The Poland anomaly with unilateral peripheral facial paralysis

The authors present the clinical history and physical findings in a 16-month baby girl with the diagnosis of Poland's syndrome associated with another congenital anomaly: unilateral peripheral facial palsy, on the opposite side. The authors believe this is the first report of such an association.     

Idiopathic facial paralysis

Although acute idiopathic facial paresis is often labelled "Bell's palsy", historical studies show that Nicolaus Anton Friedreich (1761-1836) from Würzburg was the first physician to describe the typical symptoms of the disorder in 1797, approximately 24 years prior to the paper published by Sir Charles Bell. Diagnostics has now improved to the extent that acute idiopathic facial palsy can more frequently be assigned to etiologies caused by inflammatory disorders. Herpes simplex virus type I and Borrelia burgdorferi are particularly relevant. Underestimation of the degree of paresis is, particularly in children, a drawback of the clinical examination. "Incomplete eyelid closure" is not a reliable indicator of remaining nerve function. For this reason complete electromyography (EMG) is recommended in all cases of severe facial paresis. Since electroneurography does not reliably reflect the degree of denervation present, needle EMG is preferred. The therapy of the facial palsy of unclear etiology is still not well defined. Nevertheless, we recommend that a combined treatment should be used early, at least in patients with disfiguring pareses. Combinations may consist of cortisone, virostatic agents and hemorrheologic substances and possibly antibiotics. Surgical decompression of the facial nerve remains controversial, since positive surgical results lack statistical support. Individual instructions for facial exercises, massage and muscle relaxation can support rehabilitation and possibly reduce the production of pathological synkinesia. Electrical stimulation should not be used. There are a number of possibilities available to reduce the effects of misdirected reinnervation, especially the use of botulinum-A-toxin. However, intensive diagnosis and therapy in the early phase of paresis are decisive in obtaining a favorable outcome. Further refinements in rehabilitation and comparative multicenter controlled studies are still required for future improvements in affected patients.     

An assessment of physical and psychological stress of patients with facial paralysis

The purpose of this study was to determine if there is a relationship between the appearance of facial paralysis and physical or psychological stress. Subjects were 55 patients (23 men, 32 women) with facial paralysis (32 with Bell's palsy, 23 with Ramsay Hunt syndrome) who visited the Department of Otolaryngology, Nihon University Itabashi Hospital, from August 1994 to March 1996. At the time of the initial visit, patients were asked if they had any physical or psychological stress one week before the occurrence of facial paralysis. The degree of psychological stress was rated by Niina's Psychological Stress Response Scale 50 Items Revised (PSRS-50R). Answers were obtained from 52 of the 55 subjects. Forty patients (76.9%) reported that they had felt physically fatigued, suggesting some type of relationship between the occurrence of facial paralysis and physical stress. Twenty-seven patients (51.9%) reported the existence of psychological stress. However, the evaluation of psychological stress by PSRS-50R did not reveal a high level of psychological stress in any patient, indicating lack of a strong relationship between the occurrence of facial paralysis and psychological stress. Stepwise regression analyses were made to study the relationship between the degree of paralysis at the time of the initial visit and factors which affect the prognosis of the paralysis. Those patients who reported physical fatigue before the appearance of facial paralysis had more abnormal values on the nerve excitability test than those who did not report such fatigue.     

Transtympanic facial nerve paralysis

In order to ascertain the immediate histopathological changes of rat brain following experimental surgical injury, fragments of left parietal cortex were obtained through craniotomy and fixed by immersion. Next, the animals were killed by perfusion with the same fixative used for the respective biopsy. Five groups of rats were tested, each for one different fixative. Dark neurons were by far the most prominent feature in surgically traumatized tissue, following both immersion and perfusion with all five fluids. They were morphologically identical at both sites, and fixatives with widely different chemical composition, e.g. Bouin's fluid and buffered glutaraldehyde, had no influence on their aspect. It is suggested that shrunken neurons are not to be interpreted systematically as artefacts, but also represent a form of short-latency cellular reaction to injury.     

Correlation between vocal functions and glottal measurements in patients with unilateral vocal fold paralysis

Observations and analysis of glottal characteristics are critical in choosing the best modality for surgery in patients with unilateral vocal fold paralysis (UVP). This study suggests that multiple glottal characteristics influence the vocal product in patients with UVP. In addition to the horizontal position of the paralyzed vocal fold (deviation from the midline), the glottal area, degree of bowing of the paralyzed and contralateral vocal folds, maximum separation between vocal folds, compensatory glottal maneuvers, and the vertical glottic closure plane significantly influenced the quality of the voice. Clinicians should be aware of these observations to facilitate treatment planning and assessment of the results of surgical procedures used to improve voice quality in cases of UVP.     

Peripheral facial paralysis: diagnosis

A radioimmunoassay procedure for human chorionic somatomammotrophin in indiluted plasma has been developed in which the incubation lasts only five minutes rather than one half hour to four days in the previously described methods. The values obtained had excellent correlation (p less than or equal to 0,001) with those obtained by a two-hours technique. The intra-assay and inter-assay coefficients of variation are respectively 7 percent and 11 percent. Twenty women at term showed human chorionic somatomammotrophin values of 7.5 +/- 2.5 mug/ml plasma (mean +/- SD).     

The treatment of idiopathic facial paralysis

Louisiana's new Medical Assistance Programs Provider Agreement Law creates a complex contractual relationship between the state and every person providing care, items or services to the Louisiana Medicaid Program. In privately funded healthcare, it is called a "network contract." This new law gives the Department of Health and Hospitals at least 12 express reasons to cancel a person's contract to provide care to Medicaid beneficiaries. The law also, however, gives a right to either party--either the provider or the state--to cancel the contract at will, and even without cause, by the mere giving of a 30-day advance written notice, and thus this new law might be said by some to stretch the notion that "any willing provider" may participate in Medicaid to near the breaking point. There are many new rules and conditions, some of which have not yet even found their way into privately funded healthcare.     

Validation of objective measures for facial paralysis

A shift from subjective scales to objective measures of facial paralysis requires physical models against which to validate and calibrate the new objective techniques. The purpose of this report was to demonstrate such a model and to test the capacity of an objective computer system to accurately measure physical change. The physical model consisted of an advancing edge of a spreading gelatin film. The model was measured in submillimeter increments. Concurrent measurements were made using the computed system. Ten trials were conducted. The objective system proved to be exquisitely sensitive (0.03 mm) and highly correlated with the physical criterion model (Pearson's product moment correlation coefficient [r]=0.9849). Sensitive and calibrated objective methods of analysis of facial motion are crucial to the design of clinical trials, clinical studies of nerve regeneration, and comparisons of reanimation techniques.     

Deficits and recovery of first- and second-order motion perception in patients with unilateral cortical lesions

Unilateral lesions in the posterior parietal cortex can degrade motion perception in the contralesional visual hemifield. Our aim was to investigate whether deficits caused by cortical lesions may be different for first- and second-order motion perception, and to study the time scale of any potential recovery. In nine patients with circumscribed lesions mainly in the parietal and fronto-parietal cortex, thresholds for direction discrimination were measured for stimuli presented peripherally in their ipsi- and contralesional hemifield. Subjects had to identify the direction of a vertically moving object embedded in a background of dynamic random dot noise. The object consisted of various proportions of signal and noise dots. Signal dots were either (a) coherently moving in the same direction as the object (first-order), (b) stationary (second-order: drift-balanced), or (c) coherently moving in the opposite direction (second-order: theta). Noise dots were flickering. Two patients showed significant threshold elevations for all three types of motion stimuli presented in their contralesional hemifield, while thresholds for ipsilesional targets were unaffected. Neither showed any selective deficit of first- versus second-order motion perception, but second-order motion was more impaired. Their lesions probably included the motion area V5-MT, which was spared in the other seven patients. One of the patients, who was retested several times during a 27-month postlesional period, showed complete recovery for first- and second-order motion direction discrimination, as well as for the detection of speed differences.     

Regional lung function in unilateral diaphragmatic paralysis

Radioactive xenon-133 was used to study the regional lung function of five patients with unilateral diaphragmatic paralysis unassociated with intrathoracic disease. All patients showed a reduction in total lung capacity to which the affected side contributed an average of 37%. There was a decrease in the amount of inhaled xenon and a lesser decrease in the amount of injected xenon reaching the lung base on the paralysed side.The distribution in the opposite lung did not differ significantly from that found in normal subjects although the proportion of inhaled xenon reaching the lung base was rather less than in the normal group. The washout of injected xenon was normal except for slight impairment at the lung base on the paralysed side in one patient and at both bases in another.     

Familial epilepsy with unilateral and bilateral malformations of cortical development

BACKGROUND AND OBJECTIVE: A variety of biologic or synthetic materials have been used in brow suspension ptosis surgery. We describe extrusion and granuloma formation in 5 cases of congenital ptosis operated with Mersilene mesh sling. PATIENTS AND METHODS: Case reports with review of literature. RESULTS: Two cases of extrusion and 3 cases of granuloma formation were encountered in 79 eye lids of 52 (6.3%) patients performed for the treatment of congenital ptosis. Mersilene mesh fibers in the cut surface and a foreign body reaction were found by microscopic evaluation of tissue harvested at the time of surgical removal of the sling. CONCLUSION: Despite the operative precautions, the probability of extrusion and granuloma formation should be in mind in cases of congenital ptosis operated with Mersilene mesh sling, a synthetic material.     

Autologous fat injection for unilateral vocal fold paralysis

This study evaluates the efficacy of autologous fat injection for medialization of the paralyzed vocal fold. In 21 patients with unilateral vocal fold paralysis, autologous abdominal fat was injected into the thyroarytenoid muscle to achieve medialization. All patients were followed up with serial videolaryngoscopy and voice evaluation. At 2 months' follow-up, the voice was judged to be excellent in 10 patients, slightly breathy but significantly better than the preoperative voice in 6 patients, and markedly breathy in 4 patients. At 3 to 4 months' follow-up, of the 10 patients with excellent results, 5 maintained an excellent voice, 3 had developed slight breathiness, and 1 had developed severe breathiness. Long-term (6 to 12 months) results were available in 11 patients, and all of them maintained the same voice quality that was noted during the 3 to 4 months' examination. Magnetic resonance imaging of the larynx was obtained in 7 patients at intervals ranging from 1 to 7 months and compared to the baseline scan obtained at 1 week postoperative to assess the amount of fat remaining in the muscle. The images showed fat volume to persist, but a decrease in the fat signal was observed over time. The results suggest that the duration of medialization with autologous fat is variable, but appears to last at least 2 to 3 months. This loss of volume after 3 months seems to be due to absorption of the fat and possibly muscle atrophy. Autologous fat injection is relatively safe and easy to perform, and is an ideal method of temporary vocal fold medialization in patients in whom return of vocal fold function is expected.     

Effects of primary bone grafting on facial development in patients with unilateral complete cleft lip and palate

The study is based on an anthropometric assessment of X-ray films obtained in 22 adult males with complete unilateral cleft lip and palate treated during childhood with primary bone grafts and in 32 males with the same type of cleft without bone grafting. In the series with bone grafts was recorded a more marked reduction of maxillary depth associated with a larger retrusion than in the series without bone grafts. This deviation was therapeutically compensated by a larger displacement of the mandible backwards which contributes to the increase of mandibular posterior rotation. This provided the possibility to attain an edge to edge bite. Our results confirmed the unfavourable effects of primary bone grafting on maxillary growth and development.     

Outcome and changing cause of unilateral vocal cord paralysis

In order to examine the effects of altered protein sialylation on neural cell function, B104 rat neuroblastoma cells were stably transfected with the cDNA coding for alpha2,6(N) sialyltransferase (ST(6)N). Lectin blot analysis of the clones demonstrated an increase in staining of the Sambucus nigra lectin, which detects alpha2,6 linked sialic acid, in parallel with enzyme activity. There was a concomitant decrease in staining by the Maackia amurensis lectin which labels alpha2,3-linked sialic acid, indicating that the individual sialyltransferase enzymes may compete for penultimate galactose acceptor sites. While there was an initial increase in protein-bound sialic acid in parallel with enzyme activity, the sialylation of the cells was demonstrated to be saturable. There was an inverse relationship between cell adhesion to a fibronectin substrate and ST(6)N activity suggesting that the negatively charged sugar acts to modulate cell-substrate interaction. These cells will provide an ideal model system with which to further investigate the effect of altered sialic acid on neural cell function.     

Diagnostic value of tomography in unilateral vocal cord paralysis

Tomographic studies were made on 56 patients with unilateral paralysis of the vocal cord. The findings were examined in relation to etiology, course, and laryngoscopic findings. In 74.1 percent of the cases, marked enlargement of the ventricle was noticed on the side of the paralysis, especially during inhalation. The paralyzed vocal cord was higher than the intact cord during phonation in 46.4 percent of the cases. The rest of the cases (53.6 percent) demonstrated no level difference between the vocal cords. The position of the paralyzed vocal cord, unilateral involvement of the cricothyroid muscle and other neck muscles were suggested as possible contributors to the findings.     

Bilateral peripheral facial paralysis and Guillain-Barré syndrome

The Guillain-Barrè syndrome is a polyneuropathy of acute onset that initially tends to produce motor damage of the lower limbs, albumin-cytological dissociation in cerebrospinal fluid and electrophysiological findings suggestive of demyelination. This entity produces a wide variety of symptoms, including damage to the facial nerve in as many as half of all episodes, which means that this syndrome should be considered in the differential diagnosis of facial palsy, particularly bilateral and synchronous cases. In a review of six cases of Guillain-Barrè syndrome in which bilateral facial palsy occurred at some point, a history of immediate infection was confirmed in five, mainly related to Herpesvirinae and Campylobacter. One case began with bilateral palsy, whereas palsy appeared almost simultaneously with the rest of the symptoms in the other cases. High protein levels without cells in cerebrospinal fluid and electrophysiological patterns of slow facial nerve conduction were confirmed in every patient. Treatment with intravenous immunoglobulins resolved all episodes in less than six months. We conclude by reiterating the need for awareness of this syndrome in every case of peripheral VIIth nerve palsy, especially bilateral cases, although the associated symptoms were not very useful, if at all useful.     

按摩治疗早期周围性面瘫的临床体会

目的:研究按摩配合超短波等综合治疗对周围性面瘫早期患者治疗的作用.方法:100例早期周围性面瘫患者进行按摩配合超短波治疗,恢复期仍采用电针、按摩、超短波治疗.结果:早期周围性面瘫患者进行按摩配合超短波治疗疗效确切.结论:按摩配合超短波治疗早期周围性面瘫治疗更有利于面神经运动功能恢复,可有效的缩短治疗时间避免早期针灸的副作用.     

The effect of unilateral partial facial paralysis and muscle ablation on craniofacial growth and development: an experimental study in the rabbit

The effect of unilateral partial facial nerve ablation and unilateral partial midface muscle ablation on craniofacial growth and development was investigated. New Zealand White rabbits (12 days old) were randomly assigned to three experimental groups: control group, to study normal craniofacial growth and development (n = 15); nerve ablation group, surgically induced unilateral paralysis of the buccal branches of the facial nerve (n = 15); and muscle ablation group, surgical unilateral ablation of the facial muscles innervated by the buccal branches of the facial nerve (n = 12). All animals were operated on at the age of 12 days; follow-up evaluations were performed at the ages of 2 months and 6 months. The age of 2 months represents the endpoint of the prepubertal craniofacial growth and development. At the age of 6 months, the animals are fully grown; therefore, the time period between 2 and 6 months is regarded as the pubertal growth period. Computerized dorsoventral roentgencephalometric (measurement of distances and angles) and computer tomographic (three-dimensional volumetric measurements) investigations were performed at both ages. Additional dry skull measurements were performed to determine more precisely the bone segments involved in the craniofacial growth alterations studied. The obtained results indicated the following. Unilateral partial facial paralysis involving the midface resulted in growth alterations analogous to those seen after unilateral total facial paralysis. The growth alterations were not to be seen as a growth restriction (reduction in bony volume) but as growth misdirections (alterations in shape). Major growth alterations were present in those regions closely related to the facial musculature, namely the nasal, maxillary, and premaxillary regions, resulting in a snout deviation toward the operated side. The growth alterations occurred during prepuberty and remained rather stable during puberty. Morphologic signs of muscle denervation were related to the craniofacial growth disturbances. The growth alterations after unilateral partial facial paralysis were mainly biomechanically induced, as they were analogous to those observed after unilateral midfacial muscle ablation. The fact that after unilateral midfacial muscle ablation at the age of 6 months the severity of the alterations had increased was attributed to the scar formation inherent to the surgical procedure.