中壢天晟醫(yī)療事業(yè)體系 醫(yī)療副院長

1、中壢天晟醫(yī)療事業(yè)體系 醫(yī)療副院長兼 神經(jīng)外科 主任 莊活力  醫(yī)師   所謂顏面不自主痙攣(Hemifacial spasm)，是一種無法控制地連續(xù)關閉眼臉及顏面肌肉的疾病，剛開始時可能只是眨眼次數(shù)增加引起眼睛酸澀，但當疾病逐漸惡化時，臉部一邊的肌肉產(chǎn)生不自主、間接性之收縮，可能有眨眼、嘴角抖動、及臉步皺縮等表現(xiàn)。上述三種病癥，不但病人本身會因不自主的收縮感到不適，更有甚者，它所造成的怪異表情與姿勢，會造成病人生活作息的困擾及人際關係的障礙。因此，它們雖非危及生命的大病，但於生活上的影響可謂不小。  半邊顏面不自主

2、痙攣的病因，是顏面神經(jīng)在腦幹根部剛出來的部位, 被血管壓迫造成刺激所引發(fā)臉部不自主痙攣。此發(fā)現(xiàn)是在1950-70年代，經(jīng)Prof.Dandy，Prof. Sunderlan等學者所懷疑而經(jīng)Prof. Petter Jannetta所證實。目前全世界已成為神經(jīng)外科普遍的手術  半邊顏面不自主痙攣的鑑別診斷中以排除腫瘤最要緊:顏面神經(jīng)和小腦橋腦角處的腫瘤並不少見，如：聽神經(jīng)纖維瘤、膽脂瘤、血管瘤、腦膜瘤或皮樣囊腫等，這些腫瘤引起的不自主痙攣一般並不嚴重。另外還可同時有神經(jīng)麻痹、耳鳴、眩暈、聽覺喪失、感覺喪失，以及其他顱內(nèi)腫瘤的癥狀。如頭痛、嘔吐和視神經(jīng)乳頭水腫等。必須以x光檢查，電腦斷層

3、、核磁共振造影檢查來幫助診斷。   半邊顏面不自主痙攣的治療        分為內(nèi)科療法和外科療法。內(nèi)科療法包括藥物治療、肉毒桿菌素注射療法等。適用於病程短、疼痛較輕的患者。也可作為外科療法的補充治療。外科療法是治療半邊顏面不自主痙攣最有效最徹底的療方法。但外科療法的缺點是操作技術較難、副作用及並發(fā)癥發(fā)生率略高。一、內(nèi)科藥物療法 一般在初期病患就醫(yī)時，先以藥物治療一、二個月，一方面借藥物來確定診斷，二方面可瞭解患者對藥物的療效如何。藥物治療只能治標不能治本。如果患者對藥物治療

4、效果良好，又不想開刀時，或年紀太大，或有其他疾病不適合手術時，藥物治療為第一選擇。癲通: 2/3的患者有效，但有些患者久服之後，產(chǎn)生對藥物耐受，漸漸無效。其副作用有頭暈，噁心，口腔炎，失眠，站立、行走不穩(wěn)。久服大劑量，亦可引起肝、腎及造血系統(tǒng)損傷、剝脫性皮炎等，甚或死亡。本藥對部分病人無效；有的病人因副作用大，不能用藥；有的病人因懼伯毒性不敢用。有效的病人需長期連續(xù)服用，停藥後痛即再發(fā)。口服藥物效果並不顯著，且又容易產(chǎn)生頭暈、嗜睡、意志混亂（尤其對老人家）、口乾、視力模糊等副作用。這些藥物，通常只對癥狀較輕者有部分療效。二、肉毒桿菌素的注射療法對於眼臉痙攣、斜頸癥及半面痙攣等病癥，肉毒桿菌素已

5、被公認是目前最好的治療方式。它不會產(chǎn)生一般口服藥物的副作用，效果又較口服藥物好得多。肉毒桿菌素是由一種厭氧細菌所產(chǎn)生的神經(jīng)毒素，它能阻斷神經(jīng)肌肉接合處的神經(jīng)傳導，使肌肉無法收縮。適量地使用肉毒桿菌素，能有效減少不自主的肌肉收縮，又不會產(chǎn)生口服藥物的全身性副作用，對臨床癥狀的改善有很顯著的效果。  局部肉毒桿菌素的注射，約對九成的病患有療效。一般在注射後三至四天開始感到癥狀的改善，約一至二週即可感到顯著的進步，改善的程度可達七至九成。至於注射後的藥效，約可維持三到六個月，因人而異。當藥效逐漸消失、癥狀再度顯現(xiàn)時，病患需重複施打，癥狀同樣可獲得快速且有效的控制。肉毒桿菌素的注射

6、雖非一勞永逸，但許多病患在治療一段時間後（施打數(shù)次）癥狀會漸漸消退而不再發(fā)病，此時便無須再繼續(xù)注射此藥劑。  肉毒桿菌素的價格較貴，但只要病患符合一定程度的嚴重度，且經(jīng)六個月以上的口服藥物治療而效果仍不顯著，一般皆能符合健保給付的規(guī)定，病患不必每隔幾個月就須掏腰包自費施打。一般來說，肉毒桿菌素的注射是很安全的，可能會出現(xiàn)的一些小副作用如注射部位的淤青、眼臉下垂、流淚或眼睛乾澀及嘴角歪斜等。但無須擔心，大部分的注射病患不會發(fā)生這些副作用，就算有，通常也會在一、兩週內(nèi)改善並消失。當然，求助於專業(yè)的神經(jīng)科醫(yī)師，應該能將副作用的發(fā)生率降到最低。三、手術療法: 目前治療半邊

7、顏面不自主痙攣常用的開顱手術，正式名稱叫後顱窩顏面神經(jīng)根微血管解壓術，手術的目的就是隔開壓迫顏面神經(jīng)跟部的血管並以鐵弗龍 (Teflon)隔開血管與神經(jīng)，去除神經(jīng)短路。手術過程是在全身麻醉之下,採用側(cè)躺的姿勢,頭轉(zhuǎn)向另一方向,在耳後作一個八到十公分的切口,把耳後的骨頭磨掉一塊約舊的十元硬幣大小,直徑約3公分, 在顯微鏡放大並照明下找到顏面神經(jīng)把壓迫神經(jīng)跟部的血管隔開手術效果幾達百分之九十以上。手術實例顏面神經(jīng)根微血管解壓術併發(fā)癥半邊顏面不自主痙攣的手術是個相當精細的腦部血管顯微手術，醫(yī)師不僅要對顏面神經(jīng)的顯微解剖有充分的理解，對顯微手術更要有豐富的經(jīng)驗。否則將造成後遺癥?？傊脒咁伱姣d攣不是

8、絕癥，經(jīng)由藥物治療肉毒桿菌素注射或是神經(jīng)減壓手術的治療可以為大多數(shù)患者解除困擾。新竹馬偕醫(yī)院神經(jīng)外科莊醫(yī)師對此有很多經(jīng)驗歡迎相關患者前來就診。Hemifacial Spasm and its Treatment by Microvascular DecompressionCharles B. Wilson, M.D.University of California Medical CenterSan Francisco, CAHemifacial spasm is a benign hyperactivity of the muscles enervated by the facial ner

9、ve, including the stapedius muscle in the ear that may cause a clicking sound. The spasm begins as an innocent twitching around one eye, and then progresses relentlessly to affect all facial muscles, including the platysma muscle in the neck. Once started, hemifacial spasm may be more or less active

10、 over the course of time, but I am unaware of any case in which the spasm went away and stayed away. In other words, once it starts, it does not disappear. For some reason women are affected preferentialy, slightly more often on the left side, and predominately in mid-life although I have treated pa

11、tients in their teens and eighties.I know of no effective medical treatment. Botox in expert hands achieves excellent results in the great majority of patients and can be used repeatedly and over long periods of time. Its effects are not enduring, which is its major failing.With rare exceptions, hem

12、ifacial spasm is caused by compression of the facial nerve at its junction with the brainstem by an arterial loop of a normal nearby vessel, usually one of the cerebellar arteries coursing near the origin of the facial nerve. Although the artery often compresses the intimately related eighth nerve,

13、hearing loss is rare despite slight weakening of the facial muscles in long-standing cases. In 5% or fewer cases, the arterial loop is pushed into the origin of the facial nerve by a benign tumor or an aneurysm, and this is the reason for a preoperative MRI scan.The operation itself involves microsu

14、rgical exposure of the facial nerve at its origin from the medulla, identifying the responsible artery, and then gently displacing it away from the point of compression and maintaining the separation with an interposed pad of Teflon. Function of the facial and acoustic nerves is monitored throughout

15、 the procedure by needle electrodes inserted into muscles around the eye and mouth and by means of a microphone in the ear to elicit auditory evoked responses in the brainstem.The operation carries a low morbidity, most patients leaving the hospital on the second or third day postoperatively, Seriou

16、s complications are rare, the most frequent being, loss of hearing on the same side, but this occurs in less than 10% of patients and usually slight. In more than 500 consecutive cases of microvascular decompressions for hemifacial spasm and trigeminal neuralgia, there has been no surgical fatality.

17、 Relief of the spasm following decompression is usually immediate, but in some cases it sputters for a matter of weeks before stopping permanently. Rarely, a delayed facial weakness occurs, but invariably it is temporary and transient. Late recurrence of the spasm is extremely rare in my experience. The occasional early recur