Department Of Oto-rhino-Laryngology Of Isfahan Medical Science

Bell’s palsy ( criteria )

paralysis or paresis of all muscle groups of one side.

Sudden onset. Absence of signs of CNS disease. Absence of signs of ear or CPA disease.

Bell’s palsy (epidemiology)

Incidence; 20-30/100,000 ( >65y: 59/100,000 & <13y: 13/100,000 )

M=F (<20y in women & > 40y in men)

70% complete paralysis

0.3% bilateral

9% Hx of previous paralysis

8% family history +

Bell’s palsy…

Etiology: Viral (HSV1), microcirculatory failure, ischemic neuropathy, autoimmune.

Histopathology: diffuse demyelination of intra temporal course of the facial nerve with the most sever findings in the labyrinthine segment & meatal foramen.

CNS changes: results are contradictory; * Hyperacusis in 30% (dysfunction of stapedius muscle) * ABR abnormality in few patients (↑ wave I to V interval &

interaural difference for wave V), bilaterally. * CSF changes in 10%. * MRI changes.

Bell’s palsy (test of facial nerve function)

Topodiagnostic testing; Schimer’s test, stapedial reflex, taste testing or submandibular salivary flow test.

Electrodiagnostic tests: * NET; >3.5 mA difference, >1.25 mA in upper division & >2

mA in lower division is abnormal. * MST; if is NL → 88% , if ↓movement →27% complete

recovery, if no response →always incomplete recovery.

* ENOG; excellent recovrry if dose not reach 90% degeneration, although ½ of patients with >90% also have excellent outcome.

Bell’s palsy (test of facial nerve function)…

* EMG; fibrillation after 10-14 days → 81% incomplete recovery, loss of

Voluntarily potentials within 3-4 days → poor prognosis, Voluntarily

potentials after 7 days → no complete degeneration.

* NCV; strongly correlates with ENOG in first 2 weeks, if is between 37 &

58 m/sec → good outcome, if is between 20 & 30 m/sec → 50%

residual & synkinesia, if < 10 m/sec → poor outcome.

* Electromagnetic stimulation; root entry zone of the nerve is site of

excitation, predictive value in the first 4 days.

Bell’s palsy ( prognosis )

80-90% recover completely.

The most important prognostic factor is severity of paralysis (95-100% of patients without complete paralysis recover completely).

Other factors with poor outcome are hyperacusis, ↓tearing, >60y, HTN, DM, sever aural & anterior facial or radicular pain.

Synkinesia & contracture correlate with paralysis & onset of recovery interval.

Bell’s palsy ( management )

Corton is the most common management, making the disease less sever or ↑ chance of complete recovery. no beneficial effect in children.

Antivaral agents + corton increase good outcome from 80% to 98%. It should be considered in addition to not as a substitute for steroid.

Surgical decompression; no benefit of transmastoid decompression, middle fossa decompression in >90% degeneration in ENOG and no voluntary potentials in EMG with in 2 weeks of onset of paralysis significantly improves the outcome….

Spontaneous facial paralysis in children

Is uncommon, female predominance.

High rate of spontaneous recovery related to high frequency of incomplete paralysis (adult & children with similar electrical testing had similar outcome).

Corticosteroids had no impact on recovery.

Recurrent paralysis

9.3 – 10.9 % of bell’s palsy, slight female predominance, equally on ipsi & contralateral sides, interval between attacks usually >1Y, ipsilateral palsy associated with younger age of onset.

Most found no prognostic difference between primary & subsequent attacks & whether the 2nd occurred on ipsi or contralateral side.

Careful investigation specially in ipsilateral recurrent palsy

(20% association with tumors) .

Bell’s palsy in pregnancy

3.3 times more frequent in pregnant women, most commonly in 3rd trimester or immediately in postpartum.

Preeclampsia is 6 times more common in bell’s palsy, not correlates with preterm labor, LBW, congenital abnormality.

Prognosis ?! (no difference Or decidedly worse).

Tx is prednisone (minimal risk to the developing fetus).

Ramsay hunt syndrome

2nd most common cause of facial paralysis. associated with VZV, vesicles on the pinna, retroauricular area , face & mouth.

In most patients eruption occurs with paralysis, in 25% precedes the paralysis (higher recovery rate) & in 10% after paralysis or a rise in Ab without vesicle that called rumsay hunt sine herpete.

VZV reactivated in ↓ cell mediated immunity (carcinoma, trauma, chemoradiotherapy & elderly).

Ramsay hunt syndrome…

It causes more sever symptoms (↑ CNS symptom, hyperacusis, HL & pain), higher rate of complete nerve degeneration (full recovery only in 16-22%, in 10% 0f patients with complete paralysis & 66% of patients with incomplete loss)

Management; corton; ↓ postherpetic neuralgia, acute pain & vertigo if used early,

usefulness in facial paralysis is contraversial. acyclovir; is recommended to treat of VZV facial paralysis, it

lessens pain & promotes resolution of vesicles. Not toxic for non infected cells.

Bilateral facial paralysis

0.3-2% , higher incidence of systemic disease: Guillain Barre’ syn, multiple idiopathic cranial neuropathy, brain stem encephalitis, BIH, syphilis, leukemia, sarcoidosis, lyme disease, bacterial meningitis, CPA tumors, influenza, EBV, other viruses & DM.

Association with other systemic & neurologic signs suggests the Dx.

W/U includes neurologic examination, LP (cytology, culture, chemistry & VDRL),MRI.

Recovery in bilateral bell’s palsy is similar to that unilateral palsy, although one side may recover before the other.

Bilateral facial paralysis…

Guillain barre’ syn; progressive ascending motor paralysis, CN 9>10>7 involvement, ↑ CSF pro & NL cell count.

HIV; in early stage due to acute inflammatory demyelinating polyneuropathy & in late stage due to chronic peripheral polyneuropathy or VZV or lymphoma. Prognosis in idiopathic paralysis is similar to general population.

kawasaki disease; infantile acute febrile mucocutaneous lymph node synd, cardiac (coronary aneurysm) & neurologic (aseptic meningitis & irritability) sys involvement.

facial paralysis has been reported, resulted from ischemia, resolved in 1-12 W. Tx is high dose Asprin.

Bilateral facial paralysis…

Sarcoidosis; CN involvement in 5% (facial nerve is the most), 50% of patients with heerfordt’s dis has facial paralysis due to direct invasion & may be bilateral. Dx confirm by ↑ ACE level. Tx is corton.

Benign intracranial hypertension; headache, visual disturbance, may be associated with CN palsy (VI is the most), unilateral & bilateral facial palsy has been reported.

Metabolic disorders; bell’s palsy is 4.5 times more likely in diabetics & abnormal GLU tolerance curves is significantly more in bell’s palsy (give corton with caution).

reversible facial palsy in hypovitaminosis A has been reported.

Lyme disease

3 stages; 1st stage: flue like syndrome, regional LAP, general malaise. 2nd stage: headache, neck stiffness, spinal pain, meningitis, CN & other peripheral neuropathies. 3rd stage: chronic arthritis, neurologic deficits, recurrent

meningitis, mental disorder.

Facial paralysis in 4.5%, uni or bilateral, may precede serologic evidence. Etiology of ½ of facial palsy in children. Usually resolve completely.

T2 weighted MRI of brain may be the best way for follow of the effect of AB therapy in children.

Tx is ceftriaxon 1gr/IV/BD for 14 days, improvement is not expected for several mouth and seldom complete.

Congenital Facial Paralysis incidence is 0.23% of live births.

Differentiated from birth trauma (78%) by other signs of injury (swelling, ecchymosis, or hemotympanum). Abnormalities of other CNs or ABR (↑ I to III or I to V interval) → paralysis is congenital. Supranuclear palsy secondary to ICH.

DDx of congenital palsy; * unilateral lower lip palsy: absence of depressor labii inferioris

activity because of brainstem lesion. * Möbius' syndrome: isolated unilateral facial paralysis or absence

of facial and abducens (may be bilateral), glossopharyngeal, vagus, hypoglossal, and other extraocular motor nerves. associated with significant event in utero & prostaglandin E1 analog misoprostol. Pectoralis major absence in Poland-Möbius' syndrome. * teratogens: thalidomide & rubella.

Traumatic Facial Paralysis

The most common causes of infratemporal facial nerve injury. Temporal bone fracture: labyrinthine segment and geniculate

ganglion are most susceptible to injury because of small size, lack of fibrous supporting tissue, and traction between the greater petrosal nerve and the geniculate ganglion.

* Management: onset of paralysis or age of paralysis ?… In immediate paralysis delaying surgery up to 3 weeks allows

resolution of edema and hematoma and makes the surgical field more discernible.

In longstanding paralysis, explore the nerve if no clinical or EMG signs of recovery is seen after 12 mouths, unless CT shows gross disruption of the fallopian canal.

Traumatic Facial Paralysis…

Penetrating injuries: accompanied by severe injuries, Evaluation includes CTscan, carotid arteriography, and facial nerve electrical testing. extratemporal facial nerve blast injuries may not cause transection of the nerve → conservative management even if the nerve is severely hemorrhagic and contused.

Iatrogenic facial paralysis: * concern intraoperatively → identify the nerve by

landmarks distant from the site of injury. Site is decompressed, If loss of nerve tissue is serious, an onlay or interpositional facial graft can be used.

* If paralysis is unexpected → Tight dressings and packing should be released immediately. If the face has not recovered after local anesthetic effects dissipated, urgent reexploration and decompression should be performed.

Melkersson-rosenthal syndrome

Triad: recurrent orofacial nonpintting edema (defining feature), recurrent facial palsy (in 50-90%, may be bilateral) & lingual palicata (fissured tongue) in ½ of patients. Complete triad in ¼. Site of paralysis corresponds to area of facial swelling.

Edema not explained by infection, malignancy or connective

tissue disorder. Permanent deformity & chronic painful fissures of tongue.

In Bx: noncaseating epithelioid cell granulomas → variant of

sacoidosis, some suggest vasomotor disturbance & allergy.

Cessation of recurrent paralysis after decompression has been reported.

Hyperkinetic disorder

Hemifacial Spasm: involuntary twitching and contraction of one side of the face. usually starts around the mouth and gradually involves the entire face . usually painless, unless involves the trigeminal nerve. combination of demyelination and arterial compression is required for generation of the symptoms (nerve becomes hyperactive after long-term irritation of the demyelinated portion).

* Management: partial resection of three main branches or resection of involved branches determined by EMG, separation of the aberrant vessels through a posterior fossa craniotomy, decompression of the nerve.

Hyperkinetic disorder…

Blepharospasm: idiopathic progressive involuntary spasm of the orbicularis oculi and upper face (corrugator and procerus muscles). Extension to involvement of the lower face is not uncommon, is central in origin.

* Management: selective destruction of the peripheral nerve

branches innervating the orbicularis oculi, Botulinum A toxin (duration is 11 to 14 weeks,associated with lid ptosis, facial weakness, corneal exposure, and diplopia), Periorbital myectomy.

Tanks for your attention