Alteration of the orbicularis oculi reflex by pontine lesions. Study in multiple sclerosis.
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Electric Stimulation↗
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Results for “FACIAL MUSCLES”
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Perineural spread of cutaneous basal and squamous cell carcinomas. The clinical appearance of spread into the trigeminal and facial nerves.
Five patients were studied in whom a trigeminal or facial neuropathy resulted from perineural spread of basal or squamous cell carcinomas arising in the skin of the face. The cause of the neuropathy was not immediately apparent because there was no evidence of local skin recurrence in any of the patients after the onset of their neurologic symptoms. Pain was a prominent feature in those patients with trigeminal involvement. Radiologic investigations were helpful in only one patient. The diagnosis should be suspected when symptoms and signs are confined initially to superficial branches of the trigeminal or facial nerves and later extend to more central branches in the order in which they arise. Confirmation can be made by microscopic examination of the nerves involved.
Aged↗
The blink reflex in patients with idiopathic torsion dystonia.
The blink reflex and its recovery cycle were examined in 57 patients with idiopathic dystonia affecting different parts of the body. The group comprised 9 patients with generalized and 15 with segmental forms, 19 with torticollis, and 14 with focal arm dystonia. None had blepharospasm. The duration and amplitude of the R2 component of the blink reflex showed only minor changes. However, its recovery cycle to paired supraorbital nerve stimuli was abnormal in all groups of patients, except those with focal arm dystonia. These findings may be interpreted as showing abnormal control of the interneuronal networks mediating the blink reflex in patients with dystonia affecting sites other than the facial muscles. The fact that the principal changes were seen in patients with torticollis, and generalized or segmental dystonia, suggests that the extent of dystonia (rather than the severity) and, therefore, the close proximity to the cranial muscles was important in determining the extent of the abnormal interneuron function.
Arm↗
Tibial muscular dystrophy. Late adult-onset distal myopathy in 66 Finnish patients.
OBJECTIVE: To clarify the classification of two previously reported groups of patients with anterior tibial distal dystrophy, to find additional patients with the disease, and to describe the clinical features of this disease. DESIGN: National survey of the records of patients with neuromuscular diseases in Finland. Findings of selected patients were compared with those of previously reported cases. PATIENTS: Thirty-six previously described patients and 30 additional patients from the current survey, with 41 symptomatic patients and 25 subjectively asymptomatic affected relatives. RESULTS: There were 66 patients with late adult-onset tibial muscular dystrophy. Symptoms appear after the age of 35 years with reduced ankle dorsiflexion, and progress is slow without marked disability. Facial muscles, upper extremities, and proximal muscles are usually spared. Muscle biopsy results reveal nonspecific dystrophic changes in clinically affected muscles, and frequently severe adipose replacement in the anterior tibial muscles occurs. Asymptomatic muscles have mild myopathic changes only. Vacuolar degeneration is detected in a minority of patients. Electromyography shows profound myopathic changes in the anterior tibial muscle, but extensor brevis muscles are well preserved. Computed tomography or magnetic resonance imaging of muscles discloses marked involvement of tibial extensor muscles and focal patches of fatty degeneration in various asymptomatic muscles. Pedigree data suggest autosomal dominant inheritance. CONCLUSIONS: Tibial muscular dystrophy might represent a new form of distal myopathy and it is rather common, at least in Finland.
Adolescent↗
The lesion causing continuous facial myokymia in multiple sclerosis.
OBJECTIVE: To attempt to identify the site of the lesion causing continuous facial myokymia (CFM) in multiple sclerosis (MS) through the use of magnetic resonance imaging (MRI). DESIGN: A case series was employed. SETTING: The Baird Multiple Sclerosis Center, Millard Fillmore Hospital, and the Neurology Department, Buffalo General Hospital, Buffalo, NY. PARTICIPANTS AND MEASURES: Twelve patients with MS and CFM were examined by MRI of the brain while the CFM was present. The MRI examinations were also performed before the CFM had developed and after it had ceased in eight of the patients. Another 57 patients with MS who never had CFM but who had similar disabilities to those who did were also examined by MRI. RESULTS: In 11 of the 12 patients with MS and CFM, the causative lesion was demonstrated to involve the postnuclear, postgenu portion of the facial nerve intraaxially in the dorsolateral pontine tegmentum ipsilateral to the CFM. In the majority of patients who were studied after the CFM had stopped clinically, the lesion was observed to resolve on MRI. Seventeen percent of the patients with MS but without CFM were found to have the typical pontine tegmental lesion. CONCLUSIONS: Continuous facial myokymia in MS is caused by a pontine tegmental lesion involving the postnuclear, postgenu portion of the facial nerve. The lesion is identified by MRI in approximately 90% of patients with MS who have CFM clinically. The typical MRI lesion may also be found in a minority of patients with MS who do not have CFM clinically.
Adult↗
Measurement of facial movement with computer software.
OBJECTIVE: To adapt desktop computer software to objectively grade facial movement. DESIGN: The criteria of the facial nerve grading system by House and Brackmann, the current "gold standard," are prone to ambiguous interpretation. Proposed objective grading systems compare the movement of points on each side of the face or use subtraction and thresholding of digitized images of the face to yield images that represent moving areas of the face. The movement of a point on the face and the area of motion determined by digital subtraction were compared during an increasing smile in healthy subjects. The Nottingham system (calculated using measurement of the movement of 4 points on the face) using desktop computer software (Adobe Photoshop 3.0, Adobe Systems Inc, Mountain View, Calif) to measure movement of the points was compared with the system by House and Brackmann. The computer software was used to subtract digitized images and derive a facial movement score, which was compared with the scores of the systems by Nottingham and House and Brackmann. SETTING: Academic otologic practice. STUDY PARTICIPANTS: Nine patients with varying degrees of facial nerve disability and 7 individuals with normal facial nerve function. RESULTS: The movement of the oral commissure compared with the apparent area of movement of the face determined by digital subtraction had high intersubject variability. In patients with facial weakness, the Nottingham score had a correlation coefficient of -0.97 compared with the House and Brackmann grade, and the digital subtraction score had a correlation coefficient of -0.62 (paired Student t test). CONCLUSIONS: The desktop computer software can be used to calculate the Nottingham score. Digital subtraction as a measure of facial function warrants further study.
CD-ROM↗
Resistance to botulinum toxin injections for spasmodic dysphonia.
A known complication of botulinum toxin injections in the treatment of dystonia has been the occasional development of resistance. This has been seen primarily with patients who receive injections for torticollis. We report on 2 patients who, after several years of receiving injections for spasmodic dysphonia, developed clinical resistance by failing to have expected voice improvement after receiving laryngeal reinjection. One of the 2 patients demonstrated antibodies to botulinum toxin by mouse neutralization bioassay. The second patient had negative bioassay results but had no clinical response to the test toxin injection of facial muscles. These cases demonstrate that, although uncommon, resistance to botulinum toxin injections can occur in the treatment of spasmodic dysphonia. We offer suggestions to limit this complication.
Animals↗
Teratoma of the middle ear and mastoid process. A case with aberrant innervation of the facial musculature.
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Adult↗
The surgical treatment of facial hyperkinesia.
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Aged↗
Interfascicular nerve repair. Is it applicable in facial injuries?
The reapproximation of individual fascicles has been advocated as a microsurgical method of improving neural regeneration following peripheral nerve injuries. To date, this technique has met with limited success, in part owing to an unclear understanding of the anatomic location of fibers in the proximal nerve relative to their distal distribution on the face. Fascicular repair is indicated in clean lacerations of the nerve trunk distal to the stylomastoid foramen. For long defects or injuries proximal to the midmastoid region (where the nerve is monofascicular), simple nerve trunk sutures should be performed until more detailed information on the fascicular anatomy of the intratemporal facial nerve becomes available. A case is reported in which a stab wound of the parotid region was complicated by hemorrhage and infection. A delayed neurorrhaphy was performed using a modified interfascicular grafting technique with sural nerve grafts.
Adult↗
Maximal nerve excitability testing vs electroneuronography.
In an article published in 1977, Adour et al compared electroneuronography (neuromyography) with maximal nerve excitability testing (NET) and reached the conclusion that the prognostic value of the latter was more reliable in facial paralysis. Further analysis of the results of Adour et al leads, however, to the opposite conclusion and illustrates how one may avoid the pitfalls that exist in using electrical stimulation to evaluate facial nerve function. Certainly, electroneuronography (ENoG) is not a foolproof method and experience is needed in order to avoid errors in technique and to achieve reproducible results. The great advantage of ENoG vs maximal NET is the quantitative analysis of the number of degenerated fibers and the assessment of an accurate degeneration profile in Bell's palsy.
Action Potentials↗
A new surgical technique for facial hyperkinesia.
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Facial Muscles↗
Surgical anatomy of the extraparotid distribution of the facial nerve.
The peripheral, extraparotid distribution of the clinically important branches of the facial nerve is described, with common variations, based on the anatomical dissection of 35 cadaver half heads. Methods are suggested for avoiding, isolating, and protecting the facial nerve branches during surgical procedures.
Cadaver↗
Hemifacial spasm. Occurrence in multiple sclerosis.
We present six patients with hemifacial spasm and multiple sclerosis. To our knowledge, this association has not been described previously in the North American literature. Magnetic resonance imaging was obtained in all the patients and plaques consistent with multiple sclerosis were identified. In two patients the plaques were seen in the area of the facial nucleus on the involved side. We suggest that hemifacial spasm can be a manifestation of multiple sclerosis. These cases illustrate the utility of magnetic resonance imaging in the investigation of hemifacial spasm. Our findings also support a central (nuclear) origin in multiple sclerosis associated with hemifacial spasm.
Adrenocorticotropic Hormone↗
Time course of Bell palsy.
This study was designed to quantitatively characterize the time course of facial palsy and the relationship between electroneurography and the facial nerve grading percentage as a function of onset time. Bilateral electroneurographic recordings during different stages in the course of the disease were repeated and compared with categorized videotaped facial movements using the House-Brackmann facial nerve grading system in 32 patients with Bell palsy. Preliminary results of this study demonstrate a time gap between the percentage of electroneurographic response and the category of the facial nerve grading system during the same period of disease progress. A theoretical model of the time course and specific patterns regarding the recovery of facial function is established. The theoretical time course of facial palsy is divided into 3 stages; preclinical, clinical, and postclinical. Based on the time course of electroneurographic and facial grading functions, the period between 10 and 14 days after onset was found to be most valuable for prediction of recovery. According to this theoretical model, a given time course pattern based on the results of serial electroneurographic recordings provides reliable prognostic information on recovery from Bell palsy.
Action Potentials↗
Identification of motor areas of the cat cerebral cortex based on studies of cortical stimulation and corticospinal connections.
The location and topography of motor areas in the cat cerebral cortex were studied by electrical stimulation of the cortex in five animals, and by the injection of retrograde tracers into the spinal cord of four animals. Movements evoked by intracortical microstimulation (ICMS) of the anterior, posterior and lateral sigmoid gyri, both banks of the cruciate sulcus and the dorsal bank of the presylvian sulcus were observed in anaesthetized cats. Fluorescent tracers (Fast Blue and/or Diamadino Yellow) were injected into the lateral funiculus in the second cervical segment, into the gray matter of cervical segments C3-T1 and/or into the gray matter of lumbar segments L2-S1. Contraction of the contralateral forelimb, hindlimb or facial muscles was observed following electrical stimulation of several cytoarchitectonic areas: 4 gamma, 4 delta, 6a alpha, 6a gamma, and 3a. These findings suggested representations of contralateral forelimb and hindlimb movements in areas 4 gamma and 4 delta, and of the contralateral forelimb muscles in areas 6a alpha and 6a gamma. Corticospinal neurons were located in all the above cytoarchitectonic areas as well as in areas 3b, 1, 2, 2pri, and 5. Large numbers of neurons were labeled in areas 4 gamma and 4 delta, and moderate labeling was observed in areas 6a gamma and 6a alpha. Corticospinal neurons projecting to cervical and lumbar segments were located in areas 4 gamma and 4 delta, while those projecting only to cervical segments were detected in areas 6a alpha and 6a gamma. Based on these findings it is proposed that within the motor cortex of the cat there are representations of limb movements in several cytoarchitectonic subdivisions. Many of these representations may be candidate secondary motor areas.
Animals↗
Dystonia as a presenting feature of the 3243 mitochondrial DNA mutation.
A variety of neurologic phenotypes have been described in patients with mitochondrial disorders. We report a 32-year-old man in whom dystonia was the salient and presenting feature of a mitochondrial DNA mutation. He presented at age 23 with writer's cramp and progressed over 5 years to exhibit dystonia in facial muscles and lower limbs. He also has exercise intolerance, mild, bilateral ptosis, proximal muscle weakness, and sensorineural hearing loss. Molecular genetic analysis of blood, urine, and muscle biopsy demonstrated the presence of a heteroplasmic point mutation at nucleotide position 3243. The 3243 mtDNA mutation has pleomorphic manifestations, and dystonia should be added to the list of associated clinical features.
Adult↗
Clinical study and haplotype analysis in two brothers with Partington syndrome.
Partington et al. [1988] described a three-generation family (MRXS1, MIM *309510, PRTS) with a syndromic form of X-linked mental retardation (XLMR). The clinical features in 10 affected males included mild to moderate MR, dystonic movements of the hands, and dysarthria. After refinement, the PRTS locus was mapped to marker DXS989 (with maximum LOD score of 3.1) with flanking markers DXS365 and DXS28. Since then, no other patients with a similar phenotype have been described. We present a detailed description of the neurological symptoms and the disease history of two brothers with the clinical features of PRTS. Psychomotor development was delayed in both, and neurological features included mild to moderate mental retardation, dysarthria, facial muscle weakness, severe dysdiadochokinesis, slow dystonic movements, and mild spasticity of the hands, without ataxia or spasticity of the legs. The symptoms were nonprogressive and extrapyramidal, and without cerebellar involvement. In general, behavior of the two brothers was friendly and quiet, although the elder brother had periods of depressed mood and outbursts of anger. Karyotypes and subsequent investigation of the subtelomeres as well as DNA analysis of the FMR1 gene, the androgen receptor gene, and the DM locus did not reveal a genetic abnormality. Haplotype analysis showed that the affected brothers share the PRTS region at Xp22.1. Mutation screening of the PDH-E1alpha gene did not reveal a pathogenic mutation.
Adult↗