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Aaem Case Report #21: Hemifacial Spasm: Preoperative Diagnosis and Intraoperative Management

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Aaem Case Report #21: Hemifacial Spasm: Preoperative Diagnosis and Intraoperative Management ~~ AAEM CASE REPORT #21 A 75-year-old man developed progressive involuntary hemifacial spasm. Electrophysiologic evidence of abnormal cross-transmission between neu- rons of the facial nerve was demonstrated. Electrodiagnostic studies were used to confirm the diagnosis preoperatively and determine the adequacy of vascular decompression of the facial nerve intraoperatively. Key words: hemifacial spasm nerve conduction studies, hemifacial spasm craniectomy MUSCLE & NERVE 14~213-218 1991 AAEM CASE REPORT #21: HEMIFACIAL SPASM: PREOPERATIVE DIAGNOSIS AND INTRAOPERATIVE MANAGEMENT C. MICHEL HARPER, Jr., MD Hemifacial spasm is a chronic and often progres- ments were worse with emotional stress and per- sive disorder characterized by unilateral irregular sisted during sleep. He denied any sensory distur- clonic and tonic contraction of one or more mus- bance or weakness of the face. There was a long- cles of facial expression. The condition may result standing history of partial bilateral hearing loss. from chronic compression of, or injury to, the fa- Previous treatment with phenytoin and carbam- cial The majority of patients with dis- azepine failed to improve the facial movements. abling “idiopathic” hemifacial spasm respond to surgery designed to detect and relieve vascular Physical Examination. Abnormalities were limited compression of the facial nerve at its exit from the to frequent irregular clonic contractions and inter- brainstem.“, 13,16,20,26 Electrodiagnostic studies mittent tonic spasms of the orbicularis oculi and help distinguish hemifacial spasm from other in- oris, frontalis, buccal, and mentalis muscles. There voluntary movements of the face and may help was no objective weakness of facial muscles or ab- determine when the facial nerve has been ade- normality of facial sensation. Corneal reflexes quately decompressed intraoperatively. were normal with no obvious synkinesis noted. CASE REPORT Laboratory Tests. Computerized tomography of Clinical History. A 75-year-old man presented for the head with contrast showed ectatic dilatation of evaluation of a two-year history of progressive the upper portion of the left vertebral artery. The unilateral twitching of the left face. The involun- intracranial contents were otherwise normal. tary facial movements began as mild intermittent Transfemoral cerebral angiography confirmed the brief contractions near the corner of the mouth, marked ectasia and tortuousity of the left verte- gradually progressing to a mixture of brief irregu- bral artery causing it to swing laterally into the lar clonic and more prolonged tonic contractions cerebellopontine angle. of all the muscles of facial expression. The move- ELECTRODIAGNOSTIC EVALUATION Methods. Facial nerve conduction studies and blink reflexes were performed using standard From the Department of Neurology, Division of Clinical Neurophysiology, Mayo Clinic. Rochester, Minnesota 55905 techniques with percutaneous stimulation and sur- Address reprint requests to AAEM, 21 Second Street S.W.. Suite 306, face recording electrodes. The facial M wave was Rochester, MN 55902. recorded over the ipsilateral nasalis muscle refer- Accepted for publication April 6, 1990 enced to an electrode over the contralateral nasalis during stimulation with the cathode just inferior CCC 0148- 639X/91/030213-06 $04.00 0 1991 C. Michel Harper, MD. Published by John Wiley & Sons, Inc. to the stylomastoid foramen. Blink reflexes were AAEM Case Report #21: Hemifacial Spasm MUSCLE & NERVE March 1991 213 performed with stimulation of the supraorbital low stimulus intensities. Supramaximal stimuli nerve recording over the orbicularis oculi muscles may cause the recording to be contaminated by bilaterally. Simultaneous recordings were made volume-conducted potentials generated by the over the ipsilateral mentalis muscle for the detec- masseter or facial muscles. Some patients with tion of synkinesis. The “lateral spread response” mild hemifacial spasm will have a lateral spread described by Nielsen2”22 and was re- response with either zygomatic or mandibular corded preoperatively with surface electrodes over stimulation, but not both. Therefore, if a lateral the orbicularis oculi and mentalis muscles during spread response cannot be elicited with one tech- ipsilateral percutaneous stimulation of the man- nique, the other should always be performed. dibular branch of the facial nerve 5 cm proximal Needle electromyography was performed with a to the mentalis recording electrode (cathode prox- standard concentric needle electrode. imal, see Fig. 1). Recordings of spontaneous EMG activity in fa- Although not done in this case, the lateral cial muscles, the lateral spread response, and ipsi- spread response can also be recorded from the lateral brainstem auditory evoked potentials were mentalis muscle with stimulation of the zygomatic performed during suboccipital craniectomy and branch of the facial nerve 5 cm proximal to the vascular decompression of the facial nerve. Pairs orbicularis oculi electrode (Fig. 1). The normal of fine nichrome wire electrodes were placed in range in latency of the lateral spread response the orbicularis oculi and oris muscles. Spontane- with either technique is 7 to 11 ms. A well-defined ous EMG activity was monitored continuously at response can usually be obtained with relatively standard gains, sweep speeds, and filter settings for needle electromyography. The same intramus- cular electrodes in the orbicularis oculi muscle were used to record the lateral spread response. The mandibular branch of the facial nerve was stimulated with 2 platinum needle electrodes placed after induction of anesthesia. Neuromuscu- lar blocking agents were excluded from the anes- thetic regimen. PREOPERATIVE STUDIES Nerve Conduction Studies. The results of facial nerve conduction studies and blink reflexes are il- lustrated in Table l. Facial nerve conduction stud- ies were normal. The only abnormality of the blink reflex was a synkinetic response of the left mentalis muscle with ipsilateral stimulation of the supraorbital nerve (Fig. 2). A lateral spread re- sponse with an approximate latency of 10 ms was recorded on the left (Fig. 3). No synkinesis or lat- eral spread response could be recorded on the right. Record: Record 2 Left orb. oculi FIGURE 1. Technique for recording the lateral spread response from the orbicularis oculi muscle (Record 1) with stimulation of Left mentalis the mandibular branch of the facial nerve (Stim 1) or from the mentalis muscle (Record 2) with stimulation of the zygomatic !jooIJ” branch of the facial nerve (Stim 2). The mandibular branch is 5J ms stimulated with the cathode 5 cm proximal to the mentalis elec- trode. The zygomatic branch is stimulated with the cathode 5 cm FIGURE 2. Blink reflex demonstrating synkinesis of the orbicu- proximal to the orbicularis electrode (Gl)along the zygomatic laris oculi and mentalis muscles to stimulation of the left-supraor- arch. bital nerve. 214 AAEM Case Report #21: Hernifacial Spasm MUSCLE & NERVE March 1991 Table 1. Preoperative nerve conduction studies. Nerve Record Amplitudes (mV) Distal latency (ms) Right facial, motor Nasalis 1.6 2.5 Left facial, motor Nasalis 1.9 3.0 lpsilateral (ms) Contralateral (ms) R1 R2 R2 Right trigeminal, blink Orbicularis oculi 11.3 27 31 Left trigeminal, blink Orbicularis oculi 11.3 29 30 Needle Examination. The left and right orbicu- compressing the inferior aspect of the facial nerve laris oculi and oris muscles and the left masseter at its exit from the brainstem. When the vessel was and mentalis muscles were examined. Abnormali- removed from the nerve, the lateral spread re- ties were confined to irregular brief high-fre- sponse disappeared only to reappear when the quency bursts of motor unit potentials recorded in vessel was allowed to fall back in contact with the the left orbicularis oculi and oris muscles (Fig. 4). nerve (Fig. 5). The vessel was then permanently No fibrillation potentials or abnormalities of mo- displaced from the nerve with Gelfoam and mus- tor unit potential amplitude, duration, recruit- cle. The lateral spread response could not be de- ment, or morphology were noted. tected during the remainder of the procedure. Manipulation of the facial nerve intraopera- Interpretation. The electrodiagnostic studies tively produced high-frequency “neurotonic” dis- showed synkinesis of the left blink reflex, a lateral charges in facial muscles which provided the sur- spread response from one branch of the facial geon immediate warning of potential iatrogenic nerve to another, and high-frequency motor unit injury to the facial nerve (Fig. 6). Brainstem audi- potential discharges in facial muscles. In the con- tory evoked potentials remained stable during the text of involuntary facial movements, this constel- procedure indicating that the integrity of the co- lation of findings is diagnostic of hemifacial chlear nerve was being preserved. All involuntary spasm. facial movements were absent postoperatively with no facial weakness. The patient has remained CLINICAL COURSE symptom-free over 3 months of follow-up. The patient underwet a left suboccipital craniec- tomy with continuous intraoperative monitoring DISCUSSION of spontaneous EMG activity in facial muscles and Hemifacial s asm was initially described by Gow- brainstem auditory evoked potentials. The lateral ers in 1884.p” It typically begins in the fifth or spread response of the left orbicularis oculi mus- sixth decade as mild unilateral irregular involun- cle to stimulation
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