this study. The patients were randomly assigned to a prednisolone group or a prednisolone-valacyclovir group, in whom virologic examinations for HSV-1 and VZV were performed by simple randomization scheme in sealed envelopes. The recovery rates among various groups were analyzed using the Kaplan-Meier method and the Cox proportional hazards model.
RESULTS: Reactivation of HSV-1, VZV, and both viruses was detected in 15.3%, 14.7%, and 4.0% of patients, respectively. There was no significant difference in recovery rates between the prednisolone group and the prednisolone-valacyclovir group, although recovery in the patients with HSV-1 reactivation tended to be higher in the prednisolone-valacyclovir group than in the prednisolone group. There was a significant difference in recovery among age groups and between individuals with complete and incomplete paralysis.
CONCLUSIONS: Reactivation of HSV-1 or VZV was observed in 34% of the patients with Bell's palsy. The effect of combination therapy with prednisolone and valacyclovir on recovery was not significantly higher than that with prednisolone alone.

PMID 17202945  Laryngoscope. 2007 Jan;117(1):147-56. doi: 10.1097/01.m・・・
著者: Naohito Hato, Hiroyuki Yamada, Hisashi Kohno, Shuichi Matsumoto, Nobumitsu Honda, Kiyofumi Gyo, Satoshi Fukuda, Yasushi Furuta, Fumio Ohtani, Hiroshi Aizawa, Masaru Aoyagi, Hiroo Inamura, Tsutomu Nakashima, Seiichi Nakata, Shingo Murakami, Jun Kiguchi, Koji Yamano, Taizo Takeda, Masashi Hamada, Kazuhiro Yamakawa
雑誌名: Otol Neurotol. 2007 Apr;28(3):408-13. doi: 10.1097/01.mao.0000265190.29969.12.
Abstract/Text OBJECTIVE: To investigate the effects of valacyclovir and prednisolone in comparison with those of placebo and prednisolone for the treatment of Bell's palsy, excluding zoster sine herpete.
STUDY DESIGN: Prospective, multicenter, randomized placebo-controlled study.
SETTING: Six academic tertiary referral centers.
PATIENTS: Ultimately, 221 patients with Bell's palsy who were treated within 7 days of the onset. Serological and polymerase chain reaction examinations were performed to distinguish Bell's palsy from zoster sine herpete.
INTERVENTION: The patients were treated with either valacyclovir (dosage, 1,000 mg/d for 5 days) plus prednisolone (VP [n = 114]) or placebo plus prednisolone (PP [n = 107]) administered orally.
MAIN OUTCOME MEASURE: Recovery from the palsy was defined as a score higher than 36 using Yanagihara 40-point scoring system without facial contracture or synkinesis. The patients were followed up until complete recovery occurred or for more than 6 months in cases with a poor prognosis.
RESULTS: The overall rate of patient recovery among those treated with VP (96.5%) was significantly better (p < 0.05) than the rate among those treated with PP (89.7%). The rate of patient recovery was also analyzed by classifying the initial severity of facial palsy. In cases of complete or severe palsy, the rates of patients treated with VP and PP who recovered were 95.7% (n = 92) and 86.6% (n = 82), respectively; the recovery rate for treatment with VP was significantly better than that with PP (p < 0.05).
CONCLUSION: The valacyclovir and prednisolone therapy was more effective in treating Bell's palsy, excluding zoster sine herpete, than the conventional prednisolone therapy. To our knowledge, this is the first controlled study of an antiviral agent in the treatment of a sufficient number of Bell's palsy cases based on an etiologic background.

PMID 17414047  Otol Neurotol. 2007 Apr;28(3):408-13. doi: 10.1097/01.m・・・
著者: M A Jalaludin
雑誌名: Methods Find Exp Clin Pharmacol. 1995 Oct;17(8):539-44.
Abstract/Text Sixty patients with Bell's palsy were included in an open randomized trial. Patients were assigned into three treatment groups: steroid (group 1), methylcobalamin (group 2) and methylcobalamin + steroid (group 3). Comparison between the three groups was based on the number of days needed to attain full recovery, facial nerve scores, and improvement of concomitant symptoms. The time required for complete recovery of facial nerve function was significantly shorter ( p < 0.001) in the methylcobalamin (mean of 1.95 +/- 0.51 weeks) and methylcobalamin plus steroid groups (mean of 2.05 +/- 1.23 weeks) than in the steroid group (mean of 9.60 +/- 7.79 weeks). The facial nerve score after 1-3 weeks of treatment was significantly more severe (p < 0.001) in the steroid group compared to the methylcobalamin and methylcobalamin plus steroid groups. The improvement of concomitant symptoms was better in the methylcobalamin treated groups than the group treated with steroid alone.

PMID 8749227  Methods Find Exp Clin Pharmacol. 1995 Oct;17(8):539-44.・・・
著者: B A Yaqub, A Siddique, R Sulimani
雑誌名: Clin Neurol Neurosurg. 1992;94(2):105-11.
Abstract/Text We studied the clinical and neurophysiological effects of methylcobalamin on patients with diabetic neuropathy. In a double-blind study, the active group showed statistical improvement in the somatic and autonomic symptoms with regression of signs of diabetic neuropathy. Motor and sensory nerve conduction studies showed no statistical improvement after 4 months. The drug was easily tolerated by the patients and no side effects were encountered.

PMID 1324807  Clin Neurol Neurosurg. 1992;94(2):105-11.
著者: S Murakami, N Hato, J Horiuchi, N Honda, K Gyo, N Yanagihara
雑誌名: Ann Neurol. 1997 Mar;41(3):353-7. doi: 10.1002/ana.410410310.
Abstract/Text Although the antiviral agent acyclovir is currently used for the treatment of Ramsay Hunt syndrome, its effects on facial nerve and hearing recovery remain controversial. We retrospectively analyzed the effects of acyclovir-prednisone treatment in 80 Ramsay Hunt patients. Of 28 patients for whom treatment was begun within 3 days of the onset of facial paralysis, the recovery from paralysis was complete in 21 (75%). By comparison, of 23 patients for whom treatment was begun more than 7 days after onset, recovery from facial paralysis was complete in only 7 (30%). A significant difference in facial nerve recovery was found between these groups. Early administration of acyclovir-prednisone was proved to reduce nerve degeneration by nerve excitability testing. Hearing recovery also tended to be better in patients with early treatment. There was no significant difference in facial nerve outcome between intravenous and oral acyclovir treatment.

PMID 9066356  Ann Neurol. 1997 Mar;41(3):353-7. doi: 10.1002/ana.4104・・・
著者: M Kinishi, M Amatsu, M Mohri, M Saito, T Hasegawa, S Hasegawa
雑誌名: Auris Nasus Larynx. 2001 Aug;28(3):223-6.
Abstract/Text OBJECTIVE: Although the antiviral agent, acyclovir, is currently employed for the treatment in Ramsay Hunt syndrome, the benefit of acyclovir on facial nerve is still unknown and remains controversial. This study was designed to evaluate the effect of acyclovir in facial nerve recovery in Ramsay Hunt syndrome.
METHODS: To evaluate drug effect on facial nerve function, evaluation of the facial voluntary movement and nerve excitability testing were performed. We have used an infusion therapy of acyclovir in combination with a high dose of steroid (AS), which was started within 7 days of onset of facial nerve palsy in 91 patients with Ramsay Hunt syndrome. The results were compared with those of 47 patients whose therapy was steroid alone started within 7 days of onset.
RESULTS: Out of 91 patients treated with AS, nerve exitability was good in 68 (75%), while it was poor in 17 and absent in six. Of 47 patients treated with steroid alone, nerve exitability was good in 25 (53%), while it was poor in 11 and absent in 11. There was statistically significant difference between AS and steroid therapy in the posttreatment degree of nerve function. Complete recovery to grade I in facial voluntary movement was attained in 82 of 91 patients (90%) in the AS therapy, while out of 47 patients treated with steroid alone complete recovery to grade I was attained in only 30 (64%). A statistically significant difference in the recovery rate of facial nerve function was induced between AS and steroid therapy.
CONCLUSION: The AS therapy was proved to keep good degree of nerve function indicated with nerve excitability testing and improve recovery rate of facial nerve in Ramsay Hunt syndrome. Based on this study, we now believe that the AS therapy is an advisable treatment modality to improve the recovery rate of facial nerve function in Ramsay Hunt syndrome.

PMID 11489365  Auris Nasus Larynx. 2001 Aug;28(3):223-6.
著者: L M Pereira, K Obara, J M Dias, M O Menacho, E L Lavado, J R Cardoso
雑誌名: Clin Rehabil. 2011 Jul;25(7):649-58. doi: 10.1177/0269215510395634. Epub 2011 Mar 7.
Abstract/Text UNLABELLED: The effectiveness of facial exercises therapy for facial palsy has been debated in systematic reviews but its effects are still not totally explained.
OBJECTIVE: To perform a systematic review with meta-analysis to evaluate the effects of facial exercise therapy for facial palsy.
DATA SOURCES: A search was performed in the following databases: Cochrane Controlled Trials Register Library, Cochrane Disease Group Trials Register, MEDLINE, EMBASE, LILACS, PEDro, Scielo and DARE from 1966 to 2010; the following keywords were used: 'idiopathic facial palsy', 'facial paralysis', 'Bell's palsy', 'physical therapy', 'exercise movement techniques', 'facial exercises', 'mime therapy' 'facial expression', 'massage' and 'randomized controlled trials'.
REVIEW METHODS: The inclusion criteria were studies with facial exercises, associated or not with mirror biofeedback, to treat facial palsy.
RESULTS: One hundred and thirty-two studies were found but only six met the inclusion criteria. All the studies were evaluated by two independent reviewers, following the recommendations of Cochrane Collaboration Handbook for assessment of risk of bias (kappa coefficient = 0.8). Only one study presented sufficient data to perform the meta-analysis, and significant improvements in functionality was found for the experimental group (standardized mean difference (SMD) = 13.90; 95% confidence interval (CI) 4.31, 23.49; P = 0.005).
CONCLUSION: Facial exercise therapy is effective for facial palsy for the outcome functionality.

PMID 21382865  Clin Rehabil. 2011 Jul;25(7):649-58. doi: 10.1177/02692・・・
著者: Hiroshi Aizawa, Fumio Ohtani, Yasushi Furuta, Hirofumi Sawa, Satoshi Fukuda
雑誌名: J Med Virol. 2004 Oct;74(2):355-60. doi: 10.1002/jmv.20181.
Abstract/Text The mechanism by which reactivation of varicella-zoster virus (VZV) causes facial paralysis in Ramsay Hunt syndrome remains unclear. The relationship between VZV load and the onset of facial paralysis was analyzed in 42 patients with Ramsay Hunt syndrome. The patients were divided into three groups according to the times of appearance of zoster and of facial paralysis; group I (zoster preceding, n = 13), group II (simultaneous, n = 22), group III (paralysis preceding, n = 7). A real-time quantitative PCR assay was used to measure VZV DNA copy number in saliva, and paired sera were assayed for anti-VZV IgG and IgM antibodies. In group I, the VZV DNA-positive rate was low and virus load decreased gradually after the initial hospital visit around the time of onset of paralysis. The level of anti-VZV antibodies had in most cases already increased at that time. In group III, viral load tended to increase after the onset of paralysis and peaked around the time of appearance of zoster. The level of anti-VZV antibodies was low at the onset of paralysis but showed a significant increase when paired sera were tested. In group II, virus load and changes in level of anti-VZV antibodies either resembled group I or group III behavior. These results indicate that facial paralysis in Ramsay Hunt syndrome can occur at various times between the early and the regression phase of VZV reactivation, suggesting that there are variable patterns of development of facial nerve dysfunction caused by VZV reactivation and the progression of neuritis.

PMID 15332286  J Med Virol. 2004 Oct;74(2):355-60. doi: 10.1002/jmv.20・・・
著者: C J Sweeney, D H Gilden
雑誌名: J Neurol Neurosurg Psychiatry. 2001 Aug;71(2):149-54.
Abstract/Text The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt's analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome. Compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken. In the only prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy. Further, Bell's palsy is significantly associated with herpes simplex virus (HSV) infection. In the light of the known safety and effectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or Bell's palsy with a 7-10 day course of famciclovir (500 mg, three times daily) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3-5 days). Finally, some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome of recovery from facial palsy.

PMID 11459884  J Neurol Neurosurg Psychiatry. 2001 Aug;71(2):149-54.
著者: M Morgan, D Nathwani
雑誌名: Clin Infect Dis. 1992 Jan;14(1):263-71.
Abstract/Text While it is now universally accepted that Ramsay Hunt syndrome is caused by varicella-zoster virus, Bell's palsy continues to be labeled "idiopathic." We review the literature associating Bell's palsy with various infectious agents as well as with Kawasaki disease, a condition in which an infectious etiology is suspected. Good evidence--mostly serological--exists for an etiologic role for the herpes group of viruses, mumps virus, and rubella virus. In addition, recent evidence has focused on Bell's palsy in human immunodeficiency virus infection and Lyme borreliosis. In view of the multiplicity of implicated agents, it is likely that the immunologic response associated with infection triggers a cranial or generalized polyneuropathy culminating in facial nerve compression, degeneration, and paralysis. The mounting interest in Bell's palsy, coupled with the increasing availability of more sensitive and specific tests, is likely to augment the available evidence for an infectious etiology and to clarify the role of other, previously unsuspected infectious agents.

PMID 1315161  Clin Infect Dis. 1992 Jan;14(1):263-71.
著者: Y Satoh, J Kanzaki, S Yoshihara
雑誌名: Auris Nasus Larynx. 2000 Jul;27(3):207-12.
Abstract/Text A comparison between the House-Brackmann facial nerve grading system (the HB-system) and the Yanagihara grading system (the Y-system) was studied with 199 evaluations of 62 cases of postoperative unilateral acoustic neuroma. In the beginning, an original draft of the conversion table was formulated according to the 199 evaluations, in which, 0-6, 8-14, 16-20, 22-28, 30-38, and 40 points in the Y-system were matched with grade VI, V, IV, III, II, and I in the HB-system respectively. The result of the present study for prediction of sequelae showed that it was not necessary to consider the sequelae in a conversion table. And more, the study of an inter-observer variation showed that the lower and upper limits of the scores in the Y-system may shift within about a 2-point range in the draft table. From these aspects, a newly revised conversion table was proposed as a revised conversion table, in which, 0-6, 8-14, 16-22, 24-30, 32-38, and 40 points in the Y-system were matched with grade VI, V, IV, III, II, and I in the HB-system. This revised conversion table is much easier to remember for clinical use, because the score of the lower limit of each grade is simply in multiples of 8, that is, 0, 8, 16, 24, 32 and 40.

PMID 10808106  Auris Nasus Larynx. 2000 Jul;27(3):207-12.
著者: B J Gantz, J T Rubinstein, P Gidley, G G Woodworth
雑誌名: Laryngoscope. 1999 Aug;109(8):1177-88. doi: 10.1097/00005537-199908000-00001.
Abstract/Text OBJECTIVES: Incomplete return of facial motor function and synkinesis continue to be long-term sequelae in some patients with Bell's palsy. The aim of this report is to describe a prospective study in which a well-defined surgical decompression of the facial nerve was performed in a population of patients with Bell's palsy who exhibit the electrophysiologic features associated with poor outcomes. In addition, management issues related to Bell's palsy including herpes simplex virus typel etiology, the natural history, electrodiagnostic testing, and efficacy of surgical strategies are reviewed.
STUDY DESIGN AND METHODS: A multicenter prospective clinical trial was designed utilizing electroneurography (ENOG) and voluntary electromyography (EMG) to identify patients with Bell's palsy who would most likely develop poor return of facial function, as suggested by Fisch and Esslen. Patients who displayed electrodiagnostic features of poor outcome, >90% degeneration on ENOG testing and no voluntary motor unit EMG potentials within 14 days of onset of total paralysis, were offered a surgical decompression of the facial nerve through a middle cranial fossa surgical exposure, including the tympanic segment, geniculate ganglion, labyrinthine segment, and meatal foramen. Control subjects were those who displayed similar electrodiagnostic features and time course.
RESULTS: Subjects who did not reach 90% degeneration on ENOG within 14 days of paralysis all returned to House-Brackmann grade I (n = 48) or II (n = 6) at 7 months after onset of the paralysis. Control subjects self-selecting not to undergo surgical decompression when >90% degeneration on ENOG and no motor unit potentials on EMG were identified had a 58% chance of developing a poor outcome at 7 months after onset of paralysis (House-Brackmann grade III or IV [n = 19]). A group with similar ENOG and EMG findings undergoing middle fossa facial nerve decompression exhibited House-Brackmann grade I (n = 14) or II (n = 17) in 91% of the cases. An exact permutation test confirmed that the surgical group had a significantly higher proportion of patients with a good outcome (House-Brackmann grade I or II) (P = .0002).
CONCLUSION: Electroneurography in combination with voluntary EMG successfully identified patients who will most likely return to normal from those who had a greater chance of long-term sequelae from Bell's palsy. Surgical decompression medial to the geniculate ganglion significantly improves the chances of normal or near-normal return of facial function in the group that has a high probability of a poor result. Surgical decompression must be performed within 2 weeks of onset of total paralysis for it to be effective.

PMID 10443817  Laryngoscope. 1999 Aug;109(8):1177-88. doi: 10.1097/000・・・
著者: Naohito Hato, Junpei Nota, Nobumitsu Hakuba, Kiyofumi Gyo, Naoaki Yanagihara
雑誌名: J Trauma. 2011 Dec;71(6):1789-92; discussion 1792-3. doi: 10.1097/TA.0b013e318236b21f.
Abstract/Text BACKGROUND: In the treatment of facial nerve paralysis after temporal bone trauma, it is important to appropriately determine whether nerve decompression surgery is indicated. The aim of this study was to examine the efficacy of facial nerve decompression surgery according to fracture location and the ideal time for surgery after trauma by analyzing the therapeutic outcome of traumatic facial nerve paralysis.
METHODS: In total, 66 patients with facial nerve paralysis after temporal bone trauma who were treated at our institution between 1979 and 2009 were studied retrospectively. The patients were divided into five subgroups, according to the fracture location and the period of time between trauma and surgery.
RESULTS: The number of patients who achieved complete recovery of House-Brackmann (H-B) grade 1 was 31 of 66 (47.0%). There was no difference in therapeutic outcomes among the subgroups classified by fracture location. The rate of good recovery to H-B grade 1 or 2 in patients undergoing decompression surgery within 2 weeks after trauma reached 92.9%, resulting in a significantly better outcome than that of patients undergoing later decompression surgery (p < 0.01).
CONCLUSIONS: The results of this study demonstrated that the ideal time for decompression surgery for facial nerve paralysis after temporal bone fracture was the first 2 weeks after trauma in patients with severe, immediate-onset paralysis. Our study also showed that surgery should be performed within 2 months at the latest. These findings provide useful information for patients and help to determine the priority of treatment when concomitant disease exists.

PMID 22182890  J Trauma. 2011 Dec;71(6):1789-92; discussion 1792-3. do・・・
著者: K Murakawa, T Minatogawa, M Amatsu
雑誌名: Kobe J Med Sci. 1993 Aug;39(4):147-59.
Abstract/Text Impairment of microcirculation of the facial nerves is considered to play an important role in etiology of facial palsy, and the main emphasis of the conservative treatment is aimed at circulatory improvement. Therefore, at the pain clinic, improved blood flow to the facial nerve by chemical blockage of cervical sympathetic trunks (stellate ganglion block; SGB) is employed in treating patients with facial palsy. The circulatory effects of SGB were clinically determined in 20 patients with acute Bell's palsy, and the effects of chemical blockage of cervical sympathetic trunks on the tissue circulation of facial nerve were also experimentally evaluated in dogs. In the clinical study, the blood flow volume of the common carotid artery increased significantly in 5 minutes after SGB, and has reached its peak of 176.4% in 20 minutes. This significant increase continued up to 75 minutes. In the animal model experiments, the common carotid arterial blood flow volume increased immediately after performing experimental SGB with a peak rise of 143.5% in 15 minutes, and the significant increase continued up to 45 minutes. The blood flow volume in the facial nerve tissue also increased for 5 minutes with a peak rise of 115.2% in 20 minutes after SGB, and this significant increase persisted for up to 60 minutes. A marked increase was observed in the blood flow volume in the facial nerve tissue as well as through the common carotid artery. The circulatory improvement performed by SGB extended as far as the facial nerve itself. From the facts above mentioned, SGB is considered to be an extremely effective treatment for the Bell's palsy.

PMID 7904659  Kobe J Med Sci. 1993 Aug;39(4):147-59.

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