FACIAL PALSYVivien Puspitasari

CASE A 42 year old man noticed while shaving one morning that he was unable to move the left side of his face. He worried that a serious problem possibly a stroke might have occurred. He had influenze-like symptoms the week before this sudden attack.

CASEGeneral Exam : NormalNeurologic examination : the patient could not wrinkle his forehead on the left side or show his teeth or purse his lips on that side. He had trouble closing his left eye. Taste sensation was abnormal in the left anterior two-thirds of the tongue, and a test to determine tear secretion showed that secretion on the right side was normal, but the left lacrimal gland produced little fluid. Loud noises caused discomfort in the patient, who was in good health otherwise, and there were no additional signs or symptoms.

What is the differential diagnosis?What is the most likely diagnosis

CRANIAL NERVE VII - FACIAL NERVEBrancial motorSupplies the muscles of facial expression; posterior belly of digastric muscle; stylohyoid, and stapedius. Visceral motor(general visceral efferent) Parasympathetic innervation of the lacrimal, submandibular, and sublingual glands, as well as mucous membranes of nasopharynx, hard and soft palate. Special sensory(special afferent) Taste sensation from the anterior 2/3 of tongue; hard and soft palates. General sensory(general somatic afferent) General sensation from the skin of the concha of the auricle and from a small area behind the ear

COURSE OF THE FACIAL NERVEIntracranial Arises at the pontomedullary junction and courses with CNVIII to the internal acoustic meatus - 12mmMeatal Anterior to the superior vestibular nerve and superior to the cochlear nerve 10mmIntratemporal Labyrinthe segmentPasses through narrowest part of fallopian canal - 12mmNarrowest part of facial nerve. The most susceptible to compression secondary to edema. Tympanic segmentFrom geniculate ganglion 11mmMastoid segmentExits the stylomastoid foramen 13mmExtracranial From stylomastoid foramen

CRANIAL NERVE VII - FACIAL NERVE

Corticobulbar Tract

DIAGNOSIS OF FACIAL WEAKNESSFIRST STEP : DETERMINE CENTRAL OR PERIPHERAL

Cells of the facial nucleus that innervate the lower face receive corticobulbar fibers primarily from the contralateral cerebral hemisphere

Cells of the facial nucleus that innervate the upper face receive corticobulbar fibers originating from both cerebral hemisphere

CENTRAL FACIAL PALSY

lesion above the level of the facial nuclei in the pons of the contralateral hemisphereA unilateral lesion in the cortex or underlying corticobulbar fibers usually produces contralateral voluntary central-type facial paralysis and contralateral hemiplegia but does not affect salivary & lacrimal secretions or the sense of taste

PERIPHERAL FACIAL PALSY

Weakness or paralysis of all muscles of facial expressionDue to a lesion of the ipsilateral facial nerve but can also be produced by a lesion of the ipsilateral facial nucleus or facial nerve in the ponsHyperacusis : paralysis of the stapedius muscleLesions proximal to the geniculate ganglion : permanent loss of taste & unable to produce tears

FACIAL NERVE DISORDERReduce function (palsies)Excessive activation of facial nerve-innervated muscles e.g hemifacial spasm

Facial neuropathyInfectiousInflammationNeoplasticTrauma

The most common cause of acute facial paralysis : Central : Stroke (hemisphere) Peripheral : Bells palsyHerpes zoster oticusStroke (brainstem)

PERIPHERAL FACIAL NERVE PALSYPrimary (75%) /idiopathic Bells palsySecondary (25%) Systemic viral infectionTraumaSurgeryDiabetesLocal infectionTumorImmunological disorder

BELLS PALSY~ 75% of all acute facial palsyHighest incidence 15 45 years old Incidence in UK 20/100000In pregnant women 45/100000

BELLS PALSYClinical picture varies, depending on the location of the lesion of the facial nerve along its course to the muscles Symptom:facial weakness with maximal onset developing within two days, associated symptoms : hyperacusis, decreased production of tears, altered taste, otalgia/retroauricular pain

BELLS PALSYRisk Factor Pregnancy

Diabetes mellitus

Age >30

Exposure to cold temperatures

Upper respiratory infection (e.g., coryza, influenza)

Will it recurrent ?

BELLS PALSYETIOLOGY

Inflammation of the facial nerve causes swelling and subsequent compression of both the nerve and the associated vasa nervorum

May arise secondary to reactivation of latent herpes virus (herpes simplex virus type 1 and herpes zoster virus) in cranial nerve ganglia

May arise secondary to ischemia from arteriosclerosis associated with diabetes mellitus

BELLS PALSYSIGNS AND SYMPTOMS

Weakness on affected side of face, often sudden in onset

Pain in or behind the ear in 50% of cases (may precede the palsy in 25% of cases)

Subjective numbness on the ipsilateral side of the face

Alteration of taste on the ipsilateral anterior 2/3 of the tongue (chorda tympani branch of the facial nerve)

Hyperacusis (nerve to the stapedius muscle)

Decreased tear production

HISTORY

Time course of the illness (e.g., rapid vs. slow onset)

Any predisposing factors (e.g., recent viral infection, trauma, new medications, hypertension, diabetes mellitus)

Presence of hyperacusis or history of recurrent Bell palsy (both associated with poor prognosis)

Any associated rash (suggestive of herpes zoster, Lyme disease, or sarcoid)

PHYSICAL EXAMNeurologic examination to determine if the weakness is due to a problem in either the central or peripheral nervous systems

Flaccid paralysis of muscles on the affected side, including the forehead

Impaired ability to raise the ipsilateral eyebrow

Impaired closure of the ipsilateral eye

Bell phenomenon: Upward diversion of the eye with attempted closure of the lid

Impaired ability to smile, grin or purse the lips

Patients may complain of numbness, but on sensory testing, no deficit is present.

Examine for involvement of other cranial nerves.

Bells phenomenon in facial palsyIf a patient tries to close their eyes but is unable to do so on the affected side, the globe will still move upwards in the usual fashion. The resut is that the white sclera fills most of the palpebral fissure.

EVALUATIONLaboratoryNot routinelyBlood glucose/ Hb A1CRadiologyX-rayCT scan / MRIElectromyography/Electroneurography

TREATMENT OF BELLS PALSYThe main aims of the treatment in acute phase : to speed recovery and to prevent corneal complicationTreatment should begin immediately to inhibit viral replication and the effect on subsequent patophysiological processes that affect the facial nerve

TREATMENT OF BELLS PALSYAAN Guideline:Early treatment with oral corticosteroid is probably effective in improving facial-function outcomes Addition of Acyclovir is possible effectiveInsufficient evidence exists to recommend facial-nerve decompression

TREATMENT OF BELLS PALSYCorticosteroidOral prednisone 1 mg/kg/day for 7 daysIn 2 14 days after the onsetAntiviralAcyclovir 800 mg five dailyValacyclovir 1 gr twice daily for 7 daysFamciclovir 750 mg three times dailyDecompression Still controversialShould not performed 14 days after onset

TREATMENT OF BELLS PALSYPhysiotherapyTo improve muscle contractionexercise, thermal, electrotherapy, massageCorneal protection: lucricantTarsoraphy, cosmetic surgery

PROGNOSIS OF BELLS PALSY71% untreated patient recover completely

84% near-normal function

20-30% permanent weakness

COMPLICATIONCrocodile tears syndromeTonic facial contractionHemifacial spasm

Crocodile tears syndromeAfter acute facial paralysis, preganglionic parasymppathetic fibers that previously projected to the submandibular ganglion may regrow and enter the major superficial petrosal nerve. Abberant regeneration lead to lacrimation after a salivary stimulus

PROGNOSIS OF BELLS PALSYIf complete facial paralysis is still present after 1 week of medical treatment

Electroneurography 90% degeneration , only 50% good recovery< 90%, 80-100% regain excellent function

HOUSE-BRACKMANN FACIAL NERVE GRADING SCALEINormalIINormal tone and symmetry at restSlight weakness on close inspectionGood to moderate movement of foreheadComplete eye closure with minimum effortSlight asymmetry of mouth with movementIIINormal tone and symmetry at restObvious but not disfiguring facial asymmetrySynkinesis may be noticeable but not severe+/- hemifacial spasm or contractureSlight to moderate movement of foreheadComplete eye closure with effortSlight weakness of mouth with maximum effortIVNormal tone and symmetry at restAsymmetry is disfiguring or results in obvious facial weaknessNo perceptible forehead movementIncomplete eye closureAsymmetrical motion of mouth with maximum effortVAsymmetrical facial appearance at restSlight, barely noticeable movementNo forehead movementIncomplete eye closureAsymmetrical motion of mouth with maximum effort

HERPES ZOSTER OTICUS (RAMSAY HUNT SYNDROME)10-15% of acute facial palsy casesLesions may involve the external ear, the skin of EAC or soft palateAssociated symptoms hearing loss, dysacusis and vertigoAdditional involvement of CN V, IX and X and cervical branches 2, 3 and 4Pathogenesis Neural injury due to edema at point between the meatal foramen and the geniculate fossa in the labyrinthe segment

Ramsay Hunt Syndrome

RAMSAY HUNT SYNDROMEWithout treatment 35% recover spontaneously

Steroid ---- no evidence

Acyclovir 4000 mg/hari for 7 10 hari

FRACTURESTrauma injury

-Temporal bone fracture

-Mandibular bone fracture

NEOPLASTICAbout 5% of cases of facial nerve paralysis are caused by tumors Characteristics of facial nerve palsySlow developing Additional cranial nerve deficits and or headacheRecurrent ipsilateral involvement AdenopathyPalpable neck or parotid massMost common benign tumor - facial nerve schwanommaMost common malignant tumors - mucoepidermoid carcinoma and adenoid cystic carcinoma of the parotid gland

CEREBROVASCULAR-Brainstem stroke involving antero-inferior cerebellar artery

-Aneurysm involving carotid, vertebral or basilar arteries

OTHERMultiple sclerosis

Myasthenia gravis (should be considered in cases of recurrent or bilateral facial palsy)

Guillain-Barre syndrome (may also present with bilateral facial palsy)

Sjogren syndrome

Sarcoidosis