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PubMed2004

Post-traumatic bilateral facial palsy: a case report and literature review.

Bilateral facial paralysis due to basilar skull fracture involving the temporal bone is rare and, unlike unilateral facial palsy, it can be difficult to recognize because of a lack of facial asymmetry. Thorough clinical history and physical exam, high-resolution CT scan and electrodiagnostic tests can help to make the diagnosis of bilateral facial nerve palsy and early detection, evaluation and intervention may be important for optimal functional recovery. A 16-year-old male sustained closed head injury after motor vehicle collision. The initial head CT scan showed bilateral temporal bone fractures. On admission to the neurotrauma intensive care unit, his Glasgow Coma Score was 9T. On post-injury day 4, the patient was noted to have incomplete closure of both eyes and 3 days later he had difficulty with bilateral facial muscle movement during a feeding trial. Electrodiagnostic testing confirmed the diagnosis of bilateral facial nerve injury without evidence of significant distal axonal degeneration. A high-resolution CT scan showed bilateral temporal bone fractures without involvement of the fallopian canals. There was no surgical intervention based on the high-resolution CT scan and the delayed onset of facial palsy. A short course of prednisone was administered. By 10 months, the patient showed nearly complete recovery of his bilateral facial nerve function. Early detection, evaluation and intervention are important for optimal functional recovery after facial nerve injury. When the temporal bone is fractured, high suspicion for facial nerve injury, either unilateral or bilateral, is warranted.

Adolescent↗
PubMed1991

The impact of continuous electrophysiological monitoring on preservation of the facial nerve during acoustic tumour surgery.

Continuous electromyographic (EMG) activity and responses to electrical stimulation of the facial nerve were monitored intraoperatively in 35 patients undergoing primary removal of acoustic neuroma. The rate of anatomical and immediate functional preservation of the facial nerve of these patients was compared with a matched population where a facial nerve stimulator and monitoring of facial muscle contraction by the anaesthetist only was employed. Overall, 94% (n = 33) of facial nerves were preserved anatomically in patients with continuous EMG monitoring, compared with 64% (n = 89) of patients (n = 139) without continuous monitoring. Amongst the 33 cases with anatomical facial nerve preservation, 64% (n = 21) had immediate functional preservation. Immediate functional preservation was only achieved in 39% (n = 35) of anatomically preserved facial nerves (n = 89) where conventional monitoring was used.

Electromyography↗
PubMed2005

[Hemifacial spasm as clinical presentation of intracranial meningiomas. Report of three cases and review of the literature].

Hemifacial spasm (HFS) is a clinical entity consisting of brief clonic jerking movements of the facial musculature, beginning in the orbicularis oculi with downward spreading to other facial muscles. Apart from vascular loop compression at the root exit zone of the facial nerve, other causes of HFS are rare. It is exceptional as a form of presentation of intracranial meningiomas We report three cases of patients with meningiomas who presented with HFS, either as an isolated sign or associated with symptoms of rise intracranial pressure or focal deficit. We review the literature and discuss the possible physiopathological mechanisms responsible for this association.

Adult↗
PubMed1993

[Intraoperative monitoring of the facial nerve in transtemporal surgery of acoustic neurinoma].

Acoustic neuroma ablation by the enlarged middle cranial fossa approach results in a low incidence of postoperative facial nerve dysfunction. In 25 consecutive patients with tumors in the cerebellopontine angle with a diameter up to 2.3 cm, intraoperative monitoring of the facial nerve was evaluated. The facial nerve was monitored electrophysiologically with recordings of the spontaneous EMG and electrically evoked potentials (CMAP) from at least three groups of facial muscles. In 4 cases monitoring was helpful for identification and delineation of the facial nerve. The mechanically elicited potentials during tumor preparation and CMAP after electrical stimulation close to the brain stem were compared with the postoperative facial nerve function and the time course of recovery. If the ongoing EMG recordings resulted in potentials below 0.5 mV and the CMAP at the end of tumor preparation was higher than 0.5 mV, facial nerve function was normal in 9 of 10 cases on the first postoperative day. One patient had mild facial palsy. Poor results could be predicted if numerous discharges with amplitudes higher than 0.5 mV and affected CMAP after stimulation near the brain stem were found at the end of surgery. All 3 patients with these findings had facial paralysis at hospital discharge (an average of 9 days after the operation). Recovery from the palsies was found to be prolonged. Intraoperative monitoring of the facial nerve is now used routinely in surgery of the cerebellopontine angle and the internal auditory canal.

Adult↗
PubMed2006

Topographic relationship between the muscle bands of the zygomaticus major muscle and the facial artery.

The aim of this study was to clarify the spatial relationship between the facial artery and the insertion of the zygomaticus major muscle in the perioral region. In 30 of 70 dissections (42.9%), the facial artery passed through the separate muscle bands of the zygomaticus major muscle. The mean diameter of the facial artery passing through the zygomaticus major muscle in this region was 1.74 mm (range 0.82-2.86 mm). The relationship between the facial artery and the insertion of the zygomaticus major muscle in the perioral region described in this study is expected to provide critical information for surgical planning and procedures around the perioral region.

Aged↗
PubMed2000

[Carotid surgery in patients 80 years old or older].

BACKGROUND AND OBJECTIVE: Because of their high age and markedly increased co-morbidity, physicians and geriatricians are often cautious in their indications for carotid thromboendarterectomy (TEA) in patients 80 years or older. However, it is these very patients who are subject to an exponentially increased risk of ischaemic cerebral vascular accidents (CVA). This study examined the morbidity and mortality rates of TEA in patients of this age group at one institution. PATIENTS AND METHODS: Between 1994 and 1998, among a total of 912 TEAs, 46 had been performed in patients 80 years or older (15 women, 31 men): indications, diagnosis and associated diseases as well as perioperative complications were entered prospectively into a data-bank. RESULTS: Only one patient (2.2%) sustained a perioperative CVA and no patient died. Three patients (6.5%) developed transitory neurological deficits. One patient had to have an emergency reoperation because of a postoperative carotid artery thrombosis. One patient had an intraoperative asystole due to a hypersensitive carotid sinus. There were no other serious cardiovascular or pulmonary complications. One patient sustained some oral muscle weakness as a result of intraoperative retractor pull on a branch of the facial muscle. CONCLUSION: These results indicate that even in patients of this age group carotid TEA can be performed with great safety.

Aged↗
PubMed1993

Arrector pili muscles surround human facial vellus hair follicles.

Arrector pili muscles have been assumed to attach to one side of the hair follicle, specifically the posterior wall of the bulge area. In this study, we demonstrated immunohistochemically that desminreactive arrector pili muscle, admixed with the connective tissue sheath, is present around the entire circumference of the vellus hair follicle.

Connective Tissue↗
PubMed1982

X-linked recessive bulbospinal neuronopathy: a report of ten cases.

A form of adult onset 'bulbospinal muscular atrophy' of X-linked recessive inheritance is described in 10 patients from eight families. Muscle weakness in the limbs was mainly proximal and developed in the third to fifth decades of life, often preceded by muscle cramps on exertion and tremor of the hands. Weakness and fasciculation of the facial muscles and tongue were prominent. All the patients had gynaecomastia and some were infertile. Two had diabetes mellitus. Motor nerve conduction studies were normal but most patients had small or unrecordable sensory action potentials in the absence of clinical sensory loss. Plasma creatine kinase levels were considerably elevated and muscle biopsies showed neurogenic atrophy together with secondary myopathic changes. The importance of recognising this distinctive disorder in single cases (six of the present series) is emphasised.

Biopsy↗
PubMed1987

[Transitory tetraplegia following high-pressure ventilation].

We present the case of a 22-year-old asthmatic woman, who had undergone high-pressure servo-ventilation as part of the therapy of an otherwise untreatable asthmatic attack. Ventilation-pressures reached peaks of 80 mg H2O while under relaxation and sedation arterial blood pressure fell to 50/30 mm Hg. Treatment was complicated by pulmonary ruptures, pneumothorax and massive cutaneous emphysema. After successful completion of this treatment phase (day 14), the patient was allowed to regain consciousness and was found to be in a subtotal tetraplegic state: muscles of the neck, shoulders and left leg were slightly less atonic than others. Facial muscles, different qualities of sensitivity, intellectual functions and sense organs did not seem to be afflicted. Restitution of the tetraplegia was complete by day 50 of treatment, a neurological follow up six months after discharge from hospital showed no residual deficit. We hypothesize that the extreme ventilation-pressures together with the decrease of arterial blood-pressure have impaired the blood-flow in the system of the anterior spinal arteries. Together with concordant oedema this effected a localized dysfunction mainly of the ventral medullary areas corresponding to the functions of the anterior spino-cortical tract. Diagnostically we do not fully exclude steroid myopathia, but think in this case the cumulated steroid doses were too small and the course of remission indicates a localized haemodynamic lesion.

Adult↗
PubMed1997

Cervical branch of the facial nerve in leprosy.

This study demonstrates that the platysma is occasionally palsied in leprosy and that this only occurs when the facial nerve already has some other palsy. That there needs to be a facial palsy before there can be a platysma palsy is strongly suggested, in that there was no case of an isolated platysma palsy. Patients, regardless of age or other factors, could mimic a platysma contraction. This obviates the need for electrical testing to examine for a platysma palsy. It also means that a nonfunctioning platysma on clinical examination is, in fact, a palsied platysma. While lagophthalmos is regularly examined for, and any obvious facial paresis would be noticed, less severe forms of facial muscle paresis will only be found if formally examined for. The mechanism whereby the facial nerve is involved in leprosy is not clarified, but our findings suggest that proximal spread of a lesion that began in the zygomatico-temporal branches and reaches to the facial nerve trunk is more likely than new lesions developing de novo in other peripheral facial nerve branches. That the primary lesion is within the facial nerve trunk in all cases but we only see the frequent zygomatic sequelae due to secondary factors is not excluded.

Adolescent↗
PubMed2002

Use of electroneurography as a prognostic indicator of Bell's palsy in Chinese patients.

OBJECTIVE: To evaluate the use of electroneurography (ENOG) as a prognostic indicator in Bell's Palsy for Chinese patients in Hong Kong. STUDY DESIGN: Prospective study. SETTING: Tertiary referral outpatient center. PATIENTS: Sixty-three consecutive patients with a diagnosis of Bell's Palsy in Tuen Mun Hospital, Hong Kong, from January 1995 to January 1998. INTERVENTIONS: ENOG, protective eye care, and exercise of the facial muscles. MAIN OUTCOME MEASURES: ENOG was performed 5 to 14 days after the onset of facial palsy. The recovery of facial nerve function was documented by House and Brackmann grading. All the patients were followed up monthly until recovery or up to 6 months. RESULTS: Sixty-three patients were randomly divided into two groups of 32 and 31 patients. The first part of the study was to analyze the correlation between ENOG values and the chance of recovery in Group 1 patients (n = 32) by means of a logistic regression model. The result showed that patients with ENOG values less than 72.63% had a greater than 90% chance of recovery to House Grade II or better within 2 months (Wald = 6.19, p < 0.05). The second part of the study was to assess the capability of this ENOG value to accurately predict the prognosis of Bell's Palsy in Group 2 patients (n = 31) using Fisher's exact test (p < 0.0001). The sensitivity and specificity of ENOG in predicting a good prognosis (recovery to House Grade III or better after 2 months) in patients with Bell's Palsy were 82% and 100%, respectively. CONCLUSION: The ENOG value as a useful prognostic indicator in Chinese patients with Bell's Palsy in Hong Kong was confirmed.

Adolescent↗
PubMed2000

Proximal myotonic myopathy: clinical, electrophysiological and pathological findings in a family.

Proximal myotonic myopathy (PROMM) is an autosomal dominant muscle disorder characterized by proximal weakness, myotonia, muscle pain and cataract. It resembles Steinert myotonic dystrophy (MD), but weakness is proximal, without facial muscle involvement, and the chromosome 19 CTG trinucleotide repeat expansion characteristic of MD is not present. We describe a further family with PROMM. Affected members complained of weakness of lower limbs or of myotonia. EMG revealed diffuse myotonic discharges. Muscle histology showed dystrophic abnormalities. The PROMM phenotype varies, even in the same pedigree, and may mimic MD or limb-girdle muscle dystrophy. EMG is particularly useful, since it may disclose myotonic discharges even in the absence of overt myotonia. Thus far it is not known whether PROMM is a single entity, or if it represents a heterogeneous group of disorders. This question will probably soon be settled through genetic analysis.

Aged↗
PubMed1990

Prediction of sequelae following facial nerve palsy.

The severity of facial muscle synkinesis, contracture, spasm, crocodile tears and hearing impairment and/or tinnitus were observed carefully following facial nerve palsy and evaluated quantitatively by a scoring system. Using this system, a mathematical formula was obtained to predict sequelae from the severity of facial nerve palsy. The severity of palsy at 2 weeks and 2 months after its onset was an important sign for prediction of sequelae.

Adult↗
PubMed2002

Fiber composition and oxidative capacity of hamster skeletal muscle.

The hamster is a valuable biological model for physiological investigation. Despite the obvious importance of the integration of cardiorespiratory and muscular system function, little information is available regarding hamster muscle fiber type and oxidative capacity, both of which are key determinants of muscle function. The purpose of this investigation was to measure immunohistochemically the relative composition and size of muscle fibers composed of types I, IIA, IIX, and IIB fibers in hamster skeletal muscle. The oxidative capacity of each muscle was also assessed by measuring citrate synthase activity. Twenty-eight hindlimb, respiratory, and facial muscles or muscle parts from adult (144-147 g bw) male Syrian golden hamsters (n=3) were dissected bilaterally, weighed, and frozen for immunohistochemical and biochemical analysis. Combining data from all 28 muscles analyzed, type I fibers made up 5% of the muscle mass, type IIA fibers 16%, type IIX fibers 39%, and type IIB fibers 40%. Mean fiber cross-sectional area across muscles was 1665 +/- 328 microm(2) for type I fibers, 1900 +/- 417 microm(2) for type IIA fibers, 3230 +/- 784 microm(2) for type IIX fibers, and 4171 +/- 864 microm(2) for type IIB fibers. Citrate synthase activity was most closely related to the population of type IIA fibers (r=0.68, p<0.0001) and was in the rank order of type IIA > I > IIX > IIB. These data demonstrate that hamster skeletal muscle is predominantly composed of type IIB and IIX fibers.

Animals↗
PubMed1994

Intraoperative facial nerve monitoring in the surgery of cerebellopontine angle tumors: improved preservation of nerve function.

The surgery of cerebellopontine angle tumors has shown remarkable progress over the last 20 years due to improved microsurgical techniques. However, the dissection of the facial nerve may lead to postoperative paresis as the result of the surgical trauma and the disruption of blood supply over a large distance. The functional status of the nerve can be intraoperatively monitored by means of intramuscular electromyography of facial muscles and direct electrical stimulation. In this study, the impact of monitoring techniques on the preservation of facial function was evaluated by comparing monitored (n = 30) and unmonitored (n = 34) patients. Both groups were comparable with regard to the size of the tumor, the surgical approach and the duration of operation. All patients were operated by the same surgeon (T.L.) either via the middle fossa or the translabyrinthine approach. The EMG was recorded with needle electrodes from the orbicularis oris and oculi muscles. For electrical stimulation, bipolar forcep electrodes were used delivering rectangular, constant current pulses of 100 microseconds duration and a current strength between 0.05 and 0.8 mA. The mechanically or thermally elicited activity by drilling, direct manipulation or coagulation consisted of bursts and trains which are signs of minor nerve impairment. Their occurrence can lead to a modified surgical technique with a more precise preparation around and at the facial nerve. The immediate postoperative nerve function was normal or showed only a minor impairment (classes I and II according to House and Brackmann) in 87% of the monitored as compared with 74% of the unmonitored patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebellopontine Angle↗
PubMed1990

Spontaneous trigeminal-facial reinnervation.

Although spontaneous recovery of denervated facial muscles has been anecdotally recorded in the clinical setting, it has never been fully documented. The establishment of anastomoses between the terminal trigeminal and facial nerves provides a possible explanation of this phenomenon. Mechanisms of myoneurotization have also been described, by which regenerating branches of severed peripheral motor nerves directly reach motor end plates of denervated muscles, with variable recovery of function. A case demonstrating unequivocal clinical evidence of trigeminal-facial cross-innervation is presented, and the pertinent literature is reviewed as it applies to the mechanisms of this phenomenon.

Adult↗
PubMed1996

[X chromosomal bulbospinal neuropathy (X-BSN, Kennedy syndrome): an illness with repetitive triplet sequences. Case report, differential diagnosis and molecular genetics aspects].

X-chromosomal recessive bulbospinal neuronopathy (X-BNS, Kennedy's disease) is an important differential diagnosis of amyotrophic lateral sclerosis. We present the data of ten own patients along with a review of the literature on this uncommon disease which is caused by an expanded CAG-repeat in the androgen receptor gene. This mutation probably affects the transcription regulating activity of the androgen receptor in neurons. Signs and symptoms of X-BSN can be derived from partial insensitivity for androgens and a mixed, mainly motor neuronopathy. The clinical diagnosis is based on: 1. lower motor neuron weakness of bulbar and proximal limb muscles with onset in the third to fifth decade, 2. cramps and pronounced fasciculations, particularly of facial muscles, 3. postural tremor, 4. diminished or absent sensory action potentials inspite of only minor sensory impairment, 5. gynecomastia, and 6. infertility, diabetes mellitus and hyperlipoproteinemia in a minority of cases. Unlike amyotrophic lateral sclerosis, disease progression is slow with barely shortened life expectancy, which should be stressed in patient counselling. Causal treatment is as yet unavailable but several aspects of palliative medicine should be considered.

Amyotrophic Lateral Sclerosis↗