Bell's palsy

Published by Bupa's health information team, March 2009. This factsheet is for people who have Bell's palsy, or who would like information about it. Bell's palsy is a condition in which one side of the face becomes paralysed. It's usually temporary. Symptoms of Bell's palsy generally come on suddenly and have no obvious cause.

About Bell's palsy

Bell's palsy is a condition where the muscles in your face become weak or paralysed. It happens on one side of your face only and is the most common cause of paralysis of the face. Bell's palsy was named after Sir Charles Bell, a nineteenth-century doctor who first described the condition and linked it to a problem with the facial nerve. The condition is fairly common. Every year around one in 5,000 people develop Bell's palsy. It can affect people of all age groups, including children, but it's most common in people aged 15 to 45. Men and women are equally affected.

Symptoms of Bell's palsy

The symptoms of Bell's palsy usually come on very quickly - often in a few hours or overnight. The main symptom is that one side of your face becomes weak or paralysed. You may also find that your eyebrow sags and you find it difficult to close your eye. For some people, mild earache or a pain behind the ear is the first sign of Bell's palsy. You may also find that: your mouth sags saliva and drinks dribble from your mouth you find it difficult to speak you can't taste at the front of your tongue, or you have an altered sense of taste your eye is dry or watery on the affected side of your face your eyelid droops you have unusually sharp hearing on the affected side of your face These symptoms aren't always due to Bell's palsy, but if you have them, you should visit your GP for advice. Around three in four people with Bell's palsy recover completely. Most people see a major improvement within three to eight weeks of developing the condition, but for some people it can take three to six months before symptoms improve, when damaged nerves have had a chance to re-grow. Around one in six people are left with facial weakness, muscle tightness, facial spasms or twitches.

Complications of Bell's palsy

Sometimes, new nerve fibres that grow back after you first developed Bell's palsy join up to the wrong muscles in your face. This can have long-lasting effects, including: the corner of your mouth turning up in a smile when you blink. This is caused by spontaneous twitches or spasms of the muscles in your face (also known as synkinesis) tears forming in one of your eyes while you're eating

Causes of Bell's palsy

The exact reasons why you may develop Bell's palsy aren't fully understood at present. However, it's thought that a viral infection that affects your facial nerve is the most likely cause. Herpes simplex - the virus responsible for cold sores - has been suggested as responsible for many cases. This virus may lie inactive (dormant) at the root of the facial nerve, causing swelling when it reactivates (wakes up). Ramsay Hunt syndrome is a condition that is similar to Bell's palsy, but here the virus - Varicella zoster (a strain of the herpes virus that causes chicken pox) - has been identified as the cause. Blisters in the ear are often found in Ramsay Hunt syndrome. You may be more likely to develop Bell's palsy if you have diabetes or are pregnant.

Diagnosis of Bell's palsy

Facial paralysis isn't always the result of Bell's palsy - other causes include: pressure on the facial nerve (eg caused by a tumour) infections such as Lyme disease (borreliosis) sarcoidosis facial wounds Your GP will ask you about your symptoms and examine you. He or she may also ask you about your medical history and will want to exclude other possible causes of facial paralysis. Your GP may perform blood tests to check for Lyme disease, a condition spread by ticks, which can cause muscle weakness similar to Bell's palsy. He or she may also send you for an MRI (magnetic resonance imaging) scan to check for any signs of a stroke or a brain tumour. An MRI scan uses magnets and radiowaves to produce images of the inside of your brain. Sometimes your GP will refer you to an ear, nose and throat specialist who will perform the blood tests or send you for an MRI scan. Bell's palsy is idiopathic, which means that its cause is unknown. So, if your doctor finds a specific cause of your facial paralysis, you don't have Bell's palsy.

Treatment of Bell's palsy

For most people, Bell's palsy gets better by itself without any treatment at all. However, you should try to rest as much as possible if you have the condition.

Self-help
Bell's palsy may make it hard to close your eyelid. If this happens to you, you could try using the following to help stop the surface of your eyeball drying out: an eye pad or tape to keep the eye closed before you go to sleep artificial tears (eye drops) to keep the eye moist Ask your pharmacist for advice on eye drops if you think that they will help you. Tinted sunglasses may also be helpful. Remember to keep your mouth and teeth clean, as food can become trapped in your mouth.

Medicines
Your doctor may prescribe medicines to increase your chance of recovery. Steroids (eg prednisolone) may reduce the swelling of the nerve and prevent damage to it. Your chance of recovery improves if you start taking these within three days of your symptoms first starting. The tablets are usually taken for 10 days. Antivirals (eg valaciclovir) can be prescribed to treat the herpes virus that may have caused the swelling. However, there is little evidence that an antiviral medicine helps in Bell's palsy. Always ask your doctor for advice and read the patient information leaflet that comes with your medicine.

Surgery
Surgery or cosmetic procedures may also be used if there are long-term effects of Bell's palsy. Surgery can be done to relieve pressure on the facial nerve, although this is rarely recommended.

Physical/complementary therapies
Physiotherapy, acupuncture, facial exercises and massage may be used, but more research is needed to prove that these treatments work.

Prevention of Bell's palsy

There is no way of preventing Bell's palsy because its cause is unknown, and there aren't any tests to detect it before symptoms begin.

Further information

Bell's Palsy Association www.bellspalsy.org.uk

Bell's palsy Q&As

See our answers to common questions about Bell's palsy, including: Should children with Bell's palsy be given steroids? Is there anything I can do to improve my chances of recovering from Bell's palsy? Is it true that acupuncture can help people with Bell's palsy?

Related topics

Acupuncture

Sources
Salinas RA, Alvarez G, Ferreira J. Corticosteroids for Bell's palsy (idiopathic facial paralysis). Cochrane Database of Systematic Reviews 2004, Issue 4. Art. No: CD001942.pub2. www.cochrane.org Holland NJ, Weiner GM. Recent developments in Bell's palsy. BMJ 2004; 329:553-557. www.bmj.com Update on managing Bell's palsy. Drug Ther Bull 2008; 46:53-54. http://dtb.bmj.com Finsterer J. Management of peripheral facial nerve palsy. Eur Arch Otorhinolaryngol 2008; 265:743-752. www.springerlink.com Bell's palsy factsheet. National Institute of Neurological Disorders and Stroke (NINDS). www.ninds.nih.gov, accessed 7 August 2008 Piercy J. Bell's palsy. BMJ 2005; 330:1374. www.bmj.com Tiemstra JD, Khatkhate N. Bell's palsy: diagnosis and management. Am Fam Physician 2007; 76:997-1002. www.aafp.org/afp This information was published by Bupa's health information team and is based on reputable sources of medical evidence. It has been peer reviewed by Bupa doctors. The content is intended for general information only and does not replace the need for personal advice from a qualified health professional. Publication date: March 2009

Facial Nerve Paralysis

Author: John YS Kim, MD, Assistant Professor, Department of Surgery, Division of Plastic Surgery, Northwestern Medical Faculty Foundation; Consulting Staff, Northwestern Plastic Surgery Coauthor(s): Andreas Niederbichler, MD, Staff Physician, Department of Plastic Surgery, University Hospital of Hannover, Germany; Arturo Armenta, MD, Staff Physician, Department of Surgery, Division of Plastic Surgery, Baylor College of Medicine Contributor Information and Disclosures

Updated: Nov 20, 2008

Introduction
Background
The eye cannot close and constantly weeps. The mouth dribbles, the speech is interfered with and mastication impaired. The delicate shades of continence are lost. Joy, happiness, sorrow, shock, surprise, all the emotions have for their common expression the same blank stare.1

Speech, mastication, and expression of moods and emotions are based on the ability to move facial musculaturebe it voluntary or involuntary. This article informs the reader about extracranial etiology of facial nerve paralysis and its current reconstructive options. The keystone of successful surgical treatment, anatomy, is recapitulated briefly to both review topographic anatomy of the facial nerve and enable the physician to localize the suspected site of injury. Clinical and technical diagnostic possibilities as well as guidelines in certain clinical scenarios are outlined in the decision-making and patient treatment sections. The patient treatment/operative technique section is subdivided further into acute and chronic stage facial nerve paralysis due to the different reconstructive strategies in these situations.

For more information, see eMedicine Plastic Surgery articles Facial Nerve Paralysis, Dynamic Reconstruction and Facial Nerve Paralysis, Static Reconstruction Historical review As early as 1821, Sir Charles Bell discovered that the facial nerve is responsible for facial muscle movement.2 Soon thereafter, the recognition of the anatomic basis of facial nerve injury gave way to reconstructive strategies. For example, the first facial nerve repair was performed in 1879 by Drobnick. 3 Drobnick coapted the facial nerve to the spinal accessory nerve. Alternative reconstructive options such as myoneurotization were explored with limited success by Lexer and Eden as well as Owens in 1911. 4,5 In 1927, Bunnell was the first to attempt intratemporal repair of the facial nerve in its fallopian canal course.1 In 1971, Thompson became one of the pioneers in free muscle transplantation without vascular microanastomosis.6 He discovered that the best results were achieved when the graft had been denervated 2-3 weeks before transplantation. Because these methods did not yield good results in terms of postoperative muscle strength and contraction ability, they seldom are performed presently.7 Investigation into the anatomy and pathophysiology of facial nerves was undertaken concomitantly with the surgical advances in treatment of facial nerve paralysis. In 1973, May and Miehlke successfully conducted the fascicular spatial orientation (motor, secretory, afferent) of the facial nerve.8,9 With the advent of refined microsurgical techniques advocated by Millesi, Berger, and others, the suture repair of peripheral nerves underwent a renaissance in the 1970s. Concurrently, Scaramella pioneered cross-facial nerve grafts as a technique of coapting contralateral intact facial nerves to injured facial nerve.10 Harii et al broached the idea of microneurovascular free muscle transplantation for the reconstruction of the paralyzed face in 1976. This group performed a free gracilis muscle transfer for the reconstruction of a smile with excellent results.11 Microneurovascular free muscle transfer remains one of the basic methods for reanimation of the paralyzed face. Moreover, free tissue transfer obtains predictable results, since an overall survival rate of 90% is reported in the literature. Anatomy The facial nerve (cranial nerve VII) carries motor, secretory, and afferent fibers from the anterior two thirds of the tongue. (See Image 2 for an illustration of facial nerve anatomy.) It originates in the facial nucleus, which is located at the caudal pontine area. Corticobulbar fibers from the precentral gyrus (frontal lobe) project to the facial nucleus, with most crossing to the contralateral side. As a result, both crossed and uncrossed fibers are found in the nucleus. Moreover, the facial nucleus can be divided into two parts: (1) the upper part receiving corticobulbar projections bilaterally and later coursing to the upper parts of the face, including the forehead, and (2) the lower part, the predominantly crossed projections of which supply innervation to lower facial muscles (stylohyoid; posterior belly of digastric, buccinator, and platysma).12 In terms of topography, both the facial and intermedius nerves course from the posterior pontine area ventral passing through the facial canal together with the vestibulocochlear nerve. All 3 nerves are surrounded by pia mater through their subarachnoid course, thus becoming a common sheath at the internal auditory canal.13 Both the inferior anterior cerebellar artery and venous drainage enter the auditory canal together with the facial nerve. Intratemporally, the facial and vestibular cochlear nerves split, entering the fallopian canal of the temporal bone. Topographically, the further course of the facial nerve is subdivided in 3 segments. The labyrinthine segment, measuring approximately 4 mm, extends perpendicular to the temporal bone axis. Initially, the facial nerve runs anterior obliquely, remaining separate from the intermedius nerve and unifying at the next level, the geniculate ganglion. Afferent fibers from the anterior two thirds of the tongue enter the geniculate ganglion with the chorda tympani, as the greater and lesser petrosal nerve emerge from the superior part. The tympanic segment of the fallopian canal extends approximately 1 cm.14 Here, the facial nerve runs horizontally at the medial wall of the cavum tympani. The third or mastoidal segment extends directly

vertical approximately 1.5 cm. The stylomastoid branch of the posterior auricular artery provides vascular supply to the facial nerve during its intrafallopian course. Especially considering susceptibility to nerve injury, the bony canal-facial nerve diameter is an important clinical ratio. Most often, the facial nerve takes up approximately 25-50% of the canal diameter. The facial nerve exits the fallopian canal through the stylomastoid foramen, afterward taking its extratemporal course anteriorly, inferiorly, and laterally. The posterior auricular nerve (innervating both postauricular and occipital muscles) branches posteriorly cranial just below the foramen, as do two smaller ones to the stylohyoideus and posterior belly of the digastric muscle. The facial nerve runs laterally to the styloid process. The facial nerve then enters the parotid gland between the stylohyoid and digastric muscle. The nerve gives off branches lateral to the external jugular vein, constituting the zygomatic-temporal and the cervicofacial trunks. A diverse number of classifications of the extratemporal course of the facial nerve are found in literature. One was proposed in 1956 by Davis et al, who investigated the different course patterns of the infratemporal facial nerve in 350 cervicofacial halves. The temporal trunk innervates the frontalis, orbicularis oculi, corrugator supercilii, and pyramidalis muscles. Zygomaticus major as well as minor, elevator ala nasi, levator labii superioris, caninus, depressor septi, compressor nasi, and dilatator naris muscles are innervated by the zygomatic division. The buccal division gives off fibers to innervate the buccinator and superior part of the orbicularis oris muscle. Mandibular division innervations consist of risorius, quadratus labii inferioris, triangularis, mentalis, and lower parts of the orbicularis oris muscle. The cervical division provides platysma innervation. A "facial danger zone" is known to follow an imaginary line drawn from the lateral canthus to the lateral corner of the mouth and from the zygomatic arch down to the angle of the mandible. The plastic surgeon should keep in mind that the more distal the injury to the facial nerve, the better the chances for spontaneous recovery. Generally, good reconstructive results of facial nerve repair have been yielded by Terzis et al even when a comparatively small number of axons is regenerated.15 Terzis has found a higher nerve-to-muscle fiber ratio than in other skeletal muscles (1:8 compared to 1:50 in other skeletal muscles). For more information on the anatomy of the facial nerve and surrounding nerves, see eMedicine article Facial Nerve Anatomy.

Pathophysiology
Facial nerve injury can be complete or partial. Generally, partial disruption of axonoplasmal flow reveals a greater chance of complete functional recovery. Loss of motor function can be observed immediately after facial nerve injury. Depending on the affected trunk and localization (proximal or distal), various patterns of motor function loss can be seen and used for primary diagnosis of the lesion site. Significant muscle fiber decay has been demonstrated when denervation has been present for more than 3 years.16 Early changes at cellular level (approximately 1 wk after denervation) include chromatin changes and increased mitochondria number, DNA, and satellite cells, thus reflecting the plastic state of denervated muscle. In addition to clinical and Histopathologic Findings, parasympathetic functions such as salivation, lacrimation, and taste sensation also may be impaired.

Clinical
History
Clinical diagnosis is based on 3 steps, identification of the affected site, underlying etiology (trauma, infectious, neoplastic), and finally, clinical staging (eg, with use of the House-Brackmann scale). Careful delineation of the history should include onset of symptoms, an evaluation of the quality of associated symptoms, and prior infections and systemic diseases (eg, herpes simplex virus, varicellazoster virus, neoplasms).

Physical
A thorough head and neck examination is paramount, with occasional use of tests for salivation, tearing, and taste; these are the first steps in determining the site of injury. Physical examination findings reveal affected facial musculature movement. Tests for facial innervation include the following: o Forehead wrinkling (frontalis muscle) o Eye closure (orbicularis oculi muscle) o Wide smile o Whistling o Blowing (eg, buccinator muscle, orbicularis oris muscle, zygomatic muscle) During the patient's initial consultation, evaluate general muscle status (latissimus muscle, rectus abdominis muscle) for eventual reconstruction. Clinically, injury to the infratemporal facial nerve can be subclassified into several degrees. Multiple classifications of facial nerve injury are found in the literature; the most frequently used is the HouseBrackmann scale.17 Table 1. House-Brackmann Classification of Facial Function17 Grade I. Normal II. Mild dysfunction Characteristics Normal facial function in all areas Gross Slight weakness noticeable on close inspection May have slight synkinesis At rest, normal symmetry and tone Motion Forehead - Moderate-to-good function Eye - Complete closure with minimal effort Mouth - Slight asymmetry III. Moderate dysfunction Gross Obvious but not disfiguring difference between sides Noticeable but not severe synkinesis, contracture, or hemifacial spasm At rest, normal symmetry and tone Motion Forehead - Slight-to-moderate movement Eye - Complete closure with effort Mouth - Slightly weak with maximum effort IV. Moderately severe dysfunction Gross Obvious weakness and/or disfiguring asymmetry At rest, normal symmetry and tone Motion Forehead - None Eye - Incomplete closure Mouth - Asymmetric with maximum effort V. Severe dysfunction Gross Only barely perceptible motion At rest, asymmetry Motion Forehead - None Eye - Incomplete closure Mouth - Slight movement VI. Total paralysis No movement

Grade I. Normal II. Mild dysfunction

Characteristics Normal facial function in all areas Gross Slight weakness noticeable on close inspection May have slight synkinesis At rest, normal symmetry and tone Motion Forehead - Moderate-to-good function Eye - Complete closure with minimal effort Mouth - Slight asymmetry III. Moderate dysfunction Gross Obvious but not disfiguring difference between sides Noticeable but not severe synkinesis, contracture, or hemifacial spasm At rest, normal symmetry and tone Motion Forehead - Slight-to-moderate movement Eye - Complete closure with effort Mouth - Slightly weak with maximum effort IV. Moderately severe dysfunction Gross Obvious weakness and/or disfiguring asymmetry At rest, normal symmetry and tone Motion Forehead - None Eye - Incomplete closure Mouth - Asymmetric with maximum effort V. Severe dysfunction Gross Only barely perceptible motion At rest, asymmetry Motion Forehead - None Eye - Incomplete closure Mouth - Slight movement VI. Total paralysis No movement

House and Brackmann ranged injury from stage 1-6 with different chances of spontaneous recovery. These stages correspond with the pathologic findings of neurapraxia, axonotmesis, neurotmesis, and partial and complete transection of the facial nerve. o A clinical House-Brackmann grade 1 refers to neurapraxia, which is the most likely stage for spontaneous recovery. o Secondly, axonotmesis is the term for longer compression of the nerve, clinically a HouseBrackmann level 2-3, with temporary axonoplasmal flow interruption and subsequent Wallerian anterograde degeneration. Degeneration in axonotmesis is most often incomplete with more or less axons surviving, thus clinically often a partial facial weakness results. o Neurotmesis is a state of permanent loss of axons further characterized by (partial) demyelinization leading to moderate-to-severe facial musculature dysfunction. Regenerative impulses may end in facial synkinetic movements, mass movements, or contracture. o Finally, clinical findings in House-Brackmann stages 5 and 6 (partial or complete transection of the facial nerve) are either minimal facial musculature movements or complete loss of function (grade 6).

Causes
Table 2. Causes of Facial Nerve Palsy in a Review of Medical Literature (1900-1990)*

Birth

Molding Forceps delivery Dystrophia myotonica Mbius syndrome (facial diplegia associated with other cranial nerve deficits) Trauma Basal skull fractures Facial injuries Penetrating injury to middle ear Altitude paralysis (barotrauma) Scuba diving (barotrauma) Lightning Neurologic Opercular syndrome (cortical lesion in facial motor area) Millard-Gubler syndrome (abducens palsy with contralateral hemiplegia caused by lesion in base of pons involving corticospinal tract) Infection External otitis Otitis media Mastoiditis Chickenpox Herpes zoster cephalicus (Ramsay Hunt syndrome) Encephalitis Poliomyelitis (type 1) Mumps Mononucleosis Leprosy Influenza Coxsackievirus Malaria Syphilis Scleroma Tuberculosis Botulism Acute hemorrhagic conjunctivitis (enterovirus 70) Gnathostomiasis Mucormycosis Lyme disease Cat scratch AIDS Metabolic Diabetes mellitus Hyperthyroidism Pregnancy Hypertension Acute porphyria Vitamin A deficiency Neoplastic Benign lesions of parotid Cholesteatoma Seventh nerve tumor Glomus jugulare tumor Leukemia Meningioma Hemangioblastoma Sarcoma Carcinoma (invading or metastatic)

Toxic

Iatrogenic

Idiopathic

Birth

Trauma

Anomalous sigmoid sinus Carotid artery aneurysm Hemangioma of tympanum Hydradenoma (external canal) Facial nerve tumor (cylindroma) Schwannoma Teratoma Hand-Schller-Christian disease Fibrous dysplasia Neurofibromatosis II Thalidomide (Miehlke syndrome, cranial nerves VI and VII with congenital malformed external ears and deafness) Ethylene glycol Alcoholism Arsenic intoxication Tetanus Diphtheria Carbon monoxide Mandibular block anesthesia Antitetanus serum Vaccine treatment for rabies Postimmunization Parotid surgery Mastoid surgery Post-tonsillectomy and adenoidectomy Iontophoresis (local anesthesia) Embolization Dental Familial Bell palsy Melkersson-Rosenthal syndrome (recurrent alternating facial palsy, furrowed tongue, faciolabial edema) Hereditary hypertrophic neuropathy (Charcot-Marie-Tooth disease, Dejerine-Sottas disease) Autoimmune syndrome Amyloidosis Temporal arteritis Thrombotic thrombocytopenic purpura Periarteritis nodosa Landry-Guillain-Barr syndrome (ascending paralysis) Multiple sclerosis Myasthenia gravis Sarcoidosis (Heerfordt syndrome, uveoparotid fever) Osteopetrosis Molding Forceps delivery Dystrophia myotonica Mbius syndrome (facial diplegia associated with other cranial nerve deficits) Basal skull fractures Facial injuries Penetrating injury to middle ear Altitude paralysis (barotrauma)

Scuba diving (barotrauma) Lightning Neurologic Opercular syndrome (cortical lesion in facial motor area) Millard-Gubler syndrome (abducens palsy with contralateral hemiplegia caused by lesion in base of pons involving corticospinal tract) Infection External otitis Otitis media Mastoiditis Chickenpox Herpes zoster cephalicus (Ramsay Hunt syndrome) Encephalitis Poliomyelitis (type 1) Mumps Mononucleosis Leprosy Influenza Coxsackievirus Malaria Syphilis Scleroma Tuberculosis Botulism Acute hemorrhagic conjunctivitis (enterovirus 70) Gnathostomiasis Mucormycosis Lyme disease Cat scratch AIDS Metabolic Diabetes mellitus Hyperthyroidism Pregnancy Hypertension Acute porphyria Vitamin A deficiency Neoplastic Benign lesions of parotid Cholesteatoma Seventh nerve tumor Glomus jugulare tumor Leukemia Meningioma Hemangioblastoma Sarcoma Carcinoma (invading or metastatic) Anomalous sigmoid sinus Carotid artery aneurysm Hemangioma of tympanum Hydradenoma (external canal) Facial nerve tumor (cylindroma) Schwannoma Teratoma Hand-Schller-Christian disease

Toxic

Iatrogenic

Idiopathic

Fibrous dysplasia Neurofibromatosis II Thalidomide (Miehlke syndrome, cranial nerves VI and VII with congenital malformed external ears and deafness) Ethylene glycol Alcoholism Arsenic intoxication Tetanus Diphtheria Carbon monoxide Mandibular block anesthesia Antitetanus serum Vaccine treatment for rabies Postimmunization Parotid surgery Mastoid surgery Post-tonsillectomy and adenoidectomy Iontophoresis (local anesthesia) Embolization Dental Familial Bell palsy Melkersson-Rosenthal syndrome (recurrent alternating facial palsy, furrowed tongue, faciolabial edema) Hereditary hypertrophic neuropathy (Charcot-Marie-Tooth disease, Dejerine-Sottas disease) Autoimmune syndrome Amyloidosis Temporal arteritis Thrombotic thrombocytopenic purpura Periarteritis nodosa Landry-Guillain-Barr syndrome (ascending paralysis) Multiple sclerosis Myasthenia gravis Sarcoidosis (Heerfordt syndrome, uveoparotid fever) Osteopetrosis

* Adapted from May and Klein

A common entity of facial nerve paralysis is Bell palsy, a form that is unilateral and considered of idiopathic etiology. The incidence of Bell palsy is approximately 20 cases per 100,000 per annum.18 o A viral etiology (ie, herpes simplex virus and others) has been suspected as precursor inciting factors.19 o Bell palsy normally has a sudden onset often preceded by facial dysesthesia, epiphora, pain, hyperacusis, dysgeusia, and decreased function of the lacrimal gland.20 Ramsay Hunt described a syndromic occurrence of facial paralysis, herpetiform vesicular eruptions, and vestibulocochlear dysfunction.21 o Patients presenting with Ramsay Hunt syndrome generally have a greater risk of hearing loss than patients with Bell palsy, and the course of disease is more painful. Moreover, a lower recovery rate is observed in these patients.22 o Medical treatment is equivalent to Bell palsy; most often a combination of steroids and antiviral agents is used.23,24

Infection with Borrelia burgdorferi via tick bites reveals another etiology of facial paralysis, thereby presenting along with all the symptoms of Lyme disease. Of patients affected with Lyme disease, 10% develop facial paralysis, 25% of whom present with bilateral palsy.25 Bacterial infection also may lead to facial nerve paralysis, most often correlated to acute otitis media or externa. Slow-onset facial nerve palsy is observed in patients with cholesteatoma. Noninfectious causes of facial nerve palsy include head trauma affecting the intracranial intratemporal course of the facial nerve or, less commonly, the infratemporal cause as seen in facial blunt or sharp injury. Iatrogenic injury to the facial nerve most often is seen after cervicofacial rhytidectomies, surgery of the parotid gland, acoustic neuroma resection, or tumor resection at any point along the course of the facial nerve. Therefore, when facial paralysis occurs after surgery, operative exploration must follow if uncertainty exists concerning the intactness of the facial nerve. Due to topographic relations and/or tumor extension, the facial nerve occasionally must be sacrificed voluntarily as part of sound oncologic management. Tumor of the facial nerve (eg, hemangioma, neuroma) or tumors in the direct vicinity of the facial nerve often are concomitant with facial nerve palsy. In general, gradual onset of paralysis may lead to suspected tumor cause; however, several authors have demonstrated a sudden onset of facial nerve palsy in patients with tumors (20-27%).26,27 Differential Diagnoses Diabetes Mellitus, Type 1 - A Review Diabetes Mellitus, Type 2 - A Review Fractures, Mandible Herpes Zoster Multiple Sclerosis Tick-Borne Diseases, Lyme Other Problems to Be Considered Herpes zoster Ramsey-Hunt syndrome Zoster sine herpete Pregnancy (especially third trimester) Polyneuritis Acute otitis Chronic otitis Temporal bone fracture Infectious mononucleosis Parotid tumors Sarcoidosis Cholesteatoma of the middle ear Aneurysm of vertebral, basilar artery, or carotid arteries Carcinomatous meningitis Facial trauma (blunt, penetrating, iatrogenic) Leukemic meningitis Leprosy Melkersson-Rosenthal syndrome Middle ear surgery Osteomyelitis of the skull base Skull base tumor Guillain-Barre Workup Laboratory Studies

No specific laboratory tests exist to confirm the diagnosis of Bell's palsy. Clinical setting determines tests that may be of value. Results of the following laboratory tests may confirm or suggest other potential causes in the differential diagnosis: o Complete blood count o Erythrocyte sedimentation rate o Thyroid function studies o Lyme titer o Serum glucose level o Rapid plasma reagin (RPR) or Venereal Disease Research Laboratory (VDRL) test o Human immunodeficiency virus (HIV) antibodies o Cerebral spinal fluid analysis o Immunoglobulin M (IgM), immunoglobulin G (IgG), and immunoglobulin A (IgA) titers for CMV; rubella; HSV; hepatitis A virus; hepatitis B virus; hepatitis C virus; VZV; M pneumoniae; and Borrelia burgdorferi Imaging Studies Bell's palsy remains a clinical diagnosis. Imaging studies are not indicated in the ED. Excluding other causes of facial palsy may require one of the following imaging studies, depending on clinical setting. o Facial CT scan or plain radiographs: Perform to rule out fractures or bony metastasis. o CT scan: Perform when the differential diagnosis includes stroke or CNS involvement from acquired immunodeficiency syndrome (AIDS). o MRI: Perform in patients with a suspected neoplasm of the temporal bone, brain, parotid gland, or other structure, or to evaluate for multiple sclerosis. MRI can visualize the course of the facial nerve through the intratemporal and extratemporal regions from the brain to the facial muscles and glands. MRI also may be considered in lieu of CT scan. Other Tests Electrodiagnosis of the facial nerve: These studies assess the function of the facial nerve. These tests are rarely performed on an emergent basis.10 o Electromyography (EMG) and nerve conduction velocities produce a graphic readout of the electrical currents displayed by stimulating the facial nerve and recording the excitability of the facial muscles it supplies. Comparison to the contralateral side helps determine the extent of nerve injury and has prognostic implications. This is not part of the acute workup. o The nerve excitability test determines the threshold of the electrical stimulus needed to produce visible muscle twitching. o Electroneurography (ENoG) compares evoked potentials on the paretic side versus the healthy side. Treatment Emergency Department Care The primary treatment of patients with Bell's palsy in the ED is pharmacological management. The remainder of care focuses on reassurance, eye care instructions, and appropriate follow-up care. Steroids: Inflammation and edema of the facial nerve is thought to cause the pathogenesis of Bell's palsy.10 Anti-inflammatory medications such as corticosteroids are thoughts to counter these effects. 19 o Older studies have shown conflicting results using steroids in treating Bell's palsy. These studies have been limited in their size. However, 2 recent randomized controlled trials showed significant improvement in outcomes when prednisolone was started within 72 hours of symptom onset.20,21 o Based on these 2 studies, steroids should be strongly considered to optimize outcomes. Once the decision to use steroids is made, the consensus is to start immediately. Antiviral agents o Evidence evaluating the efficacy of antiviral medicines in Bell's palsy has shown limited benefit,16,22 with 2 recent randomized controlled trials showing no benefit.20,21 However, there is

evidence to suggest a large percentage of Bell's palsy cases may result from a viral infection.16,23 Therefore, antiviral agents may be reasonable in certain situations. o Quant et al conducted a meta-analysis of published studies from 1984 to January 2009 to compare use of corticosteroids plus antiviral agents with corticosteroids alone on degree of facial muscle recovery in patients with Bell's palsy.30 Six trials (representing pooled data of 1145 patients) were examined and included 574 patients who received corticosteroids alone and 571 patients who received corticosteroids and antiviral agents. The analysis showed no improved benefit for Bell's palsy with use of corticosteroids plus antivirals compared with corticosteroids alone (odds ratio 1.50; 95% confidence interval [CI], 0.83-2.69; P=0.18). o Contrary to the Quant et al meta-analysis, de Almeida et al found that, when combined with corticosteroids, antiviral agents were associated with greater risk reduction of borderline significance compared with corticosteroids alone (relative risk [RR], 0.75; 95% CI, 0.56-1.00; P =.05).31 Their meta-analysis included 18 trials including 2786 patients. If antivirals are to be initiated, they should be done so in conjunction with corticosteroids. Future studies will be needed to determine which population will most beneift from antiviral therapy. Eye care: Impaired eye closure and abnormal tear flow are common with Bell's palsy. These leave the eyes at risk for corneal drying and foreign body exposure. Manage with tear substitutes, lubricants, and eye protection. o Artificial tears: Use these during waking hours to replace diminished or absent lacrimation. o Lubricants are used during sleep. They may be used during waking hours if artificial tears cannot provide adequate protection. One disadvantage is blurred vision during waking hours. o Eyeglasses or shields protect the eye from injury and reduce drying by decreasing direct contact of air currents with the exposed cornea. Eye patches, however, are ineffective because unopposed third nerve function will result in corneal exposure despite best efforts to approximate eyelid margins. Consultations The patient's primary care provider or consultant should provide close follow-up care. Documentation should chart the progress of the patient's recovery. Opinions widely vary on referral to a specialist. Some specific referral indications are listed below: Neurologist: Consult a neurologist when other neurologic signs are identified on physical examination and for an atypical presentation of Bell's palsy. Ophthalmologist: Consult an ophthalmologist for any unexplained ocular pain or abnormal findings on physical examination of the eyes. Otolaryngologist: Consult an otolaryngologist for patients with persistent paralysis, prolonged weakness of the facial muscles, or recurrent weakness. Surgeon: Surgery to decompress the facial nerve is occasionally recommended for patients with Bell's palsy. Patients with a poor prognosis, identified by facial nerve testing or persistent paralysis, appear to benefit the most from surgical intervention. However, studies have been mixed as far as benefit from surgery.24 It is recommended that if surgery is decided, it should be within 14 days from onset of symptoms.10 Medication Watchful waiting is an option for management of Bell's palsy, because most cases resolve without medication. However, some individuals with Bell's palsy never fully recover. For both classes of medications listed below, there are clinical trials that support their efficacy and trials that dispute it. Corticosteroids These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli. Prednisone (Deltasone, Orasone, Sterapred) Therapeutic success may be the result of anti-inflammatory effect, which presumably decreases compression of the facial nerve in the facial canal.

Adult 1 mg/kg/d PO up to 60 mg/d for 7-10 d Pediatric Administer as in adults Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics Documented hypersensitivity; viral, fungal, connective tissue, and tubercular skin infections; peptic ulcer disease; hepatic dysfunction; GI disease Pregnancy B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals Precautions Abrupt discontinuation of glucocorticoids may cause adrenal crisis; complications of glucocorticoid use include hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections Antivirals Herpes simplex infections may be a common cause of Bell's palsy. Acyclovir is the antiviral agent most often used, but others may also be appropriate Valacyclovir is a prodrug of acyclovir and produces blood levels of acyclovir that are 3-5 times higher than those produced by oral acyclovir. Valacyclovir (Valtrex) Prodrug rapidly converted to the active drug acyclovir. More expensive but has a more convenient dosing regimen than acyclovir. Adult 1000 mg/24 h PO for 5 d Pediatric Not established Probenecid, zidovudine, or cimetidine coadministration prolongs half-life and increases CNS toxicity of valacyclovir Documented hypersensitivity Pregnancy B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals Precautions Caution in renal failure (decrease dose) and coadministration of nephrotoxic drugs; associated with onset of hemolytic uremic syndrome Acyclovir (Zovirax) Has demonstrated inhibitory activity directed against both HSV-1 and HSV-2, and infected cells selectively take it up. Adult 4000 mg/24 h PO for 7-10 d Pediatric <2 years: Not recommended >2 years: 1000 mg PO divided qid for 10 d Follow-up Inpatient & Outpatient Medications Prednisone at an initial dose of 1 mg/kg/d up to 60 mg should be strongly considered within 72 hours of onset of symptoms if no contraindications exist.

Prednisone is a potent drug with a potential for side effects. While older studies are mixed in showing efficacy,19 2 recent, double-blinded, randomized controlled trials showed benefit if treatment was started within 72 hours.20,21 o The best prednisone regimen for Bell's palsy is a short burst (up to 10 d), but steroid taper may also be used. o Caution should be given to patients who are immunocompromised, pregnant, or have poorly controlled diabetes, severe peptic ulcer disease, an active infection, sarcoidosis, or sepsis. o High-dose steroids (>120 mg/d of prednisone) have been safely used to treat Bell's palsy in patients with diabetes;25,26 however, optimal dosing has not been established. Caution should be given in these cases due to the risk of hyperglycemia. Consider using antivirals within 72 hours if there is a high suspicion for HSV or VZV as a cause. o Administer acyclovir (Zovirax) 800 mg PO 5 times/d for 10 d; 20 mg/kg in patients younger than 2 years. Evidence supports HSV as a major cause of Bell's palsy. However, evidence of efficacy with acyclovir has been mixed.22 A recent trial showed no additional benefit with the addition of acyclovir to prednisolone.20 o Valacyclovir (Valtrex), 500 mg PO twice a day for 5 days, may be used instead of acyclovir. Although more expensive, this may lead to better compliance. If VZV is the cause, higher doses may be needed (1000 mg PO tid). However, evidence of efficacy with valacyclovir has been mixed.16,22,23 A recent trial showed no additional benefit with the addition of high-dose valacyclovir to prednisolone.21 Because of increased cost and increased risk of side effects with higher doses, valacyclovir cannot be routinely recommended at this time. Complications Most patients with Bell's palsy recover without any cosmetically obvious deformities; however, approximately 5% are left with an unacceptably high degree of sequelae. Incomplete motor regeneration o The largest portion of the facial nerve comprises efferent fibers that stimulate muscles of facial expression. Suboptimal regeneration of this portion results in paresis of all or some of these facial muscles. o This manifests as (1) oral incompetence, (2) epiphora (excessive tearing), and (3) nasal obstruction. Incomplete sensory regeneration o Dysgeusia (impairment of taste) may result. o Ageusia (loss of taste) may result. o Dysesthesia (impairment of sensation or disagreeable sensation to normal stimuli) may result. Aberrant reinnervation of the facial nerve o During regeneration and repair of the facial nerve, some neural fibers may take an unusual course and connect to neighboring fibers. This aberrant reconnection produces unusual neurologic pathways. o When voluntary movements are initiated, they are accompanied by involuntary movements (eg, the movement of a closed eye following that of the uncovered one). These involuntary movements accompanying voluntary movement are termed synkinesis. Prognosis The natural course of Bell's palsy varies from early complete recovery to substantial nerve injury with permanent sequelae. Prognostically, patients fall into 3 groups: o Group 1 - Complete recovery of facial motor function without sequelae o Group 2 - Incomplete recovery of facial motor function, but no cosmetic defects are apparent to the untrained eye o Group 3 - Permanent neurologic sequelae that are cosmetically and clinically apparent Patients who experience incomplete facial paralysis during the acute phase have an excellent prognosis for full recovery. Patients demonstrating complete paralysis are at higher risk for severe sequelae.7

Other factors that determine worsening prognosis are history of recurrence,27 diabetes,25 increased age, longer onset of time to recovery, presence of postauricular pain, abnormal taste, stapedius reflex, and tearing.7 Of patients with Bell's palsy, 85% achieve complete recovery, 10% have some persistent asymmetry of facial muscles, and 5% experience severe sequelae. The prognosis in pregnant women with Bell's palsy may be slightly worse than it is in nonpregnant women with Bell's palsy.12 8 Bilateral Bell's palsy is thought to share the same prognosis as unilateral Bell's palsy. Patient Education Eye care o Protect the eye from foreign objects and sunlight. o Keep the eye well lubricated. o Educate the patient to report new ocular findings such as pain, discharge, or visual changes. For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. Also, see eMedicine's patient education article Bell Palsy.