Hemifacial SpasmHemifacial SpasmPresented By:Presented By:Kate BaldwinKate Baldwin

Liz ThayerLiz ThayerMay 15May 15thth, 2008, 2008

ComDisComDis 624624Motor Speech DisordersMotor Speech Disorders

What is Hemifacial Spasm?What is Hemifacial Spasm?

HFS is a hyperkinetic movement disorder HFS is a hyperkinetic movement disorder characterized by involuntary spasmscharacterized by involuntary spasms

HFS affects the muscles innervated by the HFS affects the muscles innervated by the facial nervefacial nerve

The spasms are uncontrollable and may be The spasms are uncontrollable and may be clonic (brief) or tonic (continuous) in clonic (brief) or tonic (continuous) in naturenature

What is the Cause of Hemifacial What is the Cause of Hemifacial Spasm?Spasm?

The most common cause is contact The most common cause is contact between a blood vessel and the root exit between a blood vessel and the root exit zone of the facial nerve zone of the facial nerve

An artery often causes compression on the An artery often causes compression on the facial nerve if it is dilated or hardened facial nerve if it is dilated or hardened

The arteries most commonly involved are The arteries most commonly involved are the AICA, PICA, and vertebral arterythe AICA, PICA, and vertebral artery

TuncTunc et al. (2008)et al. (2008)

Anatomical Layout of CN VII and Anatomical Layout of CN VII and Nearby ArteriesNearby Arteries

Causes Causes ctdctd..

A limited number of cases of HFS have A limited number of cases of HFS have been attributed to tumors, demyelinating been attributed to tumors, demyelinating disorders, or infections (Park et al.,2008) disorders, or infections (Park et al.,2008)

A rare study cited by A rare study cited by TuncTunc et al. (2008) et al. (2008) found a number of patients with HFS found a number of patients with HFS without a localizable lesion without a localizable lesion

Clinical Presentation of HFSClinical Presentation of HFS

A typical spasmA typical spasm……begins with involuntary twitching of the begins with involuntary twitching of the orbicularisorbicularis oculioculi

is initially clonic in nature and then a tonic phase takes is initially clonic in nature and then a tonic phase takes place, in which the ipsilateral eye is forced closedplace, in which the ipsilateral eye is forced closed

gradually increases in intensity and begins to affect other gradually increases in intensity and begins to affect other muscles on the same side of the facemuscles on the same side of the face

““Emotional stress, voluntary contraction of any facial muscles, aEmotional stress, voluntary contraction of any facial muscles, and fatigue nd fatigue make it worse. The severity of the spasm fluctuates but tends gemake it worse. The severity of the spasm fluctuates but tends generally to nerally to worsen with timeworsen with time”” ((SauvainSauvain et al., 2001)et al., 2001)

SauvainSauvain et al. (2001) Tan et al. (2002) et al. (2001) Tan et al. (2002)

Orbicularis Oculi

How do You Diagnose How do You Diagnose Hemifacial Spasm?Hemifacial Spasm?

Use of MRIUse of MRI

Neurological featuresNeurological features

Clinical featuresClinical features

Differential diagnosisDifferential diagnosis

TreatmentsTreatments

Current treatmentsCurrent treatments

BotulinumBotulinum Toxin injectionsToxin injections

Microvascular nerve Microvascular nerve decompressiondecompression

Oral medicationsOral medications

BotoxBotox

Causes temporary weakness of musclesCauses temporary weakness of muscles

Effects last approximately 3Effects last approximately 3--4 months 4 months need to need to continuously receive injectionscontinuously receive injections

Some argue that Some argue that BotoxBotox can be used over a long can be used over a long period of time without major side effectsperiod of time without major side effects

Site of injections should reflect the individualSite of injections should reflect the individual’’s s needs however there are common injection sitesneeds however there are common injection sites

Ward et al. (2006), Tan eWard et al. (2006), Tan et al. (2002) t al. (2002)

Microvascular Nerve Microvascular Nerve DecompressionDecompression

Can provide a permanent solutionCan provide a permanent solution

Involves placing a barrier in between Involves placing a barrier in between CN VII and the arteryCN VII and the artery

Success rate of 85%Success rate of 85%--97%97%

FreiFrei et al. (2006), Park et al. (2008), et al. (2006), Park et al. (2008), HeuserHeuser et al. (2007)et al. (2007)

Microvascular Nerve Microvascular Nerve DecompressionDecompression

SauvainSauvain et al. (2001)et al. (2001)

Case StudyCase StudyK.T.K.T.

5050--yearyear--old right handed old right handed malemale

Native Cantonese Native Cantonese speakerspeaker

K.T. Background InformationK.T. Background Information

SelfSelf--referred to Center for Language, Speech, referred to Center for Language, Speech, and Hearing in March of 2006 for accent and Hearing in March of 2006 for accent reductionreduction

LeftLeft--sided sided hemifacialhemifacial spasm and paralysis spasm and paralysis observedobserved

Paralysis secondary to Paralysis secondary to BotoxBotox injections the injections the client was receiving to treat the client was receiving to treat the hemifacialhemifacialspasm = Mild Flaccid spasm = Mild Flaccid DysarthriaDysarthria!!

Microvascular nerve decompression in Microvascular nerve decompression in October of 2007October of 2007

Measures of AssessmentMeasures of Assessment

2 months pre2 months pre--op., 1 week postop., 1 week post--op., 1 month op., 1 month postpost--op, 5 months postop, 5 months post--op. op.

Oral PeripheralOral Peripheral--Neurological Motor Speech Neurological Motor Speech Examination (Mayo Clinic Procedures)Examination (Mayo Clinic Procedures)

Photo Articulation Test, 3rd EditionPhoto Articulation Test, 3rd Edition ((PATPAT--3)3)

Assessment of Intelligibility of Assessment of Intelligibility of DysarthricDysarthricSpeechSpeech ((AIDSAIDS))

Oral PeripheralOral Peripheral--Neurological Neurological Motor Speech ExaminationMotor Speech Examination

Facial symmetry improvementFacial symmetry improvement

Decreased MPT Decreased MPT musculoskeletal tensionmusculoskeletal tension

Most salient improvements:Most salient improvements:

Smile on commandSmile on command

Lip roundingLip rounding

VIDEOVIDEO

Assessments of SpeechAssessments of Speech

PATPAT--33 AccuracyAccuracy

AIDS AIDS IntelligibilityIntelligibility

AccuracyAccuracy vs. vs. IntelligibilityIntelligibility??

Photo Articulation Test, 3rd Photo Articulation Test, 3rd EditionEdition ((PATPAT--3)3)

Measured Measured accuracyaccuracy of Cof C’’s and Vs and V’’s in wordss in words

Frequency of errors decreased over timeFrequency of errors decreased over time

ALL errors made can be attributed to ESLALL errors made can be attributed to ESL

For example:For example:

[u] for [[u] for [ʊʊ], distortion of [l], and devoicing ], distortion of [l], and devoicing of [z]of [z]

TreatmentTreatment vs. vs. BotoxBotox/Surgery??/Surgery??

Assessment of Intelligibility of Assessment of Intelligibility of DysarthricDysarthric SpeechSpeech ((AIDSAIDS))

Measured Measured intelligibilityintelligibility in words in words and sentencesand sentences

Improvement over time Improvement over time 70%70% 100% (WOW!)100% (WOW!)

Sentence level intelligibility aided Sentence level intelligibility aided by contextby context

Treatment vs. Treatment vs. BotoxBotox/Surgery/Surgery????

DiscussionDiscussion

Why is K.T a unique case?Why is K.T a unique case?

ESL vs. Mild Flaccid ESL vs. Mild Flaccid DysarthriaDysarthria

Accuracy vs. IntelligibilityAccuracy vs. Intelligibility

Speech Speech TxTx vs. vs. BotoxBotox vs. Surgery vs. Surgery

Prognosis Prognosis Good!Good!

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References References CtdCtd..Ito, M., Hasegawa, M., Ito, M., Hasegawa, M., HoshidaHoshida, S., Miwa, T., Furukawa, M. (2004). , S., Miwa, T., Furukawa, M. (2004). Successful treatment of Successful treatment of hemifacialhemifacial spasm with selective facial nerve block using doxorubicin (spasm with selective facial nerve block using doxorubicin (adriamycinadriamycin) under local ) under local anesthesia.anesthesia. ActaActa OtoOto--LaryngologicaLaryngologica, 124,, 124, 217217--220.220.KurokawaKurokawa, Y., Maeda, Y., , Y., Maeda, Y., ToyookaToyooka, T., , T., InabaInaba, K. (2004). , K. (2004). Microvascular decompression for Microvascular decompression for hemifacialhemifacial spasm caused by the vertebral artery: A simple and effective trspasm caused by the vertebral artery: A simple and effective transposition method ansposition method using surgical glue.using surgical glue. Surgical Neurology, 61,Surgical Neurology, 61, 398398--404.404.Palacios, E., Palacios, E., ValvassoriValvassori, G. (1999). , G. (1999). Vascular loop and Vascular loop and hemifacialhemifacial spasm.spasm. ENT: Ear, Nose & ENT: Ear, Nose & Throat Journal, 78,Throat Journal, 78, 470470--471.471.Pang, A., Pang, A., O'DayO'Day, J. (2006). , J. (2006). Use of highUse of high--dose dose botulinumbotulinum a toxin in benign essential a toxin in benign essential blepharospasmblepharospasm: Is too high too much?: Is too high too much? Clinical & Experimental Ophthalmology, 34,Clinical & Experimental Ophthalmology, 34, 441441--444.444.Park, J.S., Kong, D.S., Lee, J.A., Park, K. (2008). Hemifacial sPark, J.S., Kong, D.S., Lee, J.A., Park, K. (2008). Hemifacial spasm: Neurovascular compressive pasm: Neurovascular compressive patterns and surgical significance. patterns and surgical significance. ActaActa NeurochirurgicaNeurochirurgica. . 150,150, 235235--241.241.RahmanRahman, E., , E., TrobeTrobe, J., , J., GebarskiGebarski, S. (2002). , S. (2002). Hemifacial spasm caused by vertebral artery Hemifacial spasm caused by vertebral artery dolichoectasiadolichoectasia.. American Journal of Ophthalmology, 133,American Journal of Ophthalmology, 133, 854854--857.857.Tan, E.K, Chan, LL. (2006). Young onset Tan, E.K, Chan, LL. (2006). Young onset hemifacialhemifacial spasm. spasm. ActaActa NeurologicaNeurologica ScandinavicaScandinavica, , 114, 114, 5959--62.62.Tan, N.C, Chan, L.L., Tan E.K. (2002). Hemifacial spasm and invoTan, N.C, Chan, L.L., Tan E.K. (2002). Hemifacial spasm and involuntary facial movements. luntary facial movements. Q Q J MedJ Med, , 9595, 493, 493--500.500.TuncTunc, T., , T., CavdarCavdar, L., , L., KaradagKaradag, Y., , Y., OkuyucaOkuyuca, E., , E., CoskinCoskin, O., , O., InanInan, L. (2008). , L. (2008). Differences in Differences in improvement between patients with idiopathic versus neurovasculaimprovement between patients with idiopathic versus neurovascular r hemifacialhemifacial spasm after spasm after botulinumbotulinum toxin treatment. toxin treatment. Journal of Clinical Neuroscience, 15, Journal of Clinical Neuroscience, 15, 253253--256.256.SauvainSauvain, M.O., , M.O., MagistrisMagistris, M.R., de , M.R., de TriboletTribolet, N. (2001). , N. (2001). Microvascular decompression of the Microvascular decompression of the facial nerve.facial nerve. Operative Techniques in Neurosurgery,4Operative Techniques in Neurosurgery,4, 127, 127--136.136.SindouSindou et. al. (2005). Microvascular decompression for primary et. al. (2005). Microvascular decompression for primary hemifacialhemifacial spasm: Importance spasm: Importance of of intraoperativeintraoperative neurophysiologicalneurophysiological monitoring. monitoring. ActaActa neurochirurgicaneurochirurgica, 147,, 147, 1019 1019 --1026.1026.Ward, A., Ward, A., MolenaersMolenaers, G., , G., ColosimoColosimo, C., , C., BerardelliBerardelli, A. (2006). , A. (2006). Clinical value of Clinical value of botulinumbotulinum toxin toxin in neurological indications.in neurological indications. European Journal of Neurology, 13,European Journal of Neurology, 13, 2020--26.26.

Supplementary SlidesSupplementary Slides

Motor and Sensory Innervation of CN Motor and Sensory Innervation of CN VIIVII

Brachial MotorBrachial Motor: Muscles of : Muscles of facial expression & facial expression & stapediusstapedius

Visceral MotorVisceral Motor: Innervation : Innervation of muscles for tears and salivaof muscles for tears and saliva

Special SensorySpecial Sensory: Taste : Taste anterior 2/3 tongue; hard and anterior 2/3 tongue; hard and soft palatesoft palate

General SensoryGeneral Sensory: : ConchaConcha of of auricle and behind earauricle and behind ear

What are the Treatments for What are the Treatments for Hemifacial Spasm?Hemifacial Spasm?

Archaic treatmentsArchaic treatments

Irrigation of the facial nerveIrrigation of the facial nerve

Injection of toxic compoundsInjection of toxic compounds

Removal of the Removal of the orbicularisorbicularisoculioculi

EbyEby et al. (2001), Tan et al. (2002)et al. (2001), Tan et al. (2002)

Common Common BotoxBotox Injection SitesInjection Sites

Pang, A., O'Day, J. (2006)

Assessment of Intelligibility of Assessment of Intelligibility of DysarthricDysarthric Speech (AIDS) Speech (AIDS) CtdCtd..

2 month 2 month prepre--opop

1 week 1 week postpost--opop

1 month 1 month postpost--opop

5 month 5 month postpost--opop

Word Level Word Level IntelligibilityIntelligibility

67%67% 73%73% 76%76% 71%*71%*

Sentence Sentence Level Level

IntelligibilityIntelligibility

70%70% 95.5%95.5% 99.5%99.5% 100%100%