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At least 757 records · Page 42Linked to original sources
PubMed1999

Plasticity of cortical hand muscle representation in patients with hemifacial spasm.

To investigate interactions between face and hand representations in the human motor cortex, we studied patients with a hemifacial spasm before and after treatment with Botulinum toxin. Focal transcranial magnetic stimulation was used to assess the cortical motor output map of the abductor pollicis brevis muscle (APB) on both sides. Prior to therapy the representation of the APB ipsilateral to the facial muscle contractions (iAPB) was significantly smaller than on the contralateral side. Two weeks after successful therapy, the iAPB output area was significantly enlarged and expanded into the direction of the face representation. The results indicate activity dependent interactions between hand and face representations in the adult human motor cortex.

Adult↗
PubMed2003

Reconstruction of a complex hemifacial deformity with multiple simultaneous free-flap transfers: case report.

Severe disfigurement, facial paralysis, abnormal continuity of oral and nasal passages, and velopharyngeal incompetence resulted, following maxillectomy and cranial-base resection for a radiation-induced sarcoma. Oral lining, bone support, facial muscle, and outer skin were provided with a single-staged transfer of radial forearm, scapular bone, scapular skin, and serratus anterior muscle with anastamoses to the contralateral neck. This combination of flaps may be considered in complex facial deformities, and may obviate the need for staged prefabrication.

Adult↗
PubMed2003

Comparative anatomical study of the gracilis and coracobrachialis muscles: implications for facial reanimation.

Since the introduction of cross-facial nerve grafting and free vascularized muscle transfer for the treatment of longstanding facial paralysis, substantial progress has been made toward restoration of facial expression that is as normal as possible. Much of the focus has remained on the gracilis as a donor muscle. However, its inherent anatomical characteristics may preclude it from ever being more than simply a mass of contractile tissue in the face. The coracobrachialis muscle, which is the analogue in the arm of the lower limb adductor mass, was proposed as an alternative donor muscle because it was thought that certain features would allow it to improve on the overall results that are currently possible with the gracilis. A comparative anatomical study was conducted to gauge this potential. A total of 133 muscles were analyzed, including 96 dissected specimens, 16 arterial and 14 venous study specimens, and seven neurovascular study specimens. Anatomical parameters were recorded for each muscle and later tabulated. Histological analysis of the nerves to 10 gracilis and 10 coracobrachialis muscles was performed, and the findings were confirmed with intraneural dissection of an additional 20 nerves under an operating microscope. The coracobrachialis was observed to be a practical alternative to the gracilis. Indeed, it has many of the attributes that initially drew attention to the gracilis as a possible donor muscle, including a reliable neurovascular supply, minimal donor-site morbidity, and the option of having two teams operate simultaneously. In addition, it has a size, shape, and form that make it an excellent choice for transfer to the face. It could be easily attached in the face to provide static support as well as animation, because of its long proximal tendon, the thick intermuscular septum along its lateral surface, and, when present, the ligament of Struthers.

Aged↗
PubMed1999

Very severe spinal muscular atrophy (SMA type 0): an expanding clinical phenotype.

The classical form of severe spinal muscular atrophy (SMA type 1; Werdnig-Hoffmann disease) has a very consistent clinical phenotype that is well recognized by paediatricians. It usually presents at birth or within the first few months of life. There is general hypotonia, with axial and limb weakness; the legs are affected more than the arms and proximal muscles more than distal, leaving residual spontaneous activity in the feet and in the forearms and hands. Facial muscles are spared so that the infant usually has a bright normal expression. The intercostal muscles are always affected, whereas the diaphragm is spared, allowing adequate spontaneous respiratory activity until the infants are precipitated into respiratory failure by an incidental respiratory infection, or aspiration. With rare exception they die by 2 years of age with a median around 7 months and with about 80% of the children dying by the time they are 1 year old. There is a consistent homozygous deletion in exons 7 and 8 of the telomeric copy of the survival motor neuron (SMN) gene. In the current issue of the journal, MacLeod and her colleagues have documented five cases of more severe spinal muscular atrophy, with a history of diminished fetal movements in utero and presenting at birth with asphyxia and severe weakness.

Cell Survival↗
PubMed2000

Experimental study of muscle-nerve-muscle neurotization.

A laboratory model for the study of possible reinnervation by muscle-nerve-muscle (MNM) neurotization is presented. Preliminary studies revealed that MNM neurotization occurs in synergistically closely-related facial muscles. However, for larger antagonistic somatic muscle (flexor and extensor), this phenomenon could not be reproduced. The potential clinical relevance of these findings is presented and discussed.

Animals↗
PubMed1990

[Unknown cause of familial neuromuscular disease with infiltration of cytotoxic/suppressor T cells].

We report two brothers, aged 68 and 61 years respectively, who had an unclassified familial neuromuscular disease with infiltration of cytotoxic/suppressor T cells in muscle biopsies. The elder brother was in good health and active during child and adulthood until the age of 65 when muscle atrophy in the quadriceps muscle was noted. His muscle weakness and atrophy progressed relatively rapidly in the course of a few years. The muscle symptoms predominated in the under extremities at the initial stage, later to be generalized, sparing the facial muscles. The serum creatine kinase (CK) was 145 mu/ml (normal 5-80 mu/ml). EMG revealed myopathic changes. A biopsy taken from biceps muscle showed extensive inflammatory cellular infiltrations among fibers and in the perimysium. The infiltrated cells were identified to be cytotoxic/suppressor T cells. The younger brother first noticed diminished bulk in quadriceps muscle bilaterally at the age of 59. The muscle symptoms were similar to those seen in his brother. Serum CK was 520 mu/ml. EMG showed myopathic changes except for fibrillation potentials in the biceps. A biopsy taken from quadriceps muscle revealed the similar findings of his brother. The classification of these cases is a controversial subjects. We differentiated quadriceps myopathy, limb-girdle muscular dystrophy and myositis, which we couldn't classify. So at this point we reported these cases as unknown cause of familial neuromuscular disease.

Aged↗
PubMed1981

[Trigeminal neuralgia and hemifacial spasm caused by the compression of tortuous vertebro-basilar system--a case report (author's transl)].

A 63-year-old man was admitted to our clinic on August 14, 1980 with the chief compliant of left-sided intermittent facial pain. He had been suffering from twitching of the left facial muscles since 1972. During two years before admission, severe pain of the left face, starting in the left upper jaw and radiating to the left periorbital region, occurred intermittently. The pain aggravated by eating, talking and face-washing, gradually worsened and he became unable to take meals. On admission, he was alert. No sensory disturbance was seen. Muscle twitching was seen in the left orbicularis oculi, orbicularis oris and sometimes in the platysma. Mild facial palsy was recognized on the left side and other cranial nerves were intact. Computed tomogram with contrast enhancement revealed shift of the basilar artery to the left side. Markedly enlarged and tortuous vertebrobasilar system kinking at the left cerebello-pontine angle, was seen on the left vertebral angiogram. This tortuous vertebrobasilar system was presumed to be the cause of the trigeminal neuralgia and hemifacial spasm. The neuralgia disappeared after a controlled thermocoagulation of the left Gesserian ganglion, and the frequency of hemifacial spasm was reduced too. He was discharged with mild hemifacial spasm. The combination of trigeminal neuralgia and hemifacial spasm caused by direct compression of tortuous vertebro-basilar system on the nerve roots is rare. A case was reported and the literature were reviewed.

Basilar Artery↗
PubMed1993

[A case of hereditary motor and sensory neuropathy with vocal cords palsy and diaphragmatic weakness].

A case of hereditary motor and sensory neuropathy (HMSN) type 1 (Charcot-Marie-Tooth disease (CMT)) is reported with vocal cords palsy, deafness, diaphragmatic weakness, and cerebellopontine atrophy. A 42-year-old man was admitted to our hospital in April, 1991 with marked respiratory distress. He had been diagnosed as having CMT 14 years previously. On admission to our hospital, he revealed dyspnea with marked stridor during inspiration. Physical examination showed marked use of respiratory accessory muscles with thoracoabdominal paradox in the supine position. Neurologic examination revealed tonic pupils, mild bilateral weakness of facial muscles, deafness, mild bulbar palsy, severe wasting and weakness in both proximal and distal muscles of the arms and legs, areflexia, distal loss of all sensory modalities. Pes cavus and hammer toe were present. Movement of upper extremities was ataxic. No hypertrophic changes were noted in his peripheral nerves. Peripheral nerve conduction study showed undetectable both sensory and motor action potentials. Electromyography showed evidence of denervation, more marked in distal muscles. Auditory brain stem response was undetectable. Chest radiographic film showed a normal-sized heart with marked elevation of both hemidiaphragm. Laryngofiberscopy confirmed the presence of bilateral vocal cord paralysis without tumor formation, inflammation or anomaly. The vocal cords lay near the midline and did not show any movement during respiration. Moderate cerebellopontine atrophy was confirmed on MRI scan. A sural nerve section showed severe decrease of myelinated fibers, and onion bulbs. Diagnosis of HMSN type 1 was made by clinical, electrodiagnostic, and sural nerve sections study.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2006

MRI and clinical studies of facial and bulbar muscle involvement in MuSK antibody-associated myasthenia gravis.

A proportion of patients with myasthenia gravis (MG) without acetylcholine receptor (AChR) antibodies have antibodies to muscle-specific kinase (MuSK). MG with MuSK antibodies (MuSK-MG) is often associated with persistent bulbar involvement, including marked facial weakness and tongue muscle wasting. The extent of muscle wasting in MuSK-MG, and whether it is also found in the few acetylcholine receptor (AChR-MG) patients who have persistent bulbar involvement, is not clear. We studied 12 MuSK-MG patients and recruited 14 AChR-MG patients matched broadly for age, sex ratio, duration of disease and degree of ocular, bulbar and facial weakness. We used coronal and sagittal T1-weighted (T1W) and T2-weighted (T2W) magnetic resonance imaging (MRI) to assess muscle wasting in facial and tongue muscles. Hyperintense signal on T1W MRI and comparison of axial T1W sequences with cUTE sequences were used to assess fibrous/fatty tissue in the tongue. We compared the results with those of four patients with myotonic dystrophy and 12 healthy individuals. We correlated the changes with clinical and treatment histories, and established a new ocular-bulbar-facial-respiratory (OBFR) score. At the time of study, none of the clinical measures, including the OBFR score, differed between the two MG groups. MRI demonstrated thinning of the buccinator, orbicularis oris (O.oris) and orbicularis oculi (O.oculi) muscles in MuSK-MG patients compared with healthy controls, whereas thinning of these muscles was not significant in AChR-MG. Tongue areas with T1W high signal were increased in MuSK-MG patients and the intensity of the signal on axial T1W sequences was greater in MuSK-MG than in controls. To look for possible correlations between imaging and clinical findings, we pooled results from all MG patients. The duration of treatment with prednisolone at >40 mg on alternate days (AD) correlated positively with the percentage of tongue area with high signal (P = 0.006) and negatively with MRI measurements of individual muscles and with the mean muscle dimensions (P = 0.001). The new OBFR score correlated positively with current Myasthenia Gravis Foundation of America grades and with the percentage of high signal (P = 0.004) and negatively with the mean muscle dimensions (P < 0.001). The results show that bulbar and facial muscle weakness and wasting are associated with significant muscle atrophy and fatty replacement in MuSK-MG, which was not found in the AChR-MG patients. MuSK antibodies per se may predispose to muscle thinning, but the difficulties in obtaining clinical remission under steroid therapy in some patients, resulting in long duration of treatment with higher doses (>40 mg AD), may be an additional factor.

Adolescent↗
PubMed1994

Effect of the location of chewing on facial and jaw muscles activity and the mandibular movement.

This paper evaluates the influence of the location of chewing on facial and jaw muscles' activity and mandibular movements. Ten subjects with complete dentition performed following chewing sequences; 1) right side chewing (unilateral location-unspecified chewing); 2) chewing on right molars; 3) chewing on right premolars; and 4) chewing on anterior teeth. Bilateral activities of upper lip, cheek, masseter (anterior, middle and posterior parts), and anterior bellies of digastrics were recorded by surface electromyography. Mandibular movements were simultaneously recorded by electrognathography. Chewing on anterior teeth showed smaller masseteric activity in posterior part, smaller envelope of motion, and lower velocity of both opening and closing than unilateral location-unspecified chewing and chewing on right molars. Chewing on premolars showed smaller envelope of motion than chewing on molars. No significant difference was found between unilateral location-unspecified chewing and chewing on premolars or molars.

Adult↗
PubMed2004

Repetitive nerve stimulation in myasthenia gravis--relative sensitivity of different muscles.

OBJECTIVE: To correlate repetitive nerve stimulation (RNS) decrement in different muscles with the predominant clinical presentation in myasthenia gravis (MG), and to study single fibre EMG (SFEMG) sensitivity in ocular MG. METHODS: Sixty-nine, untreated, consecutive patients suspected for MG were observed prospectively for a minimum of 6 months. Those who improved on medical treatment were diagnosed as MG. The others, in whom the neurophysiological studies were normal and that did not improve on medical treatment served as a control group, from which normative data for RNS and SFEMG was obtained. The MG patients were further classified in 3 subgroups according to the predominant clinical presentation: group I (ocular); group b (bulbar); and group a (axial). We performed RNS in nasalis, trapezius, anconeus, and abductor digiti minimi. All patients with ocular MG underwent jitter determination of the orbicularis oculi muscle. RESULTS: Thirty-seven patients were diagnosed as MG (group I, 15; group b, 13; group a, 9). In group I, RNS was abnormal in 33% of the patients. RNS studies disclosed at least one abnormal muscle response in every patient in groups a and b. Trapezius was significantly more sensitive in group a, and anconeus and nasalis in group b (P < 0.01). Jitter was abnormal in all patients in group I, and the most sensitive parameter was an increased number of unstable pairs, 100%. CONCLUSIONS: Based on these observations, we recommend that a shoulder muscle, as the trapezius, should be studied first in the limb-axial presentation of MG, and the anconeus-nasalis muscles in predominant bulbar MG. In ocular MG, RNS is not sensitive and jitter should be performed in facial muscles. SIGNIFICANCE: This paper shows the unequal sensitivity of several muscles to RNS in different forms of MG.

Adult↗
PubMed2006

Experiencing positive affect and negative affect during stress: relationships to cardiac reactivity and to facial expressions.

We examined the relationship between experienced positive/negative affect and cardiac reactivity and facial muscle movements during laboratory tasks with different demands. Heart rate, respiratory sinus arrhythmia, pre-ejection period, and facial electromyography were measured during startle, mental arithmetic, reaction time task, and speech task. The results revealed that individuals experiencing high levels of positive affect exhibited more pronounced parasympathetic, heart rate, and orbicularis oculi reactivity than others. Individuals who experienced high levels of negative affects during the tasks showed higher corrugator supercilii responses. Men and women showed slightly different response patterns. To conclude, cardiac reactivity may be associated with positive involvement and enthusiasm in some situations and all reactivity should not automatically be considered as potentially pathological.

Adult↗
PubMed2000

The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome.

OBJECTIVE: To evaluate the clinical spectrum of anti-GAD-positive patients with stiff-person syndrome (SPS) and provide reproducible means of assessing stiffness. BACKGROUND: SPS can be difficult to diagnose. Delineation of the clinical spectrum in a well defined population will increase diagnostic sensitivity. METHODS: In 20 anti-GAD-positive patients with SPS (six men, 14 women), screened among 38 referred patients, the authors assessed symptoms and signs, degree of disability, associated conditions, and immunogenetic markers. Degree of bending, distribution of stiff areas, timed activities, and magnitude of heightened sensitivity were examined monthly for 4 months in five patients. RESULTS: Average age at symptom onset was 41.2 years. Time to diagnosis was delayed from 1 to 18 years (mean 6.2). Stiffness with superimposed episodic spasms and co-contractures of the abdominal and thoracic paraspinal muscles were characteristic. All had stiff gait and palpable stiffness in the paraspinal muscles. Stiffness was asymmetric or prominent in one leg in 15 patients (stiff-leg syndrome) and involved facial muscles in 13. In one patient spasms lasted for days (status spasticus). Twelve patients needed a cane and seven a walker due to truncal stiffness and frequent falls (average three to four per month). Distribution of stiffness and degree of heightened sensitivity were two reproducible indices of stiffness and spasms. Autoimmune diseases or autoantibodies were noted in 80% and an association of with DRss(1) 0301 allele in 70%. CONCLUSIONS: SPS is 1) frequently misdiagnosed due to multifaceted presentations and asymmetric signs, 2) disabling if untreated, and 3) associated with other autoimmune conditions.

Adult↗
PubMed1999

Multiplane face lift with the subperiosteal dissection for orientals.

A subperiosteal face lift rejuvenates the midface and periorbital region by restoring facial muscle tone. Since 1993, the authors have performed this procedure on Oriental patients who have their own distinct facial contours: the brachycephalic cranium and a prominent zygoma and mandibular angle. Although it was thought that these protuberances might disturb the subperiosteal procedure, especially in the anterior midface, the procedure could be performed easily by adopting the ancillary upper buccovestibular and subciliary incisions; the authors found that the protuberances actually act as fulcrums to keep up the lifting vectors reliably. For older patients, the procedure was combined with a deep subcutaneous dissection. A simple lift of the periosteum would not improve a severe nasolabial fold deformity and prominent wrinkles adequately because of "lag-lifting" of the superficial layer. It was concluded that the multiplane face lift, consisting of the subperiosteal and the deep subcutaneous approaches, achieves a natural-appearing rejuvenation of the Oriental aging face.

Aged↗
PubMed1996

New perspectives in intraoperative facial nerve monitoring with antidromic potentials.

Electromyographic (EMG) activity recorded from the facial muscles in response to electrical and mechanical stimulation is the most popular method for continuous monitoring of the facial nerve during cerebellopontine-angle surgery. EMG recording is, however, extremely sensitive to the administration of neuromuscular blockers. An alternative technique for the continuous monitoring of the facial nerve [i.e., monopolar recording of facial nerve antidromic potentials (FNAPs)], is described. Ten subjects undergoing retrosigmoid vestibular neurectomy for Meniere's disease and 11 subjects operated on with acoustic neuroma surgery via a retrosigmoid approach (tumor size range, 12-28 mm) participated in the investigation. Bipolar electrical stimulation of the marginalis mandibulae was performed to elicit FNAPs. Stimulus intensity ranged from 0 to 10 mA with a delivery rate of 7/s. Antidromic potentials were recorded with a silver-wire monopolar electrode positioned intracranially on the proximal portion (root entry zone) of the acoustic-facial bundle. Bipolar recordings with two silver electrodes were also performed from the trigeminal and facial nerves in the cerebellopontine angle to define the specific origin of the action potentials. FNAP amplitude increased as a function of stimulus intensity. The average latency was 3.35 ms (range, 3.0-3.7 ms). Action potentials recorded intracranially during electrical stimulation of the marginal nerve originated specifically from the facial nerve. Changes in latency and amplitude of FNAPs were analyzed as a function of the main surgical steps in patients operated on for acoustic neuroma. FNAP monitoring provided quantitative real-time information about damaging maneuvers performed on the nerve and postoperative facial function.

Adult↗