FACIAL (VII) NERVE DISORDERS

Facial nerve: Anatomy +

2 roots
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Motor

Origin: From facial nucleus Projects to: Facial muscles; Stapedius; Digastric & Stylohyoid o Nervus intermedius Sensory afferents o Origin: Cell bodies in geniculate nucleus o Projections Skin: To spinal nucleus of cranial nerve V Taste: To nucleus tractus solitarius Preganglionic parasympathetics o From superior salivatory nucleus Branches o Large petrosal: Lacrimation & Salivation; Parasympathetic o Nerve to stapedius muscle: Origin in tympanic canal o Chorda tympani Joins lingual nerve Taste in anterior 2/3 of tongue Preganglionic Parasympathetic fibers o To submandibular ganglion o To sublingual & submaxillary glands o Sensory auricular branch Sensation o Posterior external auditory canal o Inferior ear pinna Some axons may arise from vagus (Arnold's nerve) o Motor innervation: Facial muscles (Facial expression) Facial nerve: Anatomy Neighboring nerves VI: Internal genu of VII nerve curves around VI nerve nucleus VIII: Accompanies VII nerve in internal acoustic canal EMG: Motor units vs Limbs o Amplitude: Smaller o Duration: Shorter o Firing rate: Faster
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Facial nerve paralysis

Clinical features o Facial asymmetry o Eyebrow droop o Loss of forehead & nasolabial folds o Drooping of corner of mouth o Uncontrolled tearing o Inability to close eye o Lips not held tightly together: Difficulty keeping food in mouth o Facial muscle atrophy (Late) Electrophysiology o EMG Denervation Synkinesis: Late o Blink reflex Abnormal ipsilateral o R1 (early, disynaptic) o R2 (late multisynaptic) responses

Synkinesis (Late)

Bell's Palsy8

Epidemiology o Lifetime prevalence: 6.4 to 20 per 1,000 o Incidence: Increased with age Overall: 0.2 to 0.5 per year per 1,000 Age 20: 0.1 per year per 1,000 Age 80: 0.6 per year per 1,000 o Male = Female, or slight Female Sir Charles Bell predominance o Recurrence: 7% o Side: Right in 63% o Increased incidence Diabetes Pregnant females: Especially in 3rd trimester13 Pathogenesis o Evidence for herpes simplex type 1 infection Clinical Features o Onset Paralysis: Progresses to maximal defecit over 3 to 72 hours Pain (50%): Near mastoid process Excess tearing (33%) Other: Hyperacusis; Dysgeusia Facial Paresis: Left o Signs Facial weakness o All branches of nerve: Upper & Lower o Unilateral o Degree: Partial (30%); Complete (70%) Stapedius dysfunction (33%): Hyperacusis Lacrimation: Mildly affected in some patients Taste: No clinically significant changes in most patients Sensory loss7 o Mild or None o May be present on face or tongue: On side of paralysis o Possibly related to involvement of greater superficial petrosal nerve o Prognosis better Disease features

Incomplete paralysis Early improvement Slow progression Normal Salivary flow Taste Hearing Patient characteristics o Younger age o No history of prior Bell's palsy Electrodiagnostic tests normal o Nerve excitability o Electrogustometry Early treatment ( 3 days)
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Course

Improvement onset: 10 days to 2 months Plateau: 6 weeks to 9 months Residual signs o Synkinesis Frequency: ~50%; May be reduced by corticosteroid treatment May be treated with botulinum Probably due to misregeneration of nerve o Face weakness: 30% o Contracture: 20% o Crocodile tears: 6% o Blepharospasm: May occur years after paralysis o Evaluation: Some patients Chest X-ray MR imaging: Especially in atypical cases with slow progression Lumbar puncture Lyme titers Electrodiagnositic testing: In severe paresis to determine extent of injury o Variant: Bilateral simultaneous facial palsy12 Frequency: 0.4% of Bells palsy Onset in 2nd nerve: 1 to 6 days after 1st nerve paresis Severity: Often severe bilaterally Prognosis: Similar to unilateral Bell's palsy Differential diagnosis: Infectious mononucleosis; Ramsay Hunt syndrome Treatment of Bell's palsy o Statistical degrees of benefit from drug treatment Prednisone > Placebo More benefit when treatment started within 3 days of onset No benefit from treatment starting more than 10 days after onset

Corticosteroids Use within one week of onset Adults: Prednisone 80 mg qd x 5 days Children: Prednisone 1 mg/kg/day After initial dose: Taper off over 7 to 10 days o Anti- viral medications: No clear benefit 11 Acyclovir5 o Adults 2,000 mg per day (400 mg 5x/day) for 7 days With varicella zoster 4,000 mg per day o Children: 80 mg/kg per day for 5 days o ? More effective in Ramsay-Hunt syndrome Alternative anti-viral: Valacyclovir o Protect eye from exposure o ? Facial exercise Laboratory o CSF: Protein high in 30%; Cells in 10% o Calorics: Often reduced on affected side o CNS imaging (MRI with gadolinium) Localization of lesion9 o Proximal facial (suprageniculate) segment: Intrameatal (Distal), Labyrinthine & Geniculate ganglion o Peripheral segment rarely involved Indications o No improvement in facial paresis after 1 month o Hearing loss o Multiple cranial nerve deficits o Signs of limb paresis or sensory loss
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VII disorders: Differential Diagnosis

Unilateral VII weakness o Idiopathic: Bell's palsy o Sarcoid & other granulomatous disorders o Infection Leprosy: Especially with paralysis of upper face Otitis media Lyme disease HIV Herpes Zoster (Ramsay Hunt syndrome) o Neoplasm & Masses o Trauma o Cardiofacial syndrome : Lower lip or complete facial palsy o Familial Recurrent facial Recurrent cranial nerve

CNS lesions

Bilateral VII weakness o General Definition: 2nd facial nerve paresis occuring within 30 days of 1st Frequency: 0.3% to 2% of patients with facial paralysis o VII nerve lesions Melkersson syndrome Mbius syndrome Guillain Barr: Variant Leprosy An--lipoproteinemia (Tangier) HIV infection: May occur before seroconversion Lyme disease o Other peripheral causes Motor neuron disorders Myasthenia gravis Myopathies

Central VII lesions o Pyramidal: Lower face paralysis with voluntary motion o Emotional2: Face paralysis with emotion Anatomy: Dorsolateral pons lesion Disorders o Superior cerebellar artery infarction Unilateral With Deafness, Horner's, or reduced Sweating o Extrapyramidal disorders Bilateral Parkinsonism

Hemifacial spasm

Melkersson-Rosenthal syndrome

Chromosome 9p11; Dominant

Clinical o Onset 5 to 70 years Incomplete penetrance o Recurrent facial palsy Unilateral or Bilateral May include loss of taste o Edema: Facial, Lip & Eyelid; Non-pitting External link: Image o Onset: < 16 years o Lingua plicata (Scrotal tongue) Laboratory o Eyelid pathology: Granulomatous lymphangitis

Mbius & Congenital facial syndromes

Mbius syndrome: General Features4

General: Rhombencephalic maldevelopment Onset: Congenital Facial paresis o Unilateral or Bilateral o Upper > Lower Ophthalmoplegia o Common o Especially VI nerve: Inability to abduct past midline o Patterns Bilateral: 90% Abducens nerve palsy, isolated: 9% Conjugate horizontal gaze paresis: 48% Duane retraction syndrome: 34% Congenital fibrosis of extraocular muscles: 9% Bulbar o Palate and pharynx dysfunction: 56% Dysarthria, nasal Poor feeding Chewing defects: Associated with malocclusion Normal masticatory muscle function o Tongue weakness: Hypoglossal nerve involvement Hypoplasia Asymmetry Limited range of motion Brainstem: Other o Respiratory abnormalities: 19%; Early death

Sleep disorders: Rapid eye movement sleep behavior disorder10 Motor disability, general: 88% o Poor coordination (83%) o Hypotonia: Congenital Poland syndrome: Some patients Malformations o Hands: Deformities o Legs: Hypoplasia
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Mbius & Congenital facial syndromes: Types

Sporadic o Associated with toxic exposure Attempted abortion using misoprostol: Odds ratio = 38 Cocaine MBS1 Chromosome 13q12.2-q13; Dominant o Clinical: See general features Congenital facial diplegia; Asymmetric Ophthalmoplegia, esp VI nerve Mental retardation Peripheral neuropathy Skeletal: Arthrogryposis; Orofacial malformations; Rib defects Muscle aplasia Respiratory failure: Central; Associated with tegmental brainstem calcification Hypogonadotropic hypogonadism o Pathology: Aplasia or hypoplasia of cranial nerves & nuclei

Hereditary congenital facial paresis 1 (HCPF1) Chromosome 3q21-q22; Dominant o Nosology: MBS2 o Clinical Facial weakness o Asymmetric o Unequal involvement of the 3 branches of the facial nerve No ophthalmoplegia o Pathology Facial nucleus: Reduced numbers of neurons Facial nerve: Small Rhombencephalon & corticospinal tracts: Normal

Hereditary congenital facial paresis 2 (HCPF2) Chromosome 10q21.3-q22.1; Dominant1 o Nosology: MBS3 o Genetics: Variable penetrance o Clinical Facial weakness: Unilateral or Bilateral Hearing loss: Congenital deafness, or Progressive hearing loss with age Ophthalmoplegia

Chromosome translocation Chromosome 1q22 o Genetics Mutation o Reciprocal translocation between chromosomes 1 & 11: t(1;11)(p22;p13) o Reciprocal translocation between chromosomes 1 & 2: t(1;2) (p22.3;q21.1) Mutation often on paternally derived chromosome o Clinical Facial diplegia Ptosis Other cranial nerves involved in some patients: III, VI, VIII Facial deformities: Anteverted nostrils, Malformed and low-set ears Developmental delay: Mild Poland syndrome: 1 patient Congenital heart disease o Atrial septal defects + o Similar genetic changes in cardiofacial syndrome

Carey-Fineman-Ziter syndrome Recessive

Facial Nerve Trauma & Tumors

Trauma o Petrous bone fracture o Surgery: Middle ear; Mastoidectomy; Parotid gland

Tumors & Masses6 o Tumor types Neuroma/Neurinoma/Schwannoma: Most common Meningioma Hemangioma: Produce defects even with small size Cholesteatoma Parotid gland Metastasis Carcinomatous meningitis Paget: Osteopetrosis o Tumor location Most common: Geniculate ganglion & Tympanic region Tumors along multiple segments of VII nerve common Rare: Middle cranial fossa o Signs Facial weakness: 82% Course: Weakness progressive o Usually > 1 year o Range: Over 2 to 120 months Hearing loss: Some patients; Conductive, sensorineural or mixed Tumor visible by otoscopy Other: Some tumors found incidentally o Diagnosis: MRI from brainstem to parotid o Treatment Surgery Residual or increased facial paresis common: More deficit after 1 year of symptoms Best prognosis after removal of hemangioma

Hemifacial Spasm

Onset
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Adults

Typically 5th or 6th decade Younger patients: Rule out brainstem disease, MS o Location: Orbicularis oculi muscle o More common in women Contraction pattern o Synchronous contraction of facial nerve innervated muscles o Paroxysmal o Involuntary o Duration: Up to 1 minute o May persist during sleep o Usually unilateral Weakness: Unusual Triggers: Aggravating factors Barker o Emotion o Fatigue Causes o Recovery from Bell's palsy Associated with synkinesis & contracture o Dolichoectatic brainstem artery Frequency: 30% Mechanism: Pressure on VII root entry zone Vessels: PICA; AICA; Vertebral o Brainstem disease o Idiopathic Course: Usually permanent without treatment Electrophysiology o Brief bursts of action potentials T2 image: Brainstem & Cerebellu spasm High frequency (150-400 Hz) Normal motor units o Variable rhythm & amplitude o Lateral spread response3 Stimulation: Facial nerve branch Response: In muscles not normally innervated by branch Related to cross transmission of facial nerve fibers o Location: Probably at site

of compression Treatment o Botulinum toxin o Microvascular decompression of VII nerve Morbidity & Mortality 5% o Anticonvulsants