Prednisone treatment for idiopathic facial paralysis (Bell's palsy).
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Administration, Oral↗
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Intraoperative monitoring of facial and cochlear nerves during acoustic neuroma surgery.
The likelihood of successful preservation of facial and cochlear nerve function during acoustic neuroma surgery has been improved by the advent of intraoperative monitoring techniques. The facial nerve is monitored by recording EMG from facial muscles, with no muscle relaxants used; mechanical irritation of the nerve during surgery causes increased EMG activity, which can be detected in real time using a loudspeaker. Brief episodes of activity associated with specific surgical maneuvers aid the surgeon in avoiding damage to the nerve, whereas prolonged tonic EMG activity may reflect significant neural injury. Electrical stimulation with a hand-held probe elicits evoked EMG responses, which can be used to locate and map the nerve in relation to the tumor. The threshold for eliciting evoked EMG responses provides a rough indicator of the functional status of the nerve. Different nerves in the posterior fossa (trigeminal, facial, spinal accessory) can be identified in multichannel recordings by the spatial distribution and latency of responses to electrical stimulation. The ability to elicit EMG responses from low amplitude stimulation of the facial nerve at the brain stem after tumor removal is a reasonable predictor of postoperative facial function. Cochlear nerve function is assessed by recording the ABR from ear canal and scalp electrodes or the CNAP with an electrode placed directly on the nerve at the brain stem root entry zone. The ABR is a well-known, noninvasive technique that can be adapted to intraoperative use relatively easily but is of limited utility owing to the delay inherent in signal averaging. Direct CNAP recordings require placement of an intracranial electrode in such a way as to contact the cochlear nerve without interfering with surgical access but have the distinct advantage of rapid feedback on changes in cochlear nerve status.
Cochlear Nerve↗
[Cholesteatoma in petrous part of temporal bone].
OBJECTIVE: To explore the clinic characteristic of petrous bone cholesteatoma. METHOD: One case was treated by supra-labyrinth approach. 8 cases did by labyrinth-cochlea approach. RESULT: After 2 to 9 years follow-up in the 8 cases, no local cholesteatoma relapsion occurred. 2 cases were treated by facial nerve decompression and facial paralysis did not recover postoperatively. 1 case suffered from facial muscle movement 2 months after operation and recovered after 7 months later. 1 case still needs further follow-up with 2 months postoperatively. CONCLUSION: Proper surgical approach should be selected according to location and invasion of disease. Packing with adipose tissue and blocking the external auditory meatus are effective methods to prevent the cerebrospinal fluid leak.
Adolescent↗
Characterization of the tetanus toxin model of refractory focal neocortical epilepsy in the rat.
PURPOSE: To characterize in detail a model of focal neocortical epilepsy. METHODS: Chronic focal epilepsy was induced by injecting 25-50 ng of tetanus toxin or vehicle alone (controls) into the motor neocortex of rats. EEG activity was recorded from electrodes implanted at the injection site, along with facial muscle electromyographic (EMG) activity and behavioral monitoring intermittently for up to 5 months in some animals. Drug responsiveness was assessed by using the antiepileptic drugs (AEDs) diazepam (DZP) and phenytoin (PHT) delivered systemically, while 1,2,3,4-tetrahydro-6-nitro-2,3-dioxo-benzo[f]quinoxaline-7-sulfonamide (NBQX), a competitive antagonist at AMPA receptors, was administered directly to the brain to investigate the potential benefits of focal drug delivery. RESULTS: Tetanus toxin induced mild behavioral seizures that persisted indefinitely in all animals. EEG spiking activity, occurring up to 80% of the time, correlated with clinical seizures consisting of interrupted behavioral activity, rhythmic bilateral facial twitching, and periods of abrupt motor arrest. Seizures were refractory to systemic administration of DZP and PHT. However, focal delivery of NBQX to the seizure site reversibly reduced EEG and behavioral seizure activity without detectable side effects. CONCLUSIONS: This study provides a long-term detailed characterisation of the tetanus toxin model. Spontaneous, almost continuous, well-tolerated seizures occur and persist, resembling those seen in neocortical epilepsy, including cortical myoclonus and epilepsia partialis continua. The seizures appear to be similarly resistant to conventional AEDs. The consistency, frequency, and clinical similarity of the seizures to refractory epilepsy in humans make this an ideal model for investigation of both mechanisms of seizure activity and new therapeutic approaches.
Animals↗
Measurements of respiratory capacity in patients with neuromuscular diseases.
Weakening of the respiratory muscles in patients with neuromuscular diseases (NMDs) threatens them with respiratory failure. The respiratory capacity of these patients is estimated by pulmonary functional tests. One of the most frequently used tests is the measurement of vital capacity (VC) and determination of the percentage share of the VC reference value (%VC). Different body parameters such as body height and body weight are used for determining the %VC. It is difficult to measure these parameters in wheelchair-bound NMD patients, especially those in more advanced stages of disease. Another reason for questioning the objectivity of spirometric testing in NMD patients is that many of them manifest severely weakened facial muscles and consequently have trouble with blowing. This article describes alternative testing of respiratory capacity and calculation (mathematical model) of predicted pulmonary functional values that is not based on the body height but on other more objectively measured anthropometrical parameters.
Adult↗
Neuregulin-1 isoforms are differentially expressed in the intact and regenerating adult rat nervous system.
Our knowledge on Neuregulin-1 (Nrg-1) during development of the nervous system is increasing rapidly, but little is known about Nrg-1-ErbB signaling in the adult brain. Nrg-1 is involved in determination, proliferation, differentiation, and migration of neurons and glial cells in the developing brain. In the peripheral nervous system, Nrg-1 signaling is required for Schwann cell differentiation and myelination, and establishment of neuromuscular junctions (NMJs). Multiple alternative splicing of Nrg-1 was shown, but correlation of its structural and functional diversity was rarely addressed. Therefore, we investigated the expression of Nrg-1 isoforms in the rat brain and brain-derived cell types, and their involvement in regeneration of the adult brain, using immunohistochemistry, in situ hybridization, and semiquantitative RT-PCR. We found expression of at least 12 distinct Nrg-1 isoforms in the brain and altered expression of several isoforms in the facial motor nucleus after peripheral transection of the seventh cranial nerve. An upregulation of Nrg-1 type-I mRNA, probably type- I-alpha, was observed in reactive astrocytes of the facial nucleus 1 d postaxotomy. Nrg-1 type-III and the splice variants beta1 and beta5 are dramatically downregulated in axotomized motoneurons, which lack contact to their target tissue. Baseline expression levels were reestablished when the first axons reached the facial muscles and reformed NMJs. Nrg-1-beta1 and -beta5 might act in maintenance of NMJs. The splice variants beta2 and beta4 display an initial downregulation of mRNA levels, followed by an increase during the period of axon remyelination. Thus, Nrg- 1-beta2 and -beta4 might be involved in myelination.
Alternative Splicing↗
Facial reanimation: a proposal to reduce postoperative morbidity.
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Adult↗
Routine intraoperative facial nerve monitoring during otologic surgery.
We have used intraoperative monitoring and stimulation of facial nerve function routinely in 301 consecutive otologic and neurotologic cases. The device has been safe, simple to use, and practical. Facial contraction is detected by a strain-gauge sensor in the corner of the mouth and is signalled audibly to the surgeon. Electrical stimulation of the facial nerve can be delivered through a sterile probe, which produces a constant-current-square-wave impulse. The device has several advantages: it signals unintentional mechanical stimulation of the facial nerve during surgery; it allows mapping of the nerve through soft tissue, tumor, and bone; it predicts dehiscences in the bony covering of the nerve; and it allows confirmation of the electrical integrity of the nerve before and after surgery. In this paper we present a technical description of the device, relevant intraoperative electrical measurements, and illustrative case examples. Although the device does not replace anatomic knowledge and surgical ability, it provides a margin of security during ear surgery. This system for intraoperative facial monitoring is practical, and the authors encourage its routine use.
Adolescent↗
Clinical correlates with anti-MuSK antibodies in generalized seronegative myasthenia gravis.
The term seronegative myasthenia gravis (SNMG) refers to the generalized disease without detectable anti-acetylcholine receptor (anti-AChR) antibodies. In these patients, IgG antibodies against the muscle-specific kinase (MuSK) have been described, which reduced agrin-induced AChR clustering in vitro. We have assayed anti-MuSK antibodies in 78 patients with SNMG, who have been followed for many years in our Institution. Here we describe the clinical phenotype of the 37 patients whose results were positive on this assay. MG with anti-MuSK antibodies was characterized by a striking prevalence of female patients (eight men and 29 women). Age of onset ranged from 6 to 68 years, with 56.8% of patients presenting under 40 years of age. All these patients shared a similar pattern of muscle weakness, with prevalent involvement of cranial and bulbar muscles and a high frequency of respiratory crises; the involvement of limb muscles was comparatively less severe and inconsistent. Single-fibre-EMG confirmed the most sensitive examination in the EMG diagnosis of MuSK-positive disease, while, owing to weakness topography, repetitive nerve stimulation in limb muscles was diagnostic in 56.8% of cases. The effect of edrophonium (or neostigmine) injection was equivocal or negative in 11 of 37 patients (29.7%), and the response to oral pyridostigmine was even more unsatisfactory, ranging from mild benefit to overt intolerance. In thymectomized patients, thymus was normal for age or atrophied, and no benefit from surgery was noticed. Thirty-five of 37 patients were given immunosuppressive therapy and 22 received plasma-exchange. The course of the disease was often characterized by periodic exacerbation phases requiring hospitalization and even assisted ventilation; plasma-exchange produced marked improvement in these cases. At the end of the observation period, most patients, although improved, were still symptomatic, having developed permanent facial and pharyngeal weakness together with some atrophy of facial muscles. MuSK-negative disease was comparatively more heterogeneous. Most patients were affected with mild to moderate symptoms and responded well to pharmacological treatment; however, a few subjects in this group had severe refractory disease, poorly responsive to both acetylcholinesterase inhibitors and immunosuppressants.
Adolescent↗
Facial nerve management in cranial base surgery.
This study reviewed 124 patients who required facial nerve manipulation during cranial base surgery. Most of them underwent only nerve displacement or selective transection for improved surgical access to the cranial base (70 and 34, respectively). Fourteen patients had the facial nerve resected for oncologic reasons and repaired with primary nerve grafting. Most patients regained quite satisfactory facial function with quality correlating with the degree of nerve injury. Six patients had facial nerve resected as part of oncologic palliation and had the facial deficit rehabilitated with regional tissue. A correlation between preoperative facial nerve weakness and the quality of nerve graft function was not found. An oncologic correlation, however, is suggested (patients with preoperative weakness had less favorable prognosis). Overall, patients who require facial nerve resection for oncologic reasons do not do as well as those with normal preoperative function.
Electric Stimulation↗
[A 54-year-old man with generalized muscle atrophy and oculomotor paresis].
We report a 54-year-old man with progressive generalized muscle atrophy and ophthalmoparesis in the terminal stage. He was well until 44 years of age (1982) when he noted weakness in his right hand and muscle atrophy; in May of 1985, he noted weakness in his left hand and in both legs. His weakness had become progressively worse, and he became unable to walk in November of 1985. He noted dysarthria one month later, and dysphagia in March of 1986. His difficulty in swallowing had also become worse; he regurgitated foods into the trachea in September of that year, and he developed a low grade fever on the same day. He was admitted to our service on September 24, 1987. On physical examination, general findings were unremarkable, except for low grade fever (37.3 degrees C). On neurologic examination, he was alert and mentally sound. He had normal vision and visual fields; ocular movements were normal. He had moderate weakness in facial muscles, dysarthria, dysphagia, and atrophy in his tongue. He had marked generalized muscle atrophy with fasciculation. He was unable to stand or walk. His muscle strength was not more than 1/6 in any part. The lower extremities were spastic. Deep reflexes were exaggerated in both lower extremities but were normal in upper extremities. Sensation was intact. Laboratory examination was unremarkable, and so was the cranial CT scan. He was treated with nasogastric feeding. He was able to communicate smoothly using his eyes, but a restriction in the vertical gaze was noted in February of 1989. The range of ocular movement was better in the oculocephalic reflex compared with his spontaneous vertical eye movements. In April of 1990, his horizontal gaze also had become slow, and he was complicated by bronchial asthma. He was treated with 20 mg/day of prednisolone; after the institution of prednisolone, his horizontal eye movement showed much improvement. In the terminal stage, he was able to move his eyes only very slowly; vertical gaze was impossible. His subsequent course was complicated by respiratory tract infection and septicemia, and he expired on July 15, 1992. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that this patient had amyotrophic lateral sclerosis with oculomotor paresis. Post-mortem examination revealed spongy change involving the posterior column and the posterior spinocerebellar tract, in addition to severe degenerative change in the upper and the lower motoneurons, which were consistent with amyotrophic lateral sclerosis.(ABSTRACT TRUNCATED AT 400 WORDS)
Amyotrophic Lateral Sclerosis↗
Computer-enhanced emotion in facial expressions.
Benson & Perrett's (1991 b) computer-based caricature procedure was used to alter the positions of anatomical landmarks in photographs of emotional facial expressions with respect to their locations in a reference norm face (e.g. a neutral expression). Exaggerating the differences between an expression and its norm produces caricatured images, whereas reducing the differences produces 'anti-caricatures'. Experiment 1 showed that caricatured (+50% different from neutral) expressions were recognized significantly faster than the veridical (0%, undistorted) expressions. This held for all six basic emotions from the Ekman & Friesen (1976) series, and the effect generalized across different posers. For experiment 2, caricatured (+50%) and anti-caricatured (-50%) images were prepared using two types of reference norm; a neutral-expression norm, which would be optimal if facial expression recognition involves monitoring changes in the positioning of underlying facial muscles, and a perceptually-based norm involving an average of the expressions of six basic emotions (excluding neutral) in the Ekman & Friesen (1976) series. The results showed that the caricatured images were identified significantly faster, and the anti-caricatured images significantly slower, than the veridical expressions. Furthermore, the neutral-expression and average-expression norm caricatures produced the same pattern of results.
Adult↗
An index of self-regulation of emotion and the study of repression in social contexts that threaten or do not threaten self-concept.
This research tests a model of repression (M. Mendolia, 1999; M. Mendolia, J. Moore, & A. Tesser, 1996) which specifies that the interaction of individual differences in emotional responsiveness and situational threats to self-concept contributes to one's tendency to regulate emotional responsiveness. This research demonstrates that (a) individuals regulate their autonomic activity, facial muscle activity, cognitive attention, and subjective experience during isolated and repeated exposures to self-threatening negative and positive emotional events and (b) repressive behavior can be predicted by the Index of Self-Regulation of Emotion, which complements and extends conventional categorical measures of dispositional repression. This model provides a more detailed understanding of basic mechanisms in emotion by identifying how individual differences in emotionality and particular social contexts contribute to self-regulation of emotion.
Adaptation, Psychological↗
[Contribution of early electromyography in the prognostic assessment of facial paralysis].
26 patients suffering from total facial palsy of different etiologies underwent an electromyography of the facial muscles between the 5th and 15th day, and were followed up for one year. The muscular activity in forced mimetics, the blink reflex, and the electric response latency were studied after stimulation of the stylomastoid foramen. Early EMG with detection of one or several motor units is a decisive factor in the final prognosis. The methodology and advantages of EMG are compared to those of electroneuronography.
Adolescent↗
An objective evaluation method for facial mimic motion.
This technique was designed to establish a simple, objective evaluation system for facial paralysis through the use of a personal computer. A total of 24 marks were placed on the faces of subjects for the following procedures. Movements of the face were photographed with a video-camera and fed continuously into the computer. Ten frames per movement representing facial movement from rest to maximum movement were selected for analysis. By means of a digital image-processing technique, only the marks placed on the face were extracted, and the movement of these marks was quantitatively analyzed. A total of 44 healthy subjects with no history of facial paralysis were used as a normal control group. The patients with facial paralysis consisted of nine subjects with Bell's palsy and three with Ramsay Hunt syndrome. In the eye-closing motions, no significant differences were found between the sum of the movement distances on the left and right sides in each normal subject. However, the patients with facial paralysis showed distinct differences from those obtained in the normal subjects. The improvement process was also evaluated with a ratio of affected- and normal-side facial movements.
Blinking↗
Objective computerized versus subjective analysis of facial synkinesis.
OBJECTIVES/HYPOTHESIS: To compare the sensitivity of an objective, computerized approach to measurement of facial synkinesis with that for a subjective approach and to examine the test-retest reliability of these approaches. STUDY DESIGN: Prospective, nonrandomized, and blinded. METHODS: Remote facial motion at the upper eyelids and oral commissures during a closed-lip smile and eyelid closure were measured using a commercially available computerized motion analysis system. Thirty healthy adults with normal facial nerve function were enrolled to establish normative data and a threshold value for synkinesis. Thirty consecutive patients were analyzed based on the synkinesis threshold value. Blinded subjective evaluations by two observers were also performed independently on the same patients. RESULTS: Facial synkinesis was detected significantly more frequently with objective than subjective analysis for eyelid closure; no significant differences between approaches were shown for the closed-lip smile. Interestingly, five (17%) patients developed early synkinesis within 3 months from the onset of the facial nerve injury. The test-retest reliability of all objective measures was excellent for both expressions. Close agreement in percent of patients with synkinesis between test and retest subjective evaluations was obtained for both observers for both expressions. CONCLUSIONS: The objective, computerized approach to detection of facial synkinesis has excellent reliability and is more sensitive than the subjective approach to assessment to the presence of facial synkinesis associated with eyelid closure. The finding of early synkinesis suggests that central nervous reorganization plays a role in regeneration of the facial nerve.
Adult↗
Dismissing-avoidant pattern of attachment and mimicry reactions at different levels of information processing.
Individuals with a dismissing-avoidant pattern of attachment are assumed to repress anxiety-related signals, a disposition hypothesized to interfere with facial mimicry and emotional contagion. Further, they are assumed to have one internal working model associated with anxiety, operating out of awareness at early, automatic stages of information processing, and another positive model operating at later, cognitively controlled stages of processing. The main aim of the present investigation was to compare facial mimicry in dismissing-avoidant and non-dismissing subjects at different levels of information processing. Pictures of happy and angry faces were exposed to 61 subjects at three different exposure times (17, 56, and 2,350 ms) in order to elicit facial muscle reactions, first at automatic levels and then at a more controlled levels. Corrugator activity ("frowning muscles") represented negative emotions and zygomaticus activity ("smiling muscles") positive emotions. The dismissing-avoidant subjects scored significantly lower on emotional empathy than the non-dismissing subjects. At the automatic level the dismissing-avoidant subjects showed "normal" corrugator responses (negative emotions) upon exposure to angry faces. At the cognitively controlled level of processing (2,350 ms) a significant interaction effect was shown between Faces x Muscles x Attachment pattern. The dismissing-avoidant subjects showed no corrugator response and an increased zygomaticus response ("smiling reaction") to the angry face, whereas the non-dismissing subjects reacted with a significant mimicking reaction. The dismissing-avoidant subjects' tendency to "smiling" in response to the angry face at the controlled level (2,350 ms) may be interpreted as a repression of their earlier, automatically evoked (56 ms) negative emotional reaction.
Adult↗
Evidence for intraaxonal spread of Listeria monocytogenes from the periphery to the central nervous system.
Rhombencephalitis due to Listeria monocytogenes is characterized by progressive cranial nerve palsies and subacute inflammation in the brain stem. In this paper, we report observations made on mice infected with L. monocytogenes. Unilateral inoculation of bacteria into facial muscle, or peripheral parts of a cranial nerve, induced clinical and histological signs of mainly ipsilateral rhombencephalitis. Similarly, unilateral inoculation of bacteria into lower leg muscle or peripheral parts of sciatic nerve was followed by lumbar myelitis. In these animals, intraaxonal bacteria were seen in the sciatic nerve and its corresponding nerve roots ipsilateral to the bacterial application site. Development of myelitis was prevented by transsection of the sciatic nerve proximally to the hindleg inoculation site. Altogether, our results support the hypothesis that Listeria rhombencephalitis is caused by intraaxonal bacterial spread from peripheral sites to the central nervous system.
Animals↗