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[Evaluation of the prognosis of facial paralysis after surgery of acoustic neurinoma by an early Hilger's test].

Surgery for acoustic neuroma is highly risky for the facial nerve. This is why we have tried to assess immediate postoperative facial impairment by performing Hilger's test within the first few hours. Further evolution of the facial function showed the predictive value of Hilger's test as regards facial recovery. Indeed, a difference greater than 2 mA as compared with the opposite side indicates a lack of facial recovery at the 3rd postoperative month. In addition, facial muscle testing performed within the first postoperative 72 hours makes the prognosis of facial function recovery possible. Lack of facial recovery at the 3rd postoperative month was observed for neuromas with a diameter exceeding 2 cm. Preoperative assessment of the risk of facial sequellae is fundamental and essentially depends on the size of the tumor and on the surgical approach. Intraoperative facial monitoring allows better ensuring the preservation of the VIIth cranial nerve and consequently improves postoperative function.

Electric Stimulation↗

Meiges disease: a clinical form of facial convulsion, bilateral and medial.

In 1910 the French neurologist Henry Meige described in detail a disorder characterized chiefly by symmetric dystonic spasms of the facial muscles, which he called "spasm facial median." Cases with this disorder are rare, and frequently misdiagnosed and inappropriately treated. We report here a translation of Meige's original description of "spasm facial median." We hope that this translation will make it easier to recognize and diagnose this disorder and that it will stimulate greater interest in this unusual syndrome.

Dystonia↗

[A case of facioscapulohumeral muscular dystrophy with infantile spasms, sensorineural deafness and retinal vessel abnormality].

We report an 18-year-old female with facioscapulohumeral dystrophy (FSHD), who had sensorineural deafness, retinal vessel abnormality, mental retardation, and epilepsy. She had infantile spasms at 6 months of age. Muscle atrophy and weakness of facial muscles were first noticed at 3 years of age. From 10 years of age, she had rapidly progressive generalized muscle weakness especially of facial, neck and truncal muscles with marked lordosis. Although mental retardation is commonly complicated with FSHD, infantile spasms or epilepsy has never been reported. Not only mental retardation but epilepsy may be one of the central nervous system symptoms in a systemic disorder, FSHD.

Adolescent↗

The bispectral index declines during neuromuscular block in fully awake persons.

UNLABELLED: Bispectral index (BIS) is an electroencephalographic variable promoted for measuring depth of anesthesia. Electromyographic activity influences surface electroencephalography and the calculation of BIS. In this study, we sought to determine the effect of spontaneous electromyographic activity on BIS. BIS was monitored in three volunteers by using an Aspect A-1000 monitor. The experiment was repeated in one volunteer. Electromyographic activity was recorded. Alcuronium and succinylcholine were administered. No other drugs were used. In parallel with spontaneous electromyographic activity of the facial muscles, BIS decreased in response to muscle relaxation to a minimum value of 33 and, in the repeated measurement, to a minimum value of 9 when total neuromuscular block was achieved. In two volunteers, no total block was achieved. BIS decreased to a minimal value of 64 and 57, respectively. In turn, recovery of BIS coincided with the reappearance of spontaneous electromyographic activity. During the entire experiment, the volunteers had full consciousness. BIS, assessed by software Version 3.31, correlates with spontaneous electromyographic activity of the facial muscles. BIS failed to detect awareness in completely paralyzed subjects. Thus, in paralyzed patients, BIS monitoring may not reliably indicate a decline in sedation and imminent awareness. IMPLICATIONS: The bispectral index (BIS) is an electroencephalographic variable intended for measuring depth of anesthesia. Electromyographic activity influences the calculation of BIS. We found that the administration of a muscle relaxant to unanesthetized volunteers decreases the bispectral index value. Thus, awareness in totally paralyzed patients cannot be excluded.

Alcuronium↗

Relative fatigability of muscles in response to repetitive nerve stimulation in myasthenia gravis.

BACKGROUND: Repetitive nerve stimulation (RNS) plays an important role in the diagnosis of myasthenia gravis (MG). Technically, this test can be more easily performed on distal muscles than proximal muscles. On the other hand, proximal muscles have been shown to be more easily fatigued or more sensitive than the distal ones. OBJECTIVE: Evaluate the relative fatigability of different muscles in response to RNS in ocular and generalized MG patients. MATERIAL AND METHOD: Two hundred patients with 44 ocular MG and 156 generalized MG were studied The relative fatigability of each muscle was calculated as percentage of cases with abnormal response. A decrement of 10% or more of the amplitude of the compound muscle action potentials was considered abnormal. RESULT: The cases with abnormal response of adductor digiti minimi, anconeus, trapezius, nasalis and orbicularis oculi were 17%, 50%, 55%, 62%, 66% in ocular MG and 53%, 77%, 79%, 85%, 79% in generalized MG respectively. These results showed that in generalized MG the response in all the muscles was more frequently abnormal and was fatigable in a more comparable degree, whereas in ocular MG, the facial muscles were more often abnormal than the limb muscles. CONCLUSION: The abnormal response because of fatigability was more widespread in generalized MG whereas facial muscles were relatively more sensitive in ocular MG. This finding may be useful for the selection of the muscles to be tested in RNS especially in ocular MG.

Adolescent↗

The management of hyperfunctional facial lines with botulinum toxin. A collaborative study of 210 injection sites in 162 patients.

OBJECTIVE: To determine the optimum dose and efficacy of botulinum toxin injections in the management of hyperfunctional facial lines. DESIGN: This study included 210 hyperfunctional facial sites in 162 different patients. The patients had preinjection and postinjection photographic documentation and ratings on a 4-point qualitative evaluation scale of lines at rest and with action. The patients then had botulinum toxin type A injections via a monopolar hollow bore, Teflon-coated electromyographic needle into the facial muscles associated with the hyperfunctional lines. The total dose for each region of 1.25 to 25 U was divided into 1.25- to 5-U aliquots representing 0.1 to 0.2 mL per injection site, depending on the site and the prior experience with that patient on using toxin. The patients had their reevaluation at 2 to 3 weeks after injection. Patients returned for further follow-up when the therapeutic effect diminished. PATIENTS: One hundred sixty-two patients had 210 hyperfunctional sites evaluated and injected. The group consisted of 25 male patients and 137 female patients ranging in age from 21 to 78 years with a mean (+/-SD) of 46.1 (+/-1.98) years. All patients had cosmetically troubling hyperfunctional lines involving the forehead, glabella, crow's feet (lateral canthal lines), nasolabial area, platysma, and mentalis region. RESULTS: All patients had an effect of toxin within the first 24 to 72 hours. Ninety-five percent of the patients treated had cosmetic improvement of unsightly facial lines or contractions. The best results were achieved in management of the forehead lines, followed by glabella, crow's feet, and nasolabial. The dose for forehead lines was 5 to 25 U (mean +/- SD, 17.3 +/- 6.2 U); glabellar lines, 5 to 20 U (mean +/- SD, 11.1 +/- 3.1 U); crow's feet, 5 to 15 U (mean +/- SD, 6.2 +/- 1.6 U); nasolabial, 2.5 to 5 U (mean +/- SD, 3.12 +/- 1.2 U); and platysma, 10 to 20 (mean +/- SD, 15 +/- 4.0 U). Evaluation by age and site suggested a trend of increased toxin dose with increased age. Effects of the toxin are usually seen 24 to 72 hours after injection, and last from 3 to 6 months, whereon the increased muscular activity returns, as do the hyperfunctional lines. The only morbidity was related to temporary mild weakness of other adjacent facial muscles. There were no systemic side effects noted. CONCLUSION: Botulinum toxin is a safe and important adjunctive technique for the management of patients with symptomatic hyperfunctional facial lines.

Adult↗

The spectrum of Mobius syndrome: an electrophysiological study.

We studied the nature and extent of facial muscle innervation and the involvement of the motor and sensory long tracts in Möbius syndrome, in order to shed light on the pathophysiological mechanism of the syndrome. Standardized blink reflexes, direct responses of the facial nerves to the orbicularis oculi muscles and concentric needle electrode electromyography in orbicularis oculi and/or oris muscles were measured in 11 patients with Möbius syndrome, of whom six participated in MRI studies, all showing absent facial nerves. We performed motor- and somatosensory-evoked potentials in seven Möbius patients. We demonstrated three distinct patterns of abnormalities suggesting different sites of the primary lesion in different patients. (i) Presence of normal blink reflexes and facial compound motor action potentials, normal habituation tests, a reduced recruitment in the facial muscles and an aberrant 'blink reflex-like' response of the orbicularis oculi muscle upon stimulation of the facial nerve region, which suggests a supranuclear origin of the defect. (ii) Absent blink reflexes, absent direct responses of the facial nerves and absent motor activity on needle electromyography, indicating a defect at the facial nuclear level. However, the nuclear defect might mask an additional supranuclear defect, which cannot, therefore, be excluded in these patients. (iii) A disperse pattern of facial compound action potentials combined with long latencies that were recorded with concentric needle electrodes, indicating involvement of motor axons in the facial nerve, possibly secondary to nuclear involvement. An additional supranuclear defect cannot be excluded in these cases. All evoked potentials studied were normal. The electrophysiological findings of the facial muscles show a spectrum of disturbances varying in degree of severity and diverse in the extent of structures involved, in 11 Möbius patients. At one end of the spectrum are patients with completely immobile faces in whom electrophysiological testing shows no signs of involvement of the facial nuclei, nerves or muscles, suggestive of a dysfunction at the supranuclear level. At the other extreme of the spectrum are patients with complete absence of responses upon facial nerve stimulation and absence of motor unit activity. This is at least indicative of a defect at the facial nuclear level. While a supranuclear defect is compatible with the concept that Möbius syndrome is a developmental disorder of the lower brainstem, intact facial nuclei as part of the syndrome has not been suggested before. The findings corroborate the concept of the Möbius syndrome being a complex regional developmental disorder of the brainstem.

Adolescent↗

[A patient with neuroborreliosis presenting gadolinium-enhanced MRI lesions in bilateral facial nerves].

We report a 33-year-old man with bilateral facial paralysis due to neuroborreliosis. About three weeks after rhinorrhea and fever lasting four days, he noticed fatigue in the legs and paresthesia in all four extremities. Another week later, he developed paresthesia in his tongue and bilateral facial muscle weakness, and was admitted to our hospital. On admission, neurological examination revealed moderate bilateral facial muscle weakness, mild paresthesia in the tongue and four extremities, and decreased Achilles tendon reflex bilaterally. Mild pleocytosis and increased protein were found in the cerebrospinal fluid (CSF). IgM antibodies that reacted with the antigens of Borrelia garinii and Borrelia afzelii were found in his serum. Clinically and serologically, he was thus diagnosed as having neuroborreliosis. Brain MRI revealed gadolinium-enhanced lesions of the bilateral facial nerves in the facial nerve canal portion. After three weeks of treatment with 100 mg/day doxycycline and 2 g/day ceftriaxone sodium, his symptoms and CSF abnormalities were rapidly improved. Although facial nerve paralysis is a major symptom of neuroborreliosis, the present report is the first to detect the inflammatory lesions of the facial nerves in the facial nerve canal portion by MRI.

Adult↗

Muscular reconstruction to improve the deterioration of facial appearance and speech caused by mandibular atrophy: technique and case reports.

One of the consequences of severe mandibular atrophy is the loss of attachment of the facial muscles that originate from the alveolar process and basal bone. Another is a loss of vestibular depth and reduction in the width of the attached gingiva. The result is reduced ability to chew, a changed and aged appearance, difficulties with pronunciation, and a reduced range of expressions. The traditional goal of treatment has been to improve the ability to chew. We describe a technique by which all these functions can be improved by a combination of insertion of implants and functional reconstruction of the facial muscles and position of the lips. When the muscles are repositioned, the buccal vestibule is deepened, and the incidence of gingival hyperplasia and infrabony pockets along the posts is eliminated. This treatment, which also rejuvenates the face and improves the ability to speak, should help to overcome the loss of self-confidence and self-esteem of these patients by improving their quality of life.

Atrophy↗

Mechanoreceptive units in the human infra-orbital nerve.

Eighty-four low-threshold mechanoreceptive afferents innervating facial hairy skin or the red zone of the lip were recorded with micro-electrodes from the human infra-orbital nerve. Based on their responses to skin indentations, the units were classified as slowly or fast-adapting, with small or large receptive fields. The responses to hair movement, skin stretching and contraction of facial muscles were also studied. Both hairy skin and the red zone were innervated by slowly and by fast-adapting units. The innervation density was found to be highest at the corner of the mouth and on the upper lip. Slowly adapting units with small fields in hairy skin were most common and included units responding to sustained hair displacement. These units are suggested to have two types of end-organs, either pilo-Ruffini endings or Merkel cell-neurite complexes. The slowly adapting units with large fields were spontaneously active stretch receptors and may have corresponded to Ruffini corpuscles, although the possibility of other, intramuscular, receptors could not be ruled out. Only one afferent possibly innervated a Pacinian corpuscle. Most mechanoreceptors were also activated by skin stretching or contraction of facial muscles. Many of the slowly adapting units with small fields responded to the onset and release of stretch, whereas their discharge in response to sustained stretching adapted more or less completely. Spontaneously active units had the most sustained stretch response. It is concluded that several types of cutaneous mechanoreceptors can operate as sensitive proprioceptors of importance for facial kinaesthesia and motor control.

Adult↗

[Post paralytic facial spasm: new pathogenetic findings (author's transl)].

The study of the early blink and lip reflex components in patients with postfacial palsy contracture and mass contractions shows changes in the synaptic organization of the facial nucleus. The possible contribution of nuclear factors to the altered function of reinnervated facial muscles is discussed.

Adult↗

The development of synkinesis after facial nerve paralysis.

In this study, the development of clinical synkinesis after facial nerve paralysis (FP) and its relationship to electrophysiological findings were investigated. Thirty-four patients who were examined within the first 5 days after onset of FP and who could also be followed up for at least 4 months were included in the study. Electrophysiological investigations consisted of: (1) recording of the direct responses by facial nerve stimulation at the stylomastoid fossa; (2) recording of the 'synkinetic spread' of the supraorbital nerve reflex to the lower facial muscles; (3) recording of the 'lateral spread responses' by stimulating the mandible and zygomatic branches of the facial nerve. Clinical synkinesis developed in 14 of 18 patients (78%) with a direct response ratio (DRr) of less than 40%. Among the 16 patients with a DRr of 40% or more, synkinesis was observed in 3 cases (18.7%) only. The DRr provided reliable information concerning the development of synkinesis. Forty percent seemed to be a reasonable limit to distinguish the high-risk group for the development of clinical synkinesis.

Adolescent↗

Analysis of 100 cases of free-muscle transplantation for facial paralysis.

Free-muscle transplantation is the treatment of choice for long-standing facial paralysis. It enables the reconstructive surgeon to restore facial movement and some emotional animation. Despite all technical innovations and 20 years of experience with free-muscle transplantation, the aesthetic and functional outcomes of the surgery are still unpredictable. The present report reviews 100 free-muscle transplantations to the face by a single surgeon and analyzes various preoperative, intraoperative, and postoperative factors in relation to the functional recovery of the muscle transplants. These factors were demographic variables such as age, gender, and etiology as well as intraoperative variables such as choice of muscles, number of nerve coaptations, and ischemia time of the muscle. Additionally, four independent raters not involved in the care of these patients rated standardized preoperative and postoperative videos and judged the functional and aesthetic outcomes. From 1981 to 1993, 93 patients with facial paralysis underwent free-muscle transplantation. A total of 100 muscles were transplanted, since 7 patients received two muscle transplants. There were 33 male and 60 female patients ranging in age from 3 to 57 years, with an average of 22.2 +/- 14.9 years. The gracilis muscle was used in 63 cases of free-muscle transplantation, while the pectoralis minor was used in 34 cases. In 2 patients a segment of the rectus abdominis was transferred, and in 1 patient a small segment of the latissimus dorsi was transferred. In 89 patients the onset of muscle function was reported. The range was from 6 to 48 weeks postoperatively. The average was 21.6 +/- 9.14 weeks after muscle transplantation. The correlations showed a trend to earlier onset of function and higher aesthetic rating in young female patients. The intraoperative ischemia of the free muscle did not correlate with the onset of muscle function. Using a five-step scale of judgments, a higher postoperative rating was seen in 94 percent of the patients, and 80 percent of all patients achieved a moderate or better result.

Adolescent↗

Inhibition of intracranial self-stimulation in brain stem-transected cats--a proposed mechanism of aversive effects produced by brain stimulation.

Effects of intracranial self-stimulation of central 'punishment areas' were studied on an operant conditioning of vertical eye movements in the midpontine pretrigeminal cats as well as in the encéphale isolé cats. In 36 pretrigeminal cats, the ventromedial hypothalamus (VMH), basal amygdaloid nuclei (AMY), dorsal central gray (CG) of the midbrain and the thalamic nuclei such as the ventralis posteromedialis (VPM) and ventralis posterolateralis (VPL) were tested. No suppression of eye movements indicating a passive avoidance conditioning from stimulation of these 'punishment areas' was obtained in 92 electrode tip sites. In 49 encéphale isolé cats, stimulation of the VPM associated with contraction of the facial muscles, demonstrated a marked passive avoidance effect on the eye movements. After blocking both the trigeminal (5N) and facial nerves (7N), VPM stimulation no longer produced an increase of facial EMG activity and the suppressive effect on eye movements was abolished. Extracranial blockade of 7N alone, which induced facial muscle paralysis also showed similar effects. Bilateral blockade of cranial nerves from acoustic (8N) to hypoglossal (12N) nerves had no significant effect on the avoidance conditioning. The mass neural activity recorded from the 5N showed a marked increase of discharge by VPM stimulation which was reduced significantly after 7N blockade. These results may suggest a possibility that punishing effects of brain stimulation depend on feedback from the periphery (muscles, blood vessels and visceral organs), whereas reward effects essentially depend on neural circuitry confined within the forebrain above the rostral pons.

Amygdala↗

Ipsilateral facial weakness in upper medullary infarction-supranuclear or infranuclear origin?

We describe two patients with upper medullary infarctions showing ipsilateral facial weakness and relative sparing of the upper facial muscles. Electrophysiological follow-up using transcranial magnetic stimulation of the motor cortex in combination with stimulation of the peripheral facial nerve disclosed a supranuclear (corticofacial) tract lesion in one patient and a partial nuclear/infranuclear intra-axial facial nerve lesion in another.

Aged↗

Altered synaptic organization in facial nucleus following facial nerve regeneration: an electrophysiological study in man.

Seven patients with postfacial palsy contracture and mass contractions were investigated electrophysiologically. In 3 patients the early blink reflex showed an unusually high amplitude, which can be attributed to enhanced excitability of facial motor neurons. In 5 patients the early blink reflex had acquired a crossed character. It is assumed that changes in organization of the facial nucleus contribute to the altered function of reinnervated facial muscles.

Action Potentials↗

[Treatment of facial nerve paralysis using static suspension methods].

After the injury of facial nerve, facial muscles are subjected to complex series of biochemical and histological changes, which lead to muscular atrophy if reinnervation is not restored. Facial palsy is very difficult to manage completely. Regardless this fact, the plan of correction has to be directed towards the following: restoration of normal function, normal facial appearance at rest, symmetry in voluntary movements as well as symmetry in involuntary and emotional movements. Static suspension methods were used in our study. All patients had unilateral complete facial nerve palsy but one female patient who experienced the palsy of frontal branch of n.facialis. This method was successfully used to lift the eyebrow, the lid and to improve lagophthalmus on the paralytic side, then the angle and paralytic part of the lip, to reinforce buccal wall of oral cavity as well as to reconstruct new nasolabial fold. The results were satisfactory and permanent.

Facial Muscles↗