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Reliability of the "Sydney," "Sunnybrook," and "House Brackmann" facial grading systems to assess voluntary movement and synkinesis after facial nerve paralysis.

OBJECTIVE: To investigate the extent of within-system reliability and between-system correlation for the "Sydney" and "Sunnybrook" systems of grading facial nerve paralysis, and to examine the interobserver reliability and agreement of the "House Brackmann" grading system. STUDY DESIGN: A fixed-effects reliability study in which 6 otolaryngologists viewed videotapes of patients with facial nerve paralysis. SETTING: University and medical Centers. PATIENTS: Patients with unilateral lower motor neurone facial nerve dysfunction greater than 1 year after onset, none of whom had undergone surgical reanimation procedures. Intervention Twenty-one patients with facial nerve paralysis were videotaped while they performed a protocol of facial movements. Six otolaryngologists viewed the videotapes and scored them with the Sydney and Sunnybrook systems, and then gave a House Brackmann grade. MAIN OUTCOME MEASURE: The 3 systems of grading facial nerve paralysis were evaluated and compared with the use of intraclass correlation coefficients, Pearson's weighted kappa, and percentage exact agreement values. RESULTS: The Sydney and the Sunnybrook systems had good intrasystem reliability and high intersystem association for the assessment of voluntary movement. Grading of synkinesis was found to have low reliability both within and between systems. The House Brackmann system had substantial reliability as shown by weighted kappa but had a percentage exact agreement of 44%. CONCLUSIONS: For clinical grading of voluntary movement, there is good correlation between ratings given on the Sydney and Sunnybrook systems, and within each system there is good reliability. The assessment of synkinesis was far less reliable within, and less related between, systems. Although the reliability of the House Brackmann system was found to be high, examination of individual grades revealed some wide variation between trained observers.

Adult↗

Ocular movements in essential blepharospasm.

In essential blepharospasm histopathologic and electrophysiologic evidence supports the existence of lesions in proximity to brainstem nuclei controlling ocular movements. We studied horizontal ocular movements in eight patients who had been treated previously with surgery or botulinum toxin injection to control essential blepharospasm (mean age, 58 years) and compared these with seven control subjects who did not have blepharospasm (mean age, 68 years). We examined fixation stability, saccades, the vestibulo-ocular reflex, visual enhancement and suppression of the vestibulo-ocular reflex, optokinetic nystagmus, and pursuit by using digitally sampled, direct current electro-oculography. Patients with blepharospasm exhibited no ocular movement abnormalities. Since quantitative aspects of ocular movements are sensitive to nonspecific brainstem lesions, the absence of abnormal ocular movements suggests that the lesion in blepharospasm is specifically limited to neurons regulating the facial muscles.

Adult↗

Dysphagia complications of the Minerva brace.

The effects of cervical bracing on swallowing function have not been evaluated in neurologically intact individuals. An 83-year-old woman fell, striking her head, and suffered C1, odontoid, and C3 fractures. She had no neurologic deficits and was placed in a Minerva brace. Subsequently, she developed coughing during her meals, a low-grade fever, and transient hoarseness, and complained of stiffness in the facial muscles. Aspiration pneumonia in the left lower lobe was diagnosed. In the absence of any neurologic condition, this was attributed to the Minerva brace. Less than 1 week later, the brace was replaced with a halo-vest, and the dysphagia resolved. This case shows that dysphagia and aspiration pneumonia may be caused by wearing a cervical brace and illustrates the importance of assessing swallowing in individuals who wear such braces. Particular attention should be paid to swallowing and cervical bracing in patients with additional risk factors for dysphagia such as advanced age or neurologic deficits.

Aged↗

High frequency stimulation of the subthalamic nucleus improves treadmill locomotion in unilateral 6-hydroxydopamine lesioned rats.

This study investigated the influence of electrical stimulation of the subthalamic nucleus (STN) on motor impairment induced by unilateral 6-hydroxydopamine (6-OHDA) lesions in the medial forebrain bundle. Rats were trained to walk on a treadmill and then implanted with microelectrode arrays in and near the STN. The neurotoxin 6-OHDA was injected into the medial forebrain bundle (MFB) unilaterally to produce a targeted lesion of the dopaminergic system. Successful lesions produced impaired treadmill walking behavior. High frequency stimulation (HFS) of the STN improved treadmill walking immediately and restored normal walking patterns. The same HFS failed to evoke visible side effects such as stepping, turning, raising of the head or facial muscle contraction in the absence of treadmill movement, or to change rotational behaviors elicited by the dopamine (DA) agonist apomorphine in unilateral lesioned rats. This suggests that the stimulation did not cause movement by an activation of brainstem locomotor regions or an increase attention leading to movement. Apomorphine-induced rotation may represent an imbalance of dopaminergic activation which remains during HFS. This work may provide a rodent model for deep brain stimulation (DBS) in patients with Parkinson's disease, and be suitable for further investigation of the neural mechanisms underlying the therapeutic effects of DBS.

Animals↗

Effects of D-AP5 and NMDA microiontophoresis on associative learning in the barrel cortex of awake rats.

Experiments involving single-unit recordings and microiontophoresis were carried out in the barrel cortex of awake, adult rats subjected to whisker pairing, an associative learning paradigm where deflections of the recorded neuron's principle vibrissa (S2) are repeatedly paired with those of a non-adjacent one (S1). Whisker pairing with a 300 ms interstimulus interval was applied to 61 cells. In 23 cases, there was no other manipulation whereas in the remaining 38, pairing occurred in the presence of one of three pharmacological agents previously shown to modulate learning, receptive field plasticity and long-term potentiation: N-methyl-D-aspartic acid (NMDA) (n=8), the NMDA receptor antagonist AP5 (n=17) or the nitric oxide synthase inhibitor L-nitro-arginine-N-methyl-ester (L-NAME) (n=13). Non-associative (unpaired) experiments (n=14) and delivery of pharmacological agents without pairing (n=14) served as controls. Changes in neuronal responsiveness to S1 following one of these procedures were calculated and adjusted relative to changes in the responses to S2. On average, whisker pairing alone yielded a 7% increase in the responses to S1. This enhancement differed significantly from the 17% decrease obtained in the non-associative control condition and could not be attributed to variations in the state of the animals because analysis of the cervical and facial muscle electromyograms revealed that periods of increased muscular activity, reflecting heightened arousal, were infrequent (less than 4% of a complete experiment on average) and occurred randomly. The enhancement of the responses to S1 was further increased when whisker pairing was performed in the presence of L-NAME (27%) or NMDA (35%) whereas AP5 reduced it to 1%. During the delivery period, NMDA enhanced both neuronal excitability and responsiveness to S1 whereas AP5 depressed them. However, the effects of both substances disappeared immediately after administration had ended. L-NAME did not affect the level of ongoing activity and responses to S1 significantly. From these data, we concluded that, since the changes in the responses to S1 lasted longer than the periods of both whisker pairing and drug delivery, they were not residual excitatory or inhibitory drug effects on neuronal excitability. Thus, our results indicate that, relative to the unpaired controls, whisker pairing led to a 24% increase in the responsiveness of barrel cortex neurons to peripheral stimulation and that these changes were modulated by the local application of pharmacological agents that act upon NMDA receptors and pathways involving nitric oxide. We can infer that somatosensory cerebral cortex is one site where plasticity emerges following whisker pairing.

2-Amino-5-phosphonovalerate↗

Transgenic analysis of the atrialnatriuretic factor (ANF) promoter: Nkx2-5 and GATA-4 binding sites are required for atrial specific expression of ANF.

The atrial natriuretic factor (ANF) gene is initially expressed throughout the myocardial layer of the heart, but during subsequent development, expression becomes limited to the atrial chambers. Mouse knockout and mammalian cell culture studies have shown that the ANF gene is regulated by combinatorial interactions between Nkx2-5, GATA-4, Tbx5, and SRF; however, the molecular mechanisms leading to chamber-specific expression are currently unknown. We have isolated the Xenopus ANF promoter in order to examine the temporal and spatial regulation of the ANF gene in vivo using transgenic embryos. The mammalian and Xenopus ANF promoters show remarkable sequence similarity, including an Nkx2-5 binding site (NKE), two GATA sites, a T-box binding site (TBE), and two SRF binding sites (SREs). Our transgenic studies show that mutation of either SRE, the TBE or the distal GATA element, strongly reduces expression from the ANF promoter. However, mutations of the NKE, the proximal GATA, or both elements together, result in relatively minor reductions in transgene expression within the myocardium. Surprisingly, mutation of these elements results in ectopic ANF promoter activity in the kidneys, facial muscles, and aortic arch artery-associated muscles, and causes persistent expression in the ventricle and outflow tract of the heart. We propose that the NKE and proximal GATA elements serve as crucial binding sites for assembly of a repressor complex that is required for atrial-specific expression of the ANF gene.

Animals↗

Activation of N-methyl-D-aspartate receptors in the feline retrorubral nucleus elicits orofacial dyskinesia.

Stimulation of dopamine receptors within a circumscribed subregion of the feline caudate nucleus, that is the anterodorsal part of this nucleus, with dopamine or the dopamine receptor agonist (3,4-dihydroxyphenylimino)-2-imidazoline (DPI) elicits orofacial dyskinesia. Orofacial dyskinesia is a syndrome of tic-like contractions of the facial muscles which ends with a tongue protrusion. Afferent fibres of the anterodorsal part of the caudate nucleus are known to emanate from the retrorubral nucleus, including the dopaminergic A8 cell group. The present study was undertaken to investigate whether excitation of A8 cells can mediate and/or modulate orofacial dyskinesia. For this purpose, the activity of the retrorubral nucleus was manipulated with local injections of N-methyl-D-aspartate (NMDA). These local injections into the retrorubral nucleus were subsequently combined with manipulations of the dopamine transmission in the anterodorsal part of the caudate nucleus with local injections of DPI. The present study shows that injections of NMDA into the retrorubral nucleus elicits orofacial dyskinesia. This effect is dose-dependent, NMDA-specific, and inhibited by intra-caudate injections of DPI.

Animals↗

Hemifacial spasm: results of microvascular decompression of the facial nerve in 54 patients.

During the 10-year period between January 1975 and December 1984, 367 patients with hemifacial spasm were examined at our institution. Because of the severity of the spasm, 54 patients underwent surgical management that consisted of microvascular decompression of the facial nerve. Postoperatively, the hemifacial spasm was completely resolved in 44 patients (81%), but 6 of these patients had subsequent recurrence of the condition. An additional five patients (9%) experienced improvement but were not totally free of the spasm. Five patients (9%) received no benefit from the procedure. Complications occurred in 19 patients (35%) but were usually transient. The most serious complication was permanent ipsilateral hearing loss, which occurred in eight patients (15%). Thus, in general, microvascular decompression of the facial nerve effectively alleviates hemifacial spasm.

Adult↗

[Neuromuscular abnormalities in critical illness].

Critical illness neuromuscular abnormalities (CINMA) are found in 25 percent of ITU patients who recover consciousness and are characterized by a bilateral and symmetric weakness that involves the four limbs but spares the facial muscles. Electrophysiological testing shows an axonal sensory motor polyneuropathy and/or myopathy. The main risk factors of CINMA are prolonged durations of multiple organ failure and mechanical ventilation, use of corticosteroids and hyperglycaemia. CINMA contribute also to increase the duration of mechanical ventilation, this effect being mediated by diaphragm weakness. The median duration of limb weakness is 21 days, although it can exceed several months in some patients. Few preventive measures have been assessed. Whether the benefit of strict blood glucose control in ITU patients recovering from heart surgery on CINMA incidence can be extended to medical ICU patients needs to be determined.

Critical Care↗

[Finnish amyloid polyneuropathy in a French patient].

INTRODUCTION: Finnish amyloid variety is a rare familial amiloidosis polyneuropathy essentially observed in Finland. It concerns about six hundred people in the world in which five hundred reside in Finland. OBSERVATION: We report a case of a 58-year-old French woman with a 10-year history of lattice cornea dystrophy. She consulted in January 2004 for impaired swallowing, facial paralysis principally of the right superior territory and symptoms of arthritis which had developed a few months earlier. Observation revealed facial cutis laxa, tongue amyotrophy and some fasciculation. Electroneuromyography showed chronic neurogenic involvement of the facial muscles. Limbs and the sympathetic neuronal system were free of involvement. Pathological examination revealed areas of peri vascular amiloid deposits. Molecular biology confirmed the diagnosis of Finnish amiloidosis: substitution of aspartic acid by tyrosine in the 187 codon in the 9th chromosome (gelsoline gene). This mutation has been previously found in Denmark and the Czech Republic. CONCLUSION: Finnish amiloidosis is a familial polyneuropathy characterized by an association of cornea lattice dystrophy, cutis laxa and a chronic neurogenic involvement of the cranial nerves. Two mutations are known. Life expectancy is not affected, but quality of life is altered.

Amyloid Neuropathies, Familial↗

Facial reflexes.

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Brain Diseases↗

Systematized inflammatory epidermal nevus with symmetrical involvement: an unusual case of CHILD syndrome?

The CHILD syndrome (congenital hemidysplasia with ichthyosiform nevus and limb defects) is usually characterized by lateralization of all associated anomalies. It has been assumed that the event of X-inactivation coincides and interferes with a clone of organizer cells controlling a large developmental field. A 16-year-old girl with bilateral manifestations of CHILD syndrome is described. The inflammatory skin lesions affected the body folds (ptychotropism) in a symmetrical distribution, although only the right side of the neck was involved. In addition, absence of several facial muscles, vertebral defects, and shortening of the leg on the right side were noted, and a ventricular septum defect was present. This unusual case may be explained by the assumption that X-inactivation did not coincide with the origin of inducer cell clones controlling large morphogenetic fields on either side of the body.

Abnormalities, Multiple↗

Rapid intraoperative facial nerve expansion.

The repair of nerve length defect presents a reconstructive challenge after trauma and oncologic resection. This study examined rapid intraoperative nerve expansion as a method of repairing nerve length defects with the cat facial nerve model. We compared expanded nerves with grafted nerves and intact nerves 1 year after repair using the criteria of gross function (symmetry and blink reflex according to a modified House scale), electromyography thresholds, nerve-conduction velocity, morphology, and axon count. Three of the five expanded nerves regenerated, and all of the grafted nerves regenerated. Functional results were similar for the regenerated expanded and the grafted facial nerves, and both methods achieved an equivalent level of function. The facial nerves of the regenerated expanded group, grafted group, and intact group had mean electromyography thresholds of 132 mV, 98 mV, and 134 mV, respectively, and mean conduction velocities of 48.3 mg/second, 47.9 m/second, and 44.7 m/second, respectively. Morphologic examination of all five expanded nerves immediately after the expansion process revealed an intact fascicular structure. However, 1 year after excision of the expanded segment and repair, only three of the five nerves regenerated. Axon count at 1 year was as follows: 404 for the regenerated expanded nerves, 449 for the grafted nerves, and 403 for the intact nerves. The potential advantages of rapid intraoperative nerve expansion over nerve grafting for the repair of nerve gap defects include a single suture line and absence of donor site morbidity. This pilot study demonstrates that rapid intraoperative nerve expansion and regeneration is possible and can be used to repair a nerve length deficit. The development of a rapid and reliable method of intraoperative nerve expansion deserves further study.

Animals↗

The effectiveness of neuromuscular facial retraining combined with electromyography in facial paralysis rehabilitation.

OBJECTIVE: The study goal was to present the effectiveness of neuromuscular facial retraining techniques used in combination with electromyography for improving facial function even in cases of longstanding paralysis. STUDY DESIGN AND SETTING: We conducted a retrospective case review in a tertiary neurotology clinic. PATIENTS: Twenty-four patients with facial paralysis received neuromuscular facial retraining between April 1999 and April 2001. The patient sample included 6 males and 18 females, with an average age of 44 years. A control group consisted of 6 patients (4 females and 2 males). RESULTS: All patient groups made significant improvements in function with improved symmetry in dual-channel electromyographic readings and increased facial movement percentages. Some of the percentages of posttreatment facial function were as follows: acoustic neuromas, 93%; Bell's palsy/Ramsay Hunt syndrome, 80%; and facial nerve anastomosis, 71%. Synkinesis was reduced by at least 2 levels in patients who initially demonstrated synkinesis. CONCLUSIONS: Neuromuscular facial retraining exercises and electromyography are effective for improving facial movements. SIGNIFICANCE: Facial retraining is an excellent example of the plasticity of the central nervous system to reorganize, even in cases of longstanding paralysis.

Adolescent↗