How phlebotomy affects the erythrocyte metabolism of high- and low-glutathione sheep

The introduction in the 60' s of the Nerve Excitability Test (NET) by Laumans and Jongkees for the diagnosis of Bell's palsy stimulated an interest of quantitative testing of the facial nerve. In 1970 Yanagihara reported the Evoced Myography (EMG; i.e. NET is combined with EMG). Starting with electrophysiological basis of the impulse spread of peripheral nerves May, in 1971, extended this concept by including supraliminal stimuli, i.e. Maximal Stimulation Test (MST), for diagnostic purposes. This method eventually developed into what is known as neuronography (Esslen, Huffmann, Ehrenberger, Satoh). It is undoubtedly a refinement in the question of prognosis of Bell's palsy. Continuing the original concept of May we have developed an electronic device with which to carry out automatically the neuronography that demonstrates an evaluation on a numeric display. We use the conventional bipolar negative square wave for a 1 ms stimulation. We stimulate the angle of the mandible, where the ramus marginalis can be readily found in all patients. Neuronography and Evoked Myography provide earlier a more precise prognosis of facial palsy than Nerve Excitability Test (NET). We developed an electronic device for the Maximal Stimulation Test (MST) of May, M., that is automatic. The device counts the spikes of the evoked myogram. A time window is related to the stimulus to exclude stimulus noise and background muscle noise from the counter. Only spikes seen in this window are processed. The number of spikes generated per stimulus is the measurement of the live axons in a stimulated nerve.     

A comparison of electroneuronography with facial nerve latency testing for prognostic accuracy in patients with Bell's palsy

The purpose of this study was to evaluate the ability of electroneuronography (ENOG), also called evoked electromyography (EEMG), and facial nerve latency testing (FNLT) to assess the prognosis of facial nerve palsy, using the House-Brackmann facial nerve grading system as criterion. From 1988 to 1994 these tests were employed at the ORL Clinic of the University of Ioannina in 250 patients with idiopathic facial nerve palsy. The ENOG test results indicated that when the amplitude of the compound muscle action potentials ranged from 51% to 95% of the normal value, 97% of the patients achieved complete functional recovery (grade I) within at least 2 months. When the muscle action potential decreased to a value below 51% of normal values, prognosis for recovery was considerably worse. FNLT test results indicated that as the latency time extended, the recovery grade of the facial nerve worsened. When latency time was within the normal range (group A patients), about 92% of patients had complete functional recovery. In contrast all patients having either a very extended latency time or unable to be monitored (groups C and D) demonstrated incomplete functional recoveries that ranged from grade II to grade VI. Comparing each test with the House-Brackmann facial nerve grading system, we ascertained that the percent accuracy for ENOG was 97.6%, and that for FNLT was 94.4%.     

Biological effects and cellular uptake of c-myc antisense oligonucleotides and their cationic liposome complexes

OBJECTIVE: To review patient selection and factors affecting the decision for surgical or non-surgical management of patients with trauma-induced facial nerve palsy. STUDY DESIGN: Retrospective review of 85 consecutive cases occurring over a 6-year period. METHODS: Between 1984 and 1990, 85 cases of facial nerve palsy following trauma were treated. Patient assessment included clinical, audiological, radiological and EMG examinations. Depending on test results, patients were either given medical treatment or underwent total facial nerve decompression surgery. RESULTS: Recovery was achieved in all 33 medically treated patients. Among the 52 surgically-treated patients (61.2%), 47 had immediate facial nerve palsy at admission (90.4%). Onset of facial palsy was delayed in 5 others, including 3 for whom the delay to onset was unknown. Surgical accesses used were the middle fossa and transmastoidal (60%), translabyrinthine (11%), and pure transmastoidal (29%) routes depending on hearing loss, location of fracture line and general patient condition. Lesions were predominantly found in the geniculate ganglion area. A nerve gap was found in only 13.5% of the cases. Two years post-operatively, 92.3% of the patients had grade I-III recovery. No grade V or VI cases were observed. DISCUSSION AND CONCLUSION: The rarity of severe nerve lesions encountered in surgically-treated patients raises the question of better candidate selection for surgery. At present, imaging techniques, particularly radiology, can help to choose the best indication. Immediate total facial palsy associated with a clear-cut fracture going through the Fallopian canal is perhaps the only case requiring surgery. When the delay of onset is unknown, an EMG indicating a total denervation process is the decisive argument.     

Facial palsy in Kawasaki syndrome

Facial nerve palsy, a very rare complication of Kawasaki syndrome, has been reported in only 25 patients. We treated a 12-week-old boy with bilateral coronary artery aneurysms due to Kawasaki syndrome who developed marked unilateral peripheral facial nerve palsy on day 36 of illness. None of the 25 previously reported patients with this complication were treated with immunoglobulin; they required 7 to 90 days to recover. In our patient, treatment with this agent was associated with complete resolution of facial nerve palsy within 36 hours. Review of prior cases demonstrates that children with Kawasaki-associated facial nerve palsy have more than twice the risk for coronary artery aneurysm (52% vs <25%) as that of children who do not develop this neurological complication. Unexplained facial nerve paralysis in young children with a prolonged febrile illness should provoke consideration of Kawasaki syndrome and of echocardiography to exclude coronary artery aneurysms. Although facial palsy appears likely to resolve in all patients that survive the acute phase of Kawasaki syndrome, treatment with intravenous immunoglobulin appears to considerably shorten the time to full recovery and provides an important clue to the mechanisms of neurological injury in this illness.     

A comparison of transcranial magnetic stimulation with electroneuronography as a predictive test in patients with Bell's palsy

The aim of this study was to examine the neuronographic findings of electrical and transcranial magnetic stimulation of the facial nerve and to compare their ability to predict clinical recovery from idiopathic facial nerve palsy (Bell's palsy). Eighty-six patients were examined clinically and neurophysiologically immediately on presentation to Tampere University Hospital. Electroneuronography (ENoG) and transcranial magnetic stimulation (TMS) were performed 1-6 times for each patient. The time interval between each examination varied from 2 to 7 days. Seventy-eight patients were followed for a median period of 13 months after the onset of palsy. Facial nerve function was graded according to the House-Brackmann grading system. Relative amplitude differences of ENoG and TMS during the acute phase were then correlated with clinical outcome. Statistical analysis of the results showed that a TMS response elicitable during the first 5 days of the palsy was correlatable with a good prognosis. ENoG results correlated with clinical outcome at a later time from onset of symptoms. TMS was well tolerated and no adverse effects were seen. These results indicate that TMS is a useful method for the early prediction of outcome in patients with Bell's palsy.     

Unusual metastasis of hypernephroma in the maxillary sinus

OBJECTIVES: To determine the predictive value of intraoperative threshold stimulus for facial nerve outcome and the prevalence and prognostic value of persistent trains of activity and frequent spontaneous or mechanically induced contractions during acoustic neuroma surgery. STUDY DESIGN: Prospective recording and subsequent review of facial nerve activity. SETTING: Tertiary referral centre. PATIENTS AND METHODS: Consecutive patients undergoing acoustic neuroma surgery. Intraoperative facial nerve activity was digitised and stored on a personal computer for future analysis. Operative events were flagged. Recordings were available in 27 patients. MAIN OUTCOME MEASURES: Frequent mechanically induced contractions (< 20), prolonged trains of facial nerve activity (total time > 199 seconds), and facial nerve brainstem stimulus threshold were correlated with facial nerve outcome. RESULTS: A brainstem stimulus threshold > 0.1 mA was significantly associated with intermediate or poor facial nerve function (House-Brackmann grade > 2) on the sixth postoperative day, at 1 month and 6 months. Patients with normal or near-normal facial function on the first day and a threshold of > 0.1 mA were significantly more likely to develop a delayed facial nerve palsy. Frequent contractions were noted in 74% of patients and persistent train activity in 59%. Neither was predictive of facial nerve outcome. CONCLUSIONS: An elevated brainstem threshold is helpful in predicting delayed facial nerve palsy and suboptimal facial nerve outcome. Persistent train activity and frequent contractions, do not have major prognostic significance.     

Gd-DPTA enhanced MRI in Bell's palsy and herpes zoster oticus: an overview and implications for future studies

Magnetic resonance imaging (MRI) is a new and important tool for use in diagnosing and investigating diseases affecting the facial nerve. In recent gadolinium-DTPA enhanced MRI (Gd-MRI) studies it has unequivocally been demonstrated that ipsilateral facial nerve contrast enhancement, predominantly in the meatal portion, is present in both Bell's palsy and herpes zoster oticus. In this overview, the results of MRI studies performed on patients with acute peripheral facial palsy, especially Bell's palsy and herpes zoster oticus, are discussed. The Gd-MRI pattern in Bell's palsy is very similar to that seen in herpes zoster oticus, and the findings reported so far support the theory that an inflammation may be the cause of the nerve injury in both cases. So far, however, Gd-MRI has not been helpful in evaluating the severity and/or prognosis of the facial palsy. Further studies employing improved techniques, including three-dimensional fast (or turbo) spin echo (3DFSE) MRI with heavily T2-weighted sections and high resolution three-dimensional Fourier transform (3DFT) MRI, need to be conducted in order to determine whether it is possible to follow the course of the disease and whether MRI and/or Gd-MRI are useful prognostic tools in the early stages of palsy.     

Transverse and T fractures with fracture of the posterior wall of the acetabulum. Results of orthopedic and surgical treatment. Apropos of 52 cases

A patient developed delayed facial nerve palsy at the level of House-Brackmann grade I to grade III 10 days after vestibular schwannoma surgery by the suboccipital transmeatal approach. The palsy had completely recovered after one month. Immunological study showed reactivation of herpes simplex and magnetic resonance (MR) imaging demonstrated an abnormal enhancement pattern of the facial nerve; intense enhancement of the distal intracanalicular segment and labyrinthine segment, similar to the MR findings for Bell's palsy. A prospective control study on the enhancement pattern of the functionally preserved facial nerve after vestibular schwannoma surgery in six cases showed a similar pattern to that of the normal facial nerve. Based on these findings, we propose the hypothesis that herpes simplex reactivation is an underlying cause of delayed facial palsy after vestibular schwannoma surgery.     

Facial nerve enhancement on gadolinium-DTPA in a case with neurosarcoidosis

In a case of neurosarcoidosis with bilateral facial nerve palsy and hydrocephalus, contrast-enhanced magnetic resonance imaging (MRI) study and angiotensin converting enzyme (ACE) activities in cerebrospinal fluid (CSF) were valuable for the diagnosis and the follow up. Facial nerve lesions were demonstrated on gadolinium-DTPA enhanced MRI. The disappearance of enhancement was concomitant with the amelioration of facial nerve palsy after corticosteroid therapy.     

Facial palsy following trauma to the external ear: 3 case reports

We report two children and a young adult who developed unilateral facial palsy shortly after injury to the external ear. In two instances the paralysis followed a prominent ear correction and in the other a laceration to the concha. The trauma-triggered facial palsy was most likely idiopathic although the anatomy of the facial nerve near the ear leads one to speculate on a possible pathway of a virally induced palsy (Bell's palsy). Each patient recovered over a period of 6 months.     

Computerized dynamic visual acuity test in the assessment of vestibular deficits

Hypoglossal facial anastomosis (HFA) is a standard surgical technique for restoration of facial movements in cases of intratemporal lesions of the facial nerve. Case reports provide evidence that an affected trigeminal system reduces functional outcome. In order to detect morphological changes in the hypoglossal nucleus responsible for this phenomenon, we used 18 Wistar rats and performed three different surgical combinations. In group 1, six animals received HFA only. In group 2, HFA was combined with resection of the contralateral infraorbital nerve. In group 3, HFA was combined with resection of the ipsilateral infraorbital nerve. Fifty-six days after the operation, horseradish peroxidase (HRP) was injected into the whisker pad. As shown in previous studies using HRP, retrograde-labelled motoneurons occurred in the hypoglossal and facial nuclei. Counts of the labelled motoneurons showed no change in the number of projecting hypoglossal motoneurons in group 2 when compared to HFA only, but a significantly smaller number in group 3 (-35%). Furthermore, the number of projecting facial motoneurons was significantly reduced in group 2 (-85%) and group 3 (-45%). These morphological findings indicate an absent or insufficient functional connection between the contralateral infraorbital nerve and the hypoglossal nucleus, and a strong influence of the infraorbital nerve to the ipsi- and contralateral facial nuclei. Additionally, our study provides morphological evidence that the integrity of the sensory trigeminal system is very important in reconstructive facial nerve surgery.     

Intensity of MR contrast enhancement does not correspond to clinical and electroneurographic findings in acute inflammatory facial nerve palsy

PURPOSE: To determine the value of MR contrast enhancement in predicting the course of acute inflammatory facial nerve palsy and in selecting patients for surgical decompression. METHODS: Six patients with an acute inflammatory incomplete or complete peripheral facial nerve palsy (five idiopathic and one herpetic in origin) had repeated MR imaging studies with and without contrast enhancement, electroneurography, and clinical examinations to establish a connection between the intensity of contrast enhancement on MR images, the clinical condition, and the electrophysiological data. The examinations were performed every second day starting on the first day of admission until clinical recovery was proved by clinical deblockage (spontaneous clinical improvement). The last examination was performed 3 months after the onset of the facial nerve palsy. RESULTS: An abnormal, very intense contrast enhancement of the facial nerve was always present in the distal intrameatal and proximal tympanic segments and in the geniculate ganglion. The labyrinthine segment exhibited a mild to moderate enhancement, and the distal tympanic and mastoid segments showed a moderate to intense enhancement. The intensity of contrast enhancement did not correspond to the severity, duration, or course of the facial nerve palsy, and the electroneurographic data had no predictive value in indicating the severity of the inflammatory process. Three months after clinical recovery, a persistent and more or less unchanged or even slightly more intense contrast enhancement was observed. CONCLUSION: The long-lasting intense contrast enhancement seen in the facial nerve segments of patients who have acute peripheral inflammatory facial nerve palsy is explained by a two-phase breakdown of the blood-nerve barrier.     

Effects of electrical stimulation of cervical sympathetic trunks on microcirculation in the facial nerve

This study evaluates the circulatory effects of electrical stimulation of the cervical sympathetic trunks on blood flow in the common carotid artery and facial nerve tissue in dogs. Marked increases in arterial pressure and heart rate were observed due to electrical stimulation of the cervical sympathetic trunks, while blood flow volume in the common carotid artery and in the facial nerve tissue decreased markedly. It was assumed that microcirculation of the facial nerve is definitely impaired by electrical stimulation of the cervical sympathetic trunks, and the tonicity of the sympathetic nervous system appears to be a major factor in changes in the microcirculation of the facial nerve. It is well known that impaired circulation in the nutrient vessels of the facial nerve has an important effect on the pathogenesis of facial palsy. The hypertonicity of the sympathetic nervous system is closely involved in the onset of facial palsy.     

Human herpesvirus-6 antibodies in idiopathic facial nerve palsy and sudden deafness

Human herpesvirus-6 (HHV-6) is the causative agent for exanthem subitum. This study investigated the relationship between idiopathic facial nerve palsy (Bell's palsy), sudden deafness and HHV-6 infection. Both Bell's palsy and sudden deafness are syndromes which causes are unknown. Both of them are suspected viral infection as causative agents. Paired sera from 22 patients of Bell's palsy and 39 patients of sudden deafness were examined for reactivity to HHV-6 by the indirect immunofluorescence test. On a case of Bell's palsy and two cases of sudden deafness each of the HHV-6 antibody titers was increased.     

Cardiomyopathy due to a pheochromocytoma. A reversible entity

Bilateral facial nerve palsy is relatively uncommon and may occur in association with a variety of neurological, infectious, neoplastic or degenerative disorders. Presentation is made of 4 cases of bilateral facial diplegia due to a refractory anemia with excess of blasts, a Lyme disease and a tuberculoid leprosy. In one of these patients the cause of bilateral seventh-nerve palsy was unknown (Bell's palsy). Facial palsy returned to normal after treatment with steroids in 3 patients. The patient with myelodysplastic syndrome did not show any improvement and died 6 months after diagnosis.     

Antiphospholipid syndrome associated with progressive systemic sclerosis

Hemifacial spasm is a neurological disorder due to abnormal hyperactivity of the facial nerve. The most common cause of hemifacial spasm is a neuro-vascular conflict in the cerebellopontine angle between a vascular loop and the root of the facial nerve (96% of cases). Tumors are the cause of hemifacial spasm in only 1% of cases). The authors present their results in 100 patients who underwent microvascular decompression for essential hemifacial spasm between 1990 and 1995. They used microsurgical and endoscopic procedures by a minimal retrosigmoid approach in all cases. The most common offending vessels were the posterior inferior cerebellar artery (70%), the vertebral artery (41%) and the anterior inferior cerebellar artery (28%). An aberrant vein was found in 2 cases. There were 38% of multiple artery-nerve conflicts. Physiopathology of hemifacial spasm is explained by two principal theories: in the ephaptic theory, hyperactivity and an abnormal nervous impulse pathway are due to a short demyelinated area on the nerve trunk caused by the offending vessel, inducing short circuiting between adjacent nerve fibers. In the nuclear theory, hyperactivity of the facial nerve is due to an abnormal and automatic activity of the facial nerve nucleus itself, induced by the vessel. The authors used pre and postoperative electromyographic tests and intraoperative electromyographic tests. Their results tend to prove the nuclear theory. Ninety per cent of the patients had a good result, with a mean follow-up time of 30 months in 60 cases. In 82% of the cases, there was a total recovery after a single procedure. There was no mortality and no facial palsy. Hearing loss occurred in less than 5%.     

Quantitative analyses for facial nerve MR imaging

It is clear, from our clinical experience, that the facial nerve in patients with facial palsy is enhanced on magnetic resonance (MR) imaging after intravenous administration of gadolinium diethylenetriamine. However, some problems with clinical reliability persist. There have been reports that normal facial nerves often show enhancement on MR imaging. We also question whether there are any differences in the degree of enhancement between Bell's palsy and Ramsay Hunt syndrome. To solve these problems, analyses were conducted using a personal computer by means of digital image-processing to measure the gray scale levels of enhanced facial nerves on MR imaging films. Seventeen cases of Bell's palsy, eight cases of Ramsay Hunt syndrome and fourteen normal subjects whose facial nerves showed enhancement on MR imaging were selected for the analyses. The concept of a facial nerve/whole image ratio (F/W ratio), analyzing the degree of enhancement of the facial nerve quantitatively, is introduced in this paper. The F/W ratio is the ratio of the gray scale level of the facial nerve region to the highest gray scale level in the skull at the MR imaging film. When the F/W ratios of these subjects were analyzed, no significant differences were found between Bell's Palsy and Ramsay Hunt syndrome in the degree of enhancement; facial palsy cases showed quantitatively larger F/W ratios than normal subjects.     

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.     

Diabetes associated with a novel 3264 mitochondrial tRNA(Leu)(UUR) mutation

OBJECTIVE: To present a novel mitochondrial DNA mutation in a diabetic family RESEARCH DESIGN AND METHODS: The proband was a 64-year-old man. In the family, diabetes was maternally inherited. He had diabetes, cerebellar ataxia, cervical lipoma, hearing loss, olfactory dysfunction, ophthalmoplegia, and facial nerve bilateral palsy. On examination, early insulin secretion was blunted, and the M value on glucose clamp test was low. In muscle, ragged red fibers were not found. T-to-C mutation at position 3264 was detected in the proband (0.5% mutant DNAs in leukocyte and 30% in muscle), but was not detected in 201 normal individuals. RESULTS: Heteroplasmy of mutation, maternal inheritance of diabetes, and symptoms related to mitochondrial dysfunction suggest the pathogenecity of this 3264 mutation. As for diabetes etiology, both impaired insulin secretion and decreased insulin sensitivity seem to be important. In phenotypic characteristics, the combination of cerebellar ataxia and lipoma is a symptom sometimes found in myoclonic epilepsy and ragged red fibers (MERRFs). Ophthamoplegia is a symptom of chronic progressive external ophthalmoplegia (CPEO). These suggest that our proband had phenotypic overlap with MERRF and CPEO. Conversely, facial nerve bilateral palsy is a rare finding. The pictures that focused on his cranial nerves were thus unique, suggesting the heterogeneity of mitochondrial DNA (mtDNA)-related diabetes. CONCLUSIONS: A novel 3264 mitochondrial DNA mutation in diabetes gives new insight to the etiology of mitochondrial diabetes. Its pathogenecity supports the belief that the tRNA(Leu)(UUR) gene is an etiological hot spot of mitochondrial diseases.