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貝爾氏麻痺的歷史:

西元7世紀,就有關於面神經麻痺治療的文字記載。18世紀以後,有了病理生理方面的記錄。此後,隨著對神經生理學方面的知識的認識,擴大並充實了本病的研究範圍。證實了肌肉收縮的神經支配及神經肌肉的電生理現象,特別是,發現損害的神經有再生能力。

19世紀初,歐洲湧現出許多優秀的神經病學者,貝爾(1774~1842)就是其中之一。
1829年,英國王立協會連續發表了自1821年以來有關面神經麻痺的報告,貝爾用驢作試驗,明確地把支配顏面的神經分為運動神經(面神經)和感覺神經(三叉神經),並以此為基礎研究面神經麻痺的臨床及有關內容。特別是,面神經症狀學的記錄,病初顳側頭痛,味覺障礙,聽覺過敏,沿面神經乾有壓痛,麻痺後1天達到高峰,患者面部浮腫,講話、咀嚼、閉眼障礙,不能吹口哨,與今日的貝爾氏麻痺症狀極為相似。
1849年,有人用感應電診斷神經、肌肉疾病,並用於本病的診斷治療獲成果,且能鑑別周圍性和中樞性面神經麻痺。
1870年,有科學家進行面神經切斷實驗,發現切斷後失去神經傳導作用,但肌肉的興奮性仍保持。
1914年,Dejerine發表的“神經系統疾病症候學”被譽為神經病學的經典,其中,對面神經麻痺引起的運動障礙和包括味覺、嗅覺和聽覺的感覺障礙,作了詳細的描述,認為:面神經麻痺的病因有外傷、腫瘤、血管病、壓迫、炎症(梅毒、中耳炎)、神經炎(梅毒、糖尿病、多發性神經炎)。
20世紀後期,主要為病因方面的研究。

Bell's palsy (facial palsy) is characterised by facial drooping on the affected half, due to malfunction of the facial nerve (VII cranial nerve), which controls the muscles of the face. Named after Scottish anatomist Charles Bell, who first described it, Bell's palsy is the most common acute mononeuropathy (disease involving only one nerve), and is the most common cause of acute facial nerve paralysis. The paralysis is of the infranuclear/lower motor neuron type. Bell’s palsy affects about 40,000 people in the United States every year. It affects approximately 1 person in 65 during a lifetime. Until recently, its cause was unknown in most cases, but it has now been related to both Lyme disease and Herpes simplex.
Investigation
Clinicians should determine whether all branches of the facial nerve are involved or whether the forehead muscles are spared. Since these receive innervation from both sides of the brain the forehead can still be wrinkled by a patient whose facial palsy is caused by a problem in the brain rather than in the facial nerve itself.
Facial palsy results from inability to control movement in the facial muscles. The facial muscles become weak or paralyzed. There is no specific treatment for Bell's palsy and it typically subsides on its own within 2-3 weeks.

 

Diagnosis 診斷

01    貝爾氏麻痺病因不明,表現為急性周圍性面神經麻痺。近來發現,病人血清中含有單純皰疹病毒抗體。
02    糖尿病、妊娠及遺傳等是明顯的致病因素,本病有家族聚集性,可能與遺傳有關,是一種常染色體顯性遺傳。
03    可伴有前庭症狀,眼震電圖檢查異常,可能是多發神經病變。
04    有人認為,原發於鼓索神經的炎性病變逆行影響面神經節,神經外膜水腫壓迫,導致面神經缺血,引起可逆的傳導阻滯或不可逆的髓鞘或軸索變性。若刺激神經外膜內的膠原纖維形成,造成永久的纖維壓迫或纖維化。
05    發病年齡多在2140歲,高峰在2130歲,無性別差異。
06    發病前多有鼻塞、肌肉痛、咽痛或其他病毒感染症狀。
07    70%病人味覺改變,其中,50%發生在麻痺前27天,半數以上有患側面部及耳後痛,其中20%發生在麻痺出現前,另20%患側口角及上唇有麻木感,並常擴散至整個面部及舌側。
08    半數病人開始為全身麻痺,或在1週內發展成全麻痺;另一半為部分麻痺,並且不發展成全麻痺。 70%病人在710天內開始恢復,4週內完全恢復;15%恢復良好,伴有輕微的變性合併症,這組病人一般在24週內開始恢復,48週內近於完全恢復;另15%恢復不良,一般在48週內開始出現恢復跡象,6個月後仍恢復不好,伴明顯的合併症。
09    聽力、前庭功能檢查及X光檢查,除了中耳及面神經的腫瘤、隱蔽性乳突炎、先天性膽脂瘤等。凡緩慢發生、逐漸發展及覆發性面神經麻痺提示腫瘤。
10    復發性貝爾氏麻痺僅佔10%,找不出其他原因,對一個突發性麻痺,病前有病毒感染症狀,耳後疼及味覺改變,顯微鏡檢查:透過鼓膜後上象限發現,鼓索神經充血,可考慮為貝爾氏麻痺。
11    本病應與Melkersson-Rosenthal綜合徵鑑別。後者為單側復發性面神經麻痺,伴有面部水腫及溝紋舌,水腫以上唇及眼部最明顯,常有家族史,病因不明,可能是由於自主神經系統功能失調、過敏或免疫性疾病,發病機制,考慮為面神經水腫在管內受壓。
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Pathology

It is supposed to be the result of inflammation of the facial nerve which produces pressure on the nerve as it exits the skull within its bony canal. Patients with facial palsy for which an underlying cause can be readily found are not generally considered to have Bell's palsy per se. These underlying problems include tumor meningitis stroke diabetes mellitus head trauma and inflammatory diseases of the cranial nerves (sarcoidosis brucellosis etc.) In these conditions the neurologic findings are rarely restricted to the facial nerve. Babies can be born with facial palsy and they exhibit many of the same symptoms as people with Bell's palsy; this is often due to a traumatic birth which causes irrepairable damage to the facial nerve i.e. acute facial nerve paralysis.

One disease that may be difficult to exclude in the differential diagnosis is involvement of the facial nerve in infections with the herpes zoster virus. The major differences in this condition are the presence of small blisters or vesicles of the external ear and hearing disturbances but these findings may occasionally be lacking.

In recent years two new suspects have been added to the possible causes of Bell's palsy. Lyme disease may produce the typical palsy and may be easily diagnosed by looking for Lyme-specific antibodies in the blood. In endemic areas Lyme disease may be the most common cause of facial palsy. The subsequent observation of an increased incidence of antibodies to the Herpes simplex virus in patients with Bell's palsy has led many specialists to believe that this agent is the most likely underlying cause in areas where Lyme disease is uncommon.

Epidemiology

Bell's Palsy is three times more likely to strike pregnant women than non-pregnant women .It is also considered to be four times more likely to occur in diabetics than the general population and it is more common in the elderly than children.

Treatment

Treatment is a matter of controversy. In patients presenting with incomplete facial palsy treatment may be unnecessary. However patients presenting with complete paralysis marked by an inability to close the eyes and mouth on the involved side are usually treated with anti-inflammatory corticosteroids. The efficacy of this treatment has not been reliably demonstrated. The likely association of Bell's palsy with the herpes virus has led most American neurologists to prescribe a course of anti-viral medication (such as acyclovir) to all patients with unexplained facial palsy. Surgical procedures to decompress the facial nerve have been attempted but have not been proven beneficial. Acupuncture has also been studied with inconclusive results.

People who think they may have Bell's Palsy should consult their doctor as soon as possible. Many times the medications will not be effective if administered too late after the onset.

Although most patients (60–80%) recover completely from Bell's palsy within several weeks some require several months and others may be left with deficits of varying degrees.

Complications

Major complications of the condition are chronic loss of taste called ageusia chronic facial spasm and corneal infections. To prevent the latter the eyes may be protected by covers or taped shut during sleep and for rest periods and tear-like eye drops or eye ointments may be recommended especially for cases with complete paralysis. Where the eye does not close completely the reflex is also affected; great care should be taken to protect the eye from injury.

Another complication can occur in case of incomplete or erroneous regeneration of the damaged facial nerve. The nerve can be thought of as a bundle of smaller individual nerve connections which branch out to their proper destinations. During regrowth nerves are generally able to track the original path to the right destination - but some nerves may sidetrack leading to a condition known as synkinesis. For instance regrowth of nerves controlling muscles attached to the eye may sidetrack and also regrow connections reaching the muscles of the mouth. In this way movement of one also affects the other. For example when the person closes the eye the corner of the mouth will lift or when smiling the eye will close (synkinesis).

In addition around 6% of patients exhibit crocodile tear syndrome on recovery where they will shed tears while eating. This is thought to be due to faulty regeneration of the facial nerve a branch of which controls the lacrimal and salivary glands.

There exists a small loosely connected network of physical therapists throughout the United States who specialize in treating Bell's Palsy and other forms of facial paralysis. They use refined forms of manual therapy for the face facial exercises neuromuscular re-education which includes using proprioceptive facilitation biofeedback infra-red laser sensory electrical stimulation and nerve conduction studies. This program with all elements combined together can see remarkable results in patients who have had their paralysis longer than 6 months with no real hope of a spontaneous recovery. An extensive internet search can find one of these therapists.

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