Approach to the patient with Facial Weakness

Facial weakness is usually due to dysfunction of the facial nerve (CN VII) or its supranuclear pathways, leading to paresis of facial movements on one side - broadly classified into lesions of the nerve itself (lower motor neuron) vs lesions of the corticobulbar pathways that innervate and send signals to this nerve (upper motor neuron) - differentiated by involvement of the upper face on the affected side; which is spared (esp frontalis) in UMN lesions while there is equivalent weakness of eye closure and eyebrow raising in LMN lesions

Anatomy: The facial nerve (CN VII) is a mixed motor and sensory nerve - the motor division arises from the motor nucleus in the caudal pontine tegmentum medial to the nucleus of CN V and anterolateral to CN VI nucleus - the nerve courses medially and posteriorly around the CN VI nucleus to create the facial colliculus and then ventrally to exit in the cerebellopontine angle close to the fifth, sixth and eight CNs - its supranuclear control is via corticobulbar fibres originating in the lower 1/3 of the precentral gyrus ("motor strip") which traverse the corona radiata, genu of the internal capsule and medial cerebral peduncle to synapse in the pons - majority of the fibres decussate but there is bilateral innervation to the dorsal portion (which supplies muscles of the upper face, esp frontalis); so spared relatively in UMN lesions - this pathway is for voluntary facial movements while mimetic facial movements (ie. involuntary, eg smiling spontaneously) are mediated by pathways traversing striatum or globus pallidus (and especially involved if lesions of right hemisphere) - nervus intermedius (of Wisberg) supplies the preganglionic parasympathetic fibres to the submaxillary ganglion (innervating the submandibular and sublingual salivary glands) and to pterygopalantine / sphenopalantine ganglia (to lacrimal, palatal and nasal glands) - sensory input comes from geniculate ganglion (taste from anterior 2/3 of tongue and mucosa of pharynx, nose, palate, and external auditory meatus incl. lateral pinna) - parasympathetic fibres originate in superior salivary nucleus (in pontine tegmentum) and lacrimation from lacrimal nucleus

- gustatory afferents terminate in nucleus tractus solitarius (medulla) while other sensory fibres end in spinal nucleus of trigeminal nerve - its peripheral course is together with CN VIII entering internal auditory meatus to facial canal in petrous bone where it reaches the geniculate ganglion - chorda tympani (as well as nerve to stapedius) arises in the mastoid (vertical) segment in middle ear; this supplies the submaxillary ganglion and afferents from taste - exits facial canal through stylomastoid foramen giving off branches to various muscles - pierces the parotid gland and supplies more muscles

History:

Onset: - was this acute (as with stroke or Bell's palsy) and static or was it insidious and progressive (as with mass lesions or leptomeningeal inflammations or malignancies) ? - how first noticed (by others, self in mirror, trouble closing eye - shampoo gets in eye while showering esp LMN or food accumulating in corner of mouth or drooling out of one side)

Pain: - swelling of the nerve (eg Bell's Palsy / inflammatory) commonly leads to entrapment near stylomastoid foramen, causing localized pain anterior to tragus of ear (in 50% of Bell's palsy) - otalgia or pain behind ear may occur due to involvement of the geniculate ganglion - severe pain seen with Ramsay-Hunt syndrome (geniculate neuralgia)

Hyperacusis: - subjective feeling that noises sound louder in ipsilateral ear (due to weakness of stapedius which dampens sound vibrations); esp for low tones; seen in 14% of Bell's palsy - only seen in peripheral localization with lesion proximal to stylomastoid foramen (as nerve to stapedius leaves while CN VII still in facial canal)

Dysgeusis:

- alteration in taste implies lesion proximal to point where chorda tympani joins facial nerve

Lacrimation: - despite potential impairment of lacrimation (if proximal lesion) still often see increased tearing ipsilaterally, due to irritation from inability to close eye and dysfunction of tear duct - few complain of dry eyes

Examination:

Of facial weakness itself: 1. Motor: - inspect the face at rest and with voluntary (and mimetic) movements - examine symmetry of eye blinking and speech motion (subtle lesions may only lead to asymmetry of blink rate !) - ask to raise eyebrows (frontalis), close eyes (orbicularis oculi), show teeth (orbicularis oris), blow out cheeks (buccinator), scrunch up nose (nasalis) and retract chin (platysma) NB: with true eye closure weakness, when try to pry them open, should see globe rotated upward (Bell's phenomenon) due to contraction of superior rectus (absent in 10% of normals) - determine whether peripheral (LMN) or central (UMN) lesion; in former see no furrows on affected forehead and ipsilateral eye open wider (unable to close) - look for signs of hemifacial spasm (contraction of facial muscles on one side due to nerve irritation) which can falsely mimic contralateral facial weakness

2. Sensory: - see if associated loss of taste on anterior two-thirds of tongue - test for sweet and salty on one half vs. unaffected side - only involved with peripheral lesions as supranuclear or nuclear motor lesions do not affect this sensory function

3. Reflexes: - corneal reflex may be reduced ipsilaterally (if LMN lesion) due to weak efferent loop (while consensual blinking on unaffected side remains intact)

Associated brainstem abnormalities: - to localize a nuclear (LMN) lesion of CN VII should seek other nearby brainstem problems - usually affects CN VI (abduction, lateral rectus paresis) or conjugate gaze (PPRF) - corticospinal tract may be involved in basis pontis (as nerve exits) with CL hemiparesis - occasionally trigeminal nerve and spinothalamic tract (crossed sensory loss)

Cerebellopontine Angle abnormalities: - loss of taste and hyperaccusis will be present - associated with ipsilateral hearing loss, tinnitus +/- vertigo - if extend to adjacent pons or cerebellum, may see gaze paresis, ataxia, sensory loss - similar abnormality with temporal bone fracture involving facial nerve in bone, including loss of hearing and impaired lacrimation

Look for vesicles in the ear canal or tympanic membrane as well as palate - seen in varicella-zoster reactivation involving the seventh CN (Ramsay-Hunt) - commonly also see vestibulocochlear dysfunction with hearing loss, vertigo

Differential Diagnosis of Facial Weakness:

1. UMN Pattern (supranuclear) - requires brain imaging with CT or MRI - Infarct or hemorrhage along corticobulbar pathway (cortical or subcortical) - Tumor or other mass lesion (incl. focal infections) - Demyelinating or other inflammatory lesions

2. Nuclear lesions: - with associated brainstem abnormalities - ischemic or hemorrhagic lesions (incl cavernoma), demyelinating focus or pontine gliom

3. LMN Pattern (peripheral): a. Bell's palsy; idiopathic (see below for complete differential) b. Traumatic c. Infectious (esp VZV, HIV, Lyme disease, basal meningitis) d. Inflammatory (esp sarcoidosis) e. Neoplastic (esp schwannoma or CPA meningioma) f. Metabolic (esp diabetes mellitus, pregnancy)

Bell's Palsy:

Acute unilateral mononsymptomatic and idiopathic peripheral CN VII palsy

Epidemiology: - the most common peripheral disorder of CN VII (accounts for 2/3 of LMN VII palsies) - occurs in 23 per 100,000 persons per year - affects men & women equally, all ages, and all year round (peak at ages 15-45 yrs) - each side affected with same frequency - lesion most commonly proximal to geniculate ganglion and role of HSV-1 viral infection (or postviral) implicated; alternatively role of ischemia and microinfarction of the nerve (eg DM)

History: - usually acute onset over a day or so of unilateral facial weakness - LMN pattern so problems with closing eyes, eating, drooling - may be preceded for 1-2 days by pain around the ear - ask about dysgeusis (alteration in taste) or hyperaccusis which do not always occur and localize the lesion proximal to where chorda tympani and nerve to stapedius originate - any prior history of facial palsy (is this recurrent or episodic)

R/O other problems: - no symptoms of brainstem dysfunction such as diplopia / gaze palsy, sensory loss in face, or hearing loss / vertigo - no severe pain in or around ear to suggest Herpes Zoster Oticus (Ramsay-Hunt)

Examination: - LMN pattern of facial weakness involving all muscles on that side - no objective sensory loss (though face may feel "heavy") or other neurological abnormalities (no pronator drift or other pyramidal deficits on same side to suggest an UMN pattern of corticospinal / corticobulbar abnormality) - look at the external ear and palate for vesicles suggesting VZV reactivation (Ramsay-Hunt)

Differential Diagnosis: see above for full list LMN Facial weakness: - Infections (VZV, HIV, Lyme disease, Leprosy, Syphilis, meningitis incl. TB, Parvovirus B19) - Parotid swelling (incl. mononucleosis, mumps, mass lesions) - Inflammatory diseases incl. sarcoidosis, Wegener's granulomatosis, giant cell arteritis, polyarteritis nodosa and Behcet's disease; also Guillain-Barre syndrome - Leptomeningeal processes incl. meningitis (see above), carcinomatosis

- Neoplasms compressing the nerve incl. cholesteatoma, facial nerve schwannoma, meningioma or vestibular schwannoma at CPA, glomus jugulare tumor, carcinoma (or other tumors) in ear, parotid tumors - Traumatic injuries to the facial nerve incl. temporal bone fractures, birth trauma / forceps delivery, or post-operative (ear surgery) - Metabolic dysfuntion incl. diabetes mellitus, hypothyroidism, uremia, porphyria - Drug reactions incl. Stevens-Johnson, isoniazid, lidocaine - Congenital defects of CN VII incl. Mobius syndrome (abducens palsy) or familial - Melkersson-Rosenthal syndrome with recurrent facial palsy associated with labial edema and plications of tongue

Investigations: - none required for typical case without other neurological features or concerning history - imaging of the facial nerve course with CT scan or MRI if suspect mass lesion compressing or trauma history (assess for basal skull fracture) NB: in Bell's palsy may see enhancement of distal intracanalicular and labyrinthine segments of facial nerve, geniculate ganglion and other portions (swelling of the nerve is normal) - ESR, glucose, VDRL, HIV and vasculitis testing in appropriate situations - CSF analysis if suspect leptomeningeal disease (for cytology, cells, ACE level, VDRL)

Electrophysiology

Help with prognostication and mainly done if no prompt resolution of facial weakness when peripheral lesion of the nerve suspected clinically

1. Blink response (direct R1, indirect R2) - normal (unusual with significant facial palsy), delayed, or absent

2. Direct response from nasalis (equivalent to facial CMAP)

- comparison of amplitudes on affected vs unaffected side

3. Transcranial magentic stimulation: - response may be absent early on due to edema, while still reversible - absent not necessarily poor prognosis but presence of response means likely to have good recovery (esp if < 4d from onset)

Initial testing may be done within 10 days of onset - full recovery if blink response present and direct response > 25% of normal side - poor recovery if direct response < 25% (in this case, blink R1 usually absent)

If blink response absent at initial testing but present at 1 month (with DR > 25%) then usually still good recovery

Natural History: - recovery complete or with only mild residual weakness in over 90% - 5-10% have mod-severe residual weakness (usually only if complete severe weakness at initial presentation) - 80% begin to recover within 3 weeks (demyelinating lesion) while 15% start their recovery only by 3-5 months - almost all recovery has occurred by 6 months, and little more expected after 1 year - poor prognosis if older, complete initial weakness (if partial then 94% make complete recovery), if not start to recover within 3 weeks, presence of post-auricular pain and associated conditions (diabetes, pregnancy, VZV infection); also if taste impairment - see also electrophysiologic parameters above

- risk of recurrence if low for Bell's palsy - if recurrent then consider inflammatory conditions, myasthenia gravis or lesions at skull base (incl. meningitis, sarcoidosis or tumors - lymphoma)

- sequelae include residual weakness in 10-15%, contractures in 17%, and aberrant regeneration with synkinesis in 16% (incl. crocodile tears, tear when eat in 2%)

Treatment: - careful eye care including daytime drops to lubricate (else dry eyes) and nightime lubricanting ointment lacrilube) and taping eye shut (if cannot close fully) to proect from corneal abrasions and ulcers - if seen within first few days and significant weakness, then consider prednisone 1 mg/kg daily for 10 days then tapered off rapidly (shown to reduce time to full recovery but not affect ultimate degree of recovery at 1 year) - optional (with weak evidence) to use acyclovir orally if seen early, as HSV infection is commonly implicated in Bell's palsy (but not often proven) - decompressive surgery has been advocated but complications include hearing loss (permanent) in 1-15% and no definite benefit over medical management proven - no point decompressing > 14 days from onset when severe damage and degeneration done but role of reconstructive facial nerve surgery later for complete transection possible

Bilateral Facial Weakness:

UMN Pattern (or non-neurogenic) - Myasthenia Gravis - Myopathies (incl. FSH, myotonic dystrophy, OPMD) - Bilateral strokes (usually with pseudobulbar palsy)

LMN Pattern: Facial Diplegia - unusual, accounting for less than 1% of facial weakness cases caused by: 1. Congenital:

- Mobius syndrome, Poland's anomaly (pectoralis hypoplasia with ipsilateral breast and upper limb abnormalities), maternal thalidomide use 2. Granulomatous / Connective Tissue diseases: - sarcoidosis (incl. uveoparotid fever), vasculitis (Sjogren's, SLE, GCA, Wegener's, PAN) 3. Infectious: - meningitis (incl. TB, cryptococcus), encephalitis, Lyme disease, HIV, leprosy, VZV, CMV, botulism, tetanus, diphtheria 4. Neoplasms: - pontine glioma, extra-axial lesions incl. epidermoid, cholesteatoma, ependymoma bilaterally or menigeal (leukemia, lymphoma) 5. Traumatic 6. Guillain-Barre syndrome 7. Multiple idiopathic cranial nerve palsies 8. Bulbospinal neuronopathies: incl. brainstem encephalitis, amyloidosis, Stevens-Johnson syndrome, diabetes mellitus, multiple sclerosis, intracranial hypertension, porphyria, osteopetrosis, ethylene glycol toxicity, HNPP, Tangier's disease

References: Gilden DH. Bell's palsy. N Engl J Med 2004; 351: 1323-31. Grogan PM, Gronseth GS. Practice parameter: steroids, acyclovir, and surgery for Bell's palsy (an evidence-based review). Neurology 2001; 56: 830-6. Reviewed by: pending review

Neurological Medicine Pocketbook 2003-2004 UWO Neurology Residents http://www.uwo.ca/cns/resident All Rights Reserved Disclaimer