Principales aspectos neurofisiológicos, clínicos, y genéticos, en una recién nacida con Síndrome de Cayler / Clinical, genetic, and neurophysiological main features in a newborn girl with Cayler Syndrome

En los neonatos, la hipoplasia o agenesia congénita del músculo depresor del ángulo de la boca (depresor anguli oris), es la causa de la asimetría facial durante el llanto. Los pacientes con esta anomalía, presentan caída de la comisura labial del lado intacto a diferencia de la parálisis facial de origen traumático o congénito, y su diagnóstico se obtiene fácilmente, mediante el examen físico. Esta anomalía está asociada con otras de origen congénito, sobre todo los defectos cardíacos, por lo que se nombra síndrome cardiofacial de Cayler. Se presenta una niña, recién nacida de 56 horas, atendida en el servicio de Neonatología del Hospital Pediátrico "Octavio de la Concepción de la Pedraja" de Holguín, Cuba. La paciente egresa con diagnóstico de síndrome de Cayler, al presentar asimetría facial durante el llanto, y tetralogía de Fallot. Se realizan estudios neurofisiológicos como: electroneurografía del nervio facial y electromiograma de los músculos depresores del labio.

Asymmetrical Crying Facies Syndrome, is caused by congenital hypolapsia or anguli oris agenesis. Patients usually present drop of the intact corner of their mouths while crying. Though it is easily diagnosed, it must be differentiated from traumatic or congenital facial palsies. This condition has been associated with several other anomalies, especially with heart issues. That´s why it is called Cayler Cardiofacial Syndrome. We have reported a case of a 56 hours newborn girl. She shows facial asymmetry as a valuable sign for suspecting other congenital anomalies. Clinical evaluation, including facial nerve electroneurography and electromyography, were performed.

Facial nerve decompression in treatment of patients with early peripheral traumatic facial paralysis / 局解手术学杂志

Objective To explore the effect of facial nerve decompression via mastoid-epitypanum approach on the treatment of early peripheral traumatic facial paralysis caused by temporal bone fracture.Methods The data of 21 patients with early peripheral traumatic facial paralysis caused by temporal bone fracture in our hospital from October 2011 to June 2016.The facial nerve electrogram and the blink reflex of the injured facial nerve of 21 patients who treated facial nerve decompression via mastoid-epitypanum approach were compared before and after operation.The degree of facial nerve function recovery was evluated by H-B grading method.Results The facial nerve function of all patients had improved in different degrees,85.7% patients recovered to Ⅰ~Ⅱ level.Compared with those before operation,the latency,amplitude and latent period of blink reflex of the ipsilateral facial electroneurography were significantly improved(P<0.05).Conclusion The facial nerve decompression has good effect in the treatment of early peripheral traumatic facial paralysis.

Clinical Efficacy of Electroneurography in Acute Facial Paralysis

The estimated incidence of acute facial paralysis is approximately 30 patients per 100000 populations annually. Facial paralysis is an extremely frightening situation and gives extreme stress to patients because obvious disfiguring face may cause significant functional, aesthetic, and psychological disturbances. For stressful patients with acute facial paralysis, it is very important for clinicians to answer the questions like whether or not their facial function will return to normal, how much of their facial function will be recovered, and how long this is going to take. It is also important for clinicians to treat the psychological aspects by adequately explaining the prognosis, in addition to providing the appropriate medical treatment. For decades, clinicians have used various electrophysiologic tests, including the nerve excitability test, the maximal stimulation test, electroneurography, and electromyography. In particular, electroneurography is the only objective measure that is useful in early stage of acute facial paralysis. In this review article, we first discuss the pathophysiology of injured peripheral nerve. And then, we describe about various electrophysiologic tests and discuss the electroneurography extensively.

Clinical Study of Du’s Manipulative Acupuncture with Gold Needles for Treatment of Intractable Facial Paralysis / 上海针灸杂志

Objective To investigate the clinical efficacy of Du’s manipulative acupuncture with gold needles in treating intractable facial paralysis.Methods Two hundred and ten patients with intractable facial paralysis were randomly allocated to treatment and control groups, 105 cases each. The same acupoints were selected in the two groups. The treatment group received Du’s manipulative acupuncture with gold needles and the control group, conventional acupuncture. Facial nerve electroneurography (ENoG) was performed in the two groups before and after treatment to evaluate the therapeutic effects.Results There were statistically significant pre-/post-treatment differences in musculus frontalis and orbicularis oris ENoG amplitude values on the affected side in the two groups (P<0.05). There were statistically significant post-treatment differences in musculus frontalis and orbicularis oris ENoG amplitude values on the affected side between the treatment and control groups (P<0.05).Conclusion Du’s manipulative acupuncture with gold needles is an effective way to treat intractable facial paralysis.

Study on prediction of involvement in facial nerve in the patients with parotid tumors by using facial electroneurography / 北京大学学报(医学版)

Objective:To investigate the possibility of predicting facial nerve involvement in the pa-tients with parotid tumors using facial electroneurography (ENoG).Methods:In the study, 53 patients with primary parotid tumors were included in the study , 28 were benign tumors and 25 were malignant . There was no significant difference of tumor locations and sizes between the two groups . House-Brackmann facial nerve function evaluation was gradeⅠin all the patients who received examination of fa-cial electroneurography , including stimulation strength , amplitude , and latent time bilaterally .The facial electroneurography results in the affected side were compared with the results of contralateral normal side , intraoperative appearance and postoperative histopathological diagnosis .The facial electroneurography results were analyzed by Wilcoxon signed rank test and receiver operator characteristic ( ROC) curve. Results:During the facial electroneurography examination , the mean stimulation strength in the patients with benign parotid tumor was 20.0 mA.There was significant decrease in the amplitude at the affected side compared with the normal side upon posterior auricular stimulation ( P0.05).However, in the patients with malignant parotid tumor , the mean stimulation strength was higher at 24.5 mA.There was signifi-cant decrease in the amplitude or even no response at the affected side compared to the normal side upon posterior auricular stimulation ( P<0 .05 ) .No response was detected in the multiple branches of facial nerve of affected sides in 36 .0%patients upon posterior auricular stimulation .The amplitude of branchesⅢand Ⅳwas significantly lower at the affected side than that at the normal side upon anterior auricular stimulation (P<0.05).The area under the ROC curve for ENoG was 0.884.Conclusion: When the facial nerve was involved by the parotid tumors , the stimulation strength in the electroneurography was larger .There was significant difference in the amplitude and the latent time of the facial nerve between the affected side and the normal side upon the posterior auricular stimulation .The rate of absence of reac-tion wave was higher in the patients with malignant tumors .It was feasible to predict the facial nerve in-volvement by ENoG for the parotid gland tumor patients without clinical appearances of facial paralysis .

Relationship among Characteristic of Blink Reflex, Facial Nerve Electroneurography and Functional Lesion at the Early Stage of Bell's Palsy / 中国康复理论与实践

@#Objective To investigate the relationship among blink reflex, facial nerve electroneurography and functional lesion at the early stage of Bell's palsy. Methods Blink reflex and electroneurography of facial nerve were performed in 25 patients with early Bell's palsy.They were divided into mild to moderate damage and severe damage according to the result. They were evaluated by House-Blackmann (H-B) scale and were identified: grade I, normal; grade II-III, mild-moderate facial paralysis; grade IV-VI, severe facial paralysis. Results The positive rate of blink reflex was 100%, with the proportion of mild- moderate damage was 28% and severe damage was 72%. H-B scale evaluation showed the percentage of mild-moderate facial paralysis was 44%, severe facial paralysis was 56%, which was consistent with blink reflex (P>0.05). The positive rate of electroneurography was 52%, with the proportion of mild- moderate damage was 44% and severe damage was 8%, which was inconsistent with H-B scale evaluation (P<0.05). Conclusion Blink reflex is preferable to electroneurography in evaluating facial paralysis degree at the early stage of Bell's palsy.

Diagnostic Value of Facial Nerve Antidromic Evoked Potential in Patients With Bell's Palsy: A Preliminary Study

OBJECTIVE: To assess the practical diagnostic value of facial nerve antidromic evoked potential (FNAEP), we compared it with the diagnostic value of the electroneurography (ENoG) test in Bell's palsy. METHODS: In total, 20 patients with unilateral Bell's palsy were recruited. Between the 1st and 17th days after the onset of facial palsy, FNAEP and ENoG tests were conducted. The degeneration ratio and FNAEP latency difference between the affected and unaffected sides were calculated in all subjects. RESULTS: In all patients, FNAEP showed prolonged latencies on the affected side versus the unaffected side. The difference was statistically significant. In contrast, there was no significant difference between sides in the normal control group. In 8 of 20 patients, ENoG revealed a degeneration ratio less than 50%, but FNAEP show a difference of more than 0.295+/-0.599 ms, the average value of normal control group. This shows FNAEP could be a more sensitive test for Bell's palsy diagnosis than ENoG. In particular, in 10 patients tested within 7 days after onset, an abnormal ENoG finding was noted in only four of them, but FNAEP showed a significant latency difference in all patients at this early stage. Thus, FANEP was more sensitive in detecting facial nerve injury than the ENoG test (p=0.031). CONCLUSION: FNAEP has some clinical value in the diagnosis of facial nerve degeneration. It is important that FNAEP be considered in patients with facial palsy at an early stage and integrated with other relevant tests.

Relationship among Characteristic of Blink Reflex, Facial Nerve Electroneurography and Functional Lesion at the Early Stage of Bell's Palsy / 中国康复理论与实践

Objective To investigate the relationship among blink reflex, facial nerve electroneurography and functional lesion at the ear-ly stage of Bell's palsy. Methods Blink reflex and electroneurography of facial nerve were performed in 25 patients with early Bell's palsy. They were divided into mild to moderate damage and severe damage according to the result. They were evaluated by House-Blackmann (H-B) scale and were identified:grade I, normal;grade II-III, mild-moderate facial paralysis;grade IV-VI, severe facial paralysis. Results The positive rate of blink reflex was 100%, with the proportion of mild-moderate damage was 28%and severe damage was 72%. H-B scale evaluation showed the percentage of mild-moderate facial paralysis was 44%, severe facial paralysis was 56%, which was consistent with blink reflex (P>0.05). The positive rate of electroneurography was 52%, with the proportion of mild-moderate damage was 44%and severe damage was 8%, which was inconsistent with H-B scale evaluation (P<0.05). Conclusion Blink reflex is preferable to electroneurography in evaluating facial paralysis degree at the early stage of Bell's palsy.

Influence of 125I seed interstitial brachytherapy on recovery of facial nerve function / 中华放射医学与防护杂志

Objective To study the influence of 125I seed interstitial brachytherapy in parotid region on the recovery of facial nerve function. Methods A total of the data of 21 patients with primary parotid carcinoma were treated with resection and 125I interstitial brachytherapy. All the patients had no facial palsy before operation and the prescribed dose was 60 Gy. During 4 years of follow-up, the HouseBrackmann grading scales and ENoG were used to evaluate the function of facial nerve. According to the modified regional House-Brackmann grading scales, the facial nerve branches of patients in affected side were divided into normal and abnormal groups, and were compared with those in contra-lateral side.Results Post-operation facial palsy occurred in all the patients, but the facial palsy recovered within 6 months. The latency time differences between affected side and contralateral side were statistically significant in abnormal group from 1 week to 6 months after treatment ( t = 2.362, P = 0.028 ), and were also different in normal group 1 week after treatment ( t = 2.522, P = 0.027 ). Conclusions 125I interstitial brachytherapy has no influence on recovery of facial nerve function after tumor resection and no delayed facial nerve damage.

Prognostic Value of Electrophysiologic Tests in Children with Facial Nerve Palsy / 대한소아신경학회지

PURPOSE: This study was aimed to evaluate the value of electrophysiologic tests for determining prognosis in children with facial nerve palsy. METHODS: We retrospectively analyzed 37 children diagnosed as the facial nerve palsy at the pediatric neurology clinic, Kyungpook National University Hospital from January 1, 2000 to March 31, 2007. RESULTS: A total of thirty seven children were involved in the study(male to female 21:16, and the mean age 87.5 months). Among those twenty one had electrophysiologic tests. As compared with the normal values, the amplitude decreased by 54.5%(0.6+/-0.5 mV) and the latency was prolonged by 11.0%(3.6+/-0.5 msec) in electroneurography(ENoG). Early response(R1) was absent in 15 out of 21(71.4%) and ipsilateral response(R2) was absent in 19 children(90.5%). As compared with the children who had the decrease of amplitude 90% or less in ENoG, the children with the decrease of amplitude greater than 90% showed poor recovery(100% vs 60%, P<0.05) and longer duration of follow-up(43.7+/-30.0 days vs 184.00+/-196.8 days, P<0.05). All children who had R1 and R2 responses in the Blink test were completely recovered from the illness, but they were not statistically different from the other groups. CONCLUSION: Decrease of amplitude in ENoG and responsiveness in the Blink test can be important prognostic determinants in children with facial nerve palsy, but further studies are needed.

A electroneurographic technique of the facial nerve / 实用口腔医学杂志

砄bjective: To explore an effective method for Electroneurography(ENoG) of facial nerve. Methods: ENoG of 114 normal subjets and 69 patients with traumatic facial nerve injuries were investigated. Latency time(LT), duration time(DT), amplitude(A) and square under the curve(S) were measured in facial muscles when facial nerve was stimulated postauricularly or anteroauricularly. Results: In most facial musles except square muscle of lower lip postauricular stimulation gave longer LT, shorter DT, lower A and smaller S. Conclusion: The postauricular stimulation is effective prior to anteroauricular stimulation for electroneurography of the facial nerve.